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ae CH Neurology Self-Assessment Test — =] = oa = wn wn This self-assessment test contains one-best-answer multiple-choice questions. Please read these directions carefully @o m2 before answering the questions. Answers, critiques, and bibliographies immediately follow these multiple-choice wn 4 questions. The American College of Physicians (ACP) is accredited by the Accreditation Council for Continuing ao uw Medical Education (ACCME) to provide continuing medical education for physicians. n The American College of Physicians designates MKSAP 19 Neurology for a maximum of 24 AMA PRA Category 1 CreditsTM. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Successful completion of the CME activity, which includes participation in the evaluation component, enables the participant to earn up to 24 medical knowledge MOC points in the American Board of Internal Medicine’s Maintenance of Certification (MOC) program. It is the CME activity provider’s responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

Successful completion of the CME activity, which includes participation in the evaluation component, enables the participant to earn up to 24 medical knowledge MOC points in the American Board of Internal Medicine’s Maintenance of Certification (MOC) program. It is the CME activity provider’s responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit. Earn Credits or MOC Points Online To earn CME credits or to apply for MOC points, MKSAP users need to answer at least one of two questions correctly (earning a score of at least 50%) and click the Submit CME button. Each single MKSAP 19 self-assessment question qualifies for one quarter of a CME credit hour or ABIM MOC point. MKSAP 19 Subscribers can enter their self-assessment question answers and submit for CME/MOC in two ways: 1. Users of MKSAP 19 Complete who prefer to use their print books and a paper answer sheet to study and record their answers can use the printed answer sheet at the back of this book to record their answers. The corresponding online answer sheets, which are available on the MKSAP 19 Resource Page, may be used to transcribe answers onto the online answer sheets. Users may then submit their answers to qualify for CME credits or MOC points (see below for information on Opting in for MOC). Users who prefer to record their answers on a paper answer sheet should save their answer sheet for future use. Users who study with MKSAP 19 print can also submit their answers directly within MKSAP 19 Digital by accessing the self-assessment questions dashboard and selecting the preferred subspecialty section to begin answering questions. 2. Users of MKSAP 19 Digital can enter their answers within the digital program by accessing the self-assessment questions dashboard and selecting the preferred subspecialty section to begin answering questions and click- ing the Submit CME button once they qualify for CME and are ready to submit. Learners should keep in mind their yearly CME and MOC deadlines when determining the appropriate time to submit. Learners’ CME/MOC submission progress will be shown on the MKSAP 19 Digital CME/MOC/CPD page.

2. Users of MKSAP 19 Digital can enter their answers within the digital program by accessing the self-assessment questions dashboard and selecting the preferred subspecialty section to begin answering questions and click- ing the Submit CME button once they qualify for CME and are ready to submit. Learners should keep in mind their yearly CME and MOC deadlines when determining the appropriate time to submit. Learners’ CME/MOC submission progress will be shown on the MKSAP 19 Digital CME/MOC/CPD page. Opting in for MOC MKSAP 19 users can opt in for simultaneous submission of CME and MOC points as they answer self-assessment questions. To opt in, users will be required to complete a form requesting their name, date of birth, and ABIM number. The MOC Opt-in Form will be presented upon the user’s first CME submission and needs to be completed only once. 95

re Each of the numbered items is followed by lettered answers. Select the ONE lettered answer that is BEST in each case. j ct Sot i=

Each of the numbered items is followed by lettered answers. Select the ONE lettered answer that is BEST in each case. j ct Sot i= E wn Item 1 in the toes, decreased sensation to pinprick below the nipple nn a line bilaterally, and a palpable bladder. Upper extremity w A 25-year-old man is evaluated for a 1-year history of head- n reflexes are 2+ in both biceps. Arm strength is normal. Gait aches. He recently returned from active military duty where = is ataxic. ae he sustained a mild traumatic brain injury (TBI). A CT scan we 172) obtained after injury was normal. Headaches, which started Which of the following is the most appropriate diagnostic soon after the TBI, typically occur three times weekly, last test to perform next? 24 hours, and are bifrontal, dull, and moderate in intensity. The patient also reports occasional photosensitivity accom- (A) CT myelography panying the headaches but has had no nausea, phonopho- (B) MRI of the brain bia, visual disturbances, or neurologic symptoms. He takes (C) MRI of the lumbosacral spine ibuprofen as needed to relieve the pain. (D) MRI of the thoracic spine On physical examination, vital signs and all other physical examination findings are normal. Results of standard laboratory studies are normal. Item 4 Which of the following is the most appropriate diagnostic A 27-year-old man is evaluated in the emergency depart- test to perform next? ment after three episodes of loss of consciousness and falling down that started 2 weeks ago. He has no memory of the (A) Brain MRI events. The third event was witnessed by a coworker who (B) Depression screening noted that the patient cried out, had whole-body stiffening, (C) Electroencephalography and fell backward off his chair. While on the ground, the (D) Erythrocyte sedimentation rate patient’s whole body shook with synchronous limb jerking for 2 minutes. Afterward, the patient was very sleepy and (E) Montreal Cognitive Assessment had slow, snoring-like breathing. He remained in a state of impaired alertness for 8 minutes. He gradually awoke by the time he reached the emergency department 20 minutes Item 2 later, and now has recovered to normal. A 56-year-old woman is evaluated for recent difficulty On physical examination, vital signs are normal. All with tasks at her job as a paralegal, especially those other examination findings, including those from a neuro- involving short-term memory and multitasking. She has logic examination, are unremarkable. multiple sclerosis. She has had previous major depressive episodes, which are currently managed with venlafaxine. Which of the following is the most likely diagnosis?

E wn Item 1 in the toes, decreased sensation to pinprick below the nipple nn a line bilaterally, and a palpable bladder. Upper extremity w A 25-year-old man is evaluated for a 1-year history of head- n reflexes are 2+ in both biceps. Arm strength is normal. Gait aches. He recently returned from active military duty where = is ataxic. ae he sustained a mild traumatic brain injury (TBI). A CT scan we 172) obtained after injury was normal. Headaches, which started Which of the following is the most appropriate diagnostic soon after the TBI, typically occur three times weekly, last test to perform next? 24 hours, and are bifrontal, dull, and moderate in intensity. The patient also reports occasional photosensitivity accom- (A) CT myelography panying the headaches but has had no nausea, phonopho- (B) MRI of the brain bia, visual disturbances, or neurologic symptoms. He takes (C) MRI of the lumbosacral spine ibuprofen as needed to relieve the pain. (D) MRI of the thoracic spine On physical examination, vital signs and all other physical examination findings are normal. Results of standard laboratory studies are normal. Item 4 Which of the following is the most appropriate diagnostic A 27-year-old man is evaluated in the emergency depart- test to perform next? ment after three episodes of loss of consciousness and falling down that started 2 weeks ago. He has no memory of the (A) Brain MRI events. The third event was witnessed by a coworker who (B) Depression screening noted that the patient cried out, had whole-body stiffening, (C) Electroencephalography and fell backward off his chair. While on the ground, the (D) Erythrocyte sedimentation rate patient’s whole body shook with synchronous limb jerking for 2 minutes. Afterward, the patient was very sleepy and (E) Montreal Cognitive Assessment had slow, snoring-like breathing. He remained in a state of impaired alertness for 8 minutes. He gradually awoke by the time he reached the emergency department 20 minutes Item 2 later, and now has recovered to normal. A 56-year-old woman is evaluated for recent difficulty On physical examination, vital signs are normal. All with tasks at her job as a paralegal, especially those other examination findings, including those from a neuro- involving short-term memory and multitasking. She has logic examination, are unremarkable. multiple sclerosis. She has had previous major depressive episodes, which are currently managed with venlafaxine. Which of the following is the most likely diagnosis? Other medications are glatiramer acetate and a vitamin D (A) Generalized convulsive status epilepticus supplement. (B) Generalized tonic-clonic seizure On physical examination, vital signs are normal. On neurologic examination, the patient can recall only one of (C) Myoclonic seizure three objects at 3 minutes and recites “d-r-l-o-w” when (D) Psychogenic nonepileptic spells/events asked to spell “world” backward. Her score on the Patient Health Questionnaire 9 for depression is 4 because of difficulties with concentration Item 5 and sleep. A 25-year-old woman is evaluated for a 3-month history of

Other medications are glatiramer acetate and a vitamin D (A) Generalized convulsive status epilepticus supplement. (B) Generalized tonic-clonic seizure On physical examination, vital signs are normal. On neurologic examination, the patient can recall only one of (C) Myoclonic seizure three objects at 3 minutes and recites “d-r-l-o-w” when (D) Psychogenic nonepileptic spells/events asked to spell “world” backward. Her score on the Patient Health Questionnaire 9 for depression is 4 because of difficulties with concentration Item 5 and sleep. A 25-year-old woman is evaluated for a 3-month history of Which of the following is the most appropriate manage- headaches that previously occurred weekly but now occur ment? daily. She describes the headaches as holocranial, dull, and vice-like, and worsened by the Valsalva maneuver. The & ) Increase venlafaxine dose patient reports associated neck stiffness, intermittent visual Refer for cognitive rehabilitation blurring without diplopia, and nocturnal pulsatile tinnitus. Refer to psychiatry She has had no other symptoms. She also has a history of acne and vitamin D deficiency. Medications are topical Start memantine benzoyl peroxide, oral doxycycline, and vitamin D. BSCE Start methylphenidate On physical examination, vital signs and BMI are within normal limits. Neurologic examination reveals bilateral papilledema. Physical examination findings are Item 3 otherwise unremarkable. A 65-year-old man is evaluated in the emergency depart- Cerebral spinal fluid analysis shows an opening pres- ment for a 24-hour history of difficulty walking and inability sure of 280 mm H,O. Other laboratory studies are within to urinate. He has hypertension treated with amlodipine. normal limits. On physical examination, vital signs are normal. Mus- A contrast-enhanced brain MRI shows a partially cle strength is 4/5 for both hip flexors. Patellar reflexes are empty sella and widened optic nerve sheaths. Results of 3+ bilaterally. There are bilateral extensor plantar responses magnetic resonance venography are normal.

Which of the following is the most appropriate manage- headaches that previously occurred weekly but now occur ment? daily. She describes the headaches as holocranial, dull, and vice-like, and worsened by the Valsalva maneuver. The & ) Increase venlafaxine dose patient reports associated neck stiffness, intermittent visual Refer for cognitive rehabilitation blurring without diplopia, and nocturnal pulsatile tinnitus. Refer to psychiatry She has had no other symptoms. She also has a history of acne and vitamin D deficiency. Medications are topical Start memantine benzoyl peroxide, oral doxycycline, and vitamin D. BSCE Start methylphenidate On physical examination, vital signs and BMI are within normal limits. Neurologic examination reveals bilateral papilledema. Physical examination findings are Item 3 otherwise unremarkable. A 65-year-old man is evaluated in the emergency depart- Cerebral spinal fluid analysis shows an opening pres- ment for a 24-hour history of difficulty walking and inability sure of 280 mm H,O. Other laboratory studies are within to urinate. He has hypertension treated with amlodipine. normal limits. On physical examination, vital signs are normal. Mus- A contrast-enhanced brain MRI shows a partially cle strength is 4/5 for both hip flexors. Patellar reflexes are empty sella and widened optic nerve sheaths. Results of 3+ bilaterally. There are bilateral extensor plantar responses magnetic resonance venography are normal. 97

Seli-Assesonuent Test n 2 = Which of the following is the most appropriate next step in ground. He reports no pain but has mild tingling on the > treatment? lateral border of his left foot. He has no history of trauma or nn %) low back pain. He takes simvastatin for dyslipidemia. oO (A) Administer prednisone wn On physical examination, vital signs are normal. | @ (B) Administer topiramate BMI is 27. Weakness on dorsiflexion, eversion of the left = (C) Discontinue doxycycline foot, and extension of the left big toe are observed. There Coa} (D) Discontinue vitamin D is mild sensory loss on the lateral border of the left foot. o w Result of a straight-leg-raise test is negative. Plantar -r responses are flexor. The rest of the neurologic examina- Item 6 tion is normal. Serum creatine kinase level is normal. A 44-year-old man is evaluated for a 15-year history of An electromyogram reveals slow velocity of peroneal headaches that have become increasingly frequent over nerve conduction around the fibular neck. Other lower the past 6 months, now occurring between 15 and 20 days extremity motor and sensory nerve conduction measure- per month. He describes the headache pain as a global feel- ments and needle examination results are normal. ing of heaviness or tightness that seems to improve with exercise or distraction. Headaches are often preceded and Which of the following is the most appropriate next step in accompanied by neck tightness. He has had no photopho- management? bia, phonophobia, nausea, aura, or neurologic symptoms. Acetaminophen, relaxation training, and cognitive behav- (A) Avoid leg crossing ioral therapy have been ineffective. NSAIDs caused gastro- (B) Avoid prolonged standing esophageal reflux symptoms. (C) Discontinue simvastatin All physical examination findings, including vital signs, (D) Pursue weight loss are normal.

Seli-Assesonuent Test n 2 = Which of the following is the most appropriate next step in ground. He reports no pain but has mild tingling on the > treatment? lateral border of his left foot. He has no history of trauma or nn %) low back pain. He takes simvastatin for dyslipidemia. oO (A) Administer prednisone wn On physical examination, vital signs are normal. | @ (B) Administer topiramate BMI is 27. Weakness on dorsiflexion, eversion of the left = (C) Discontinue doxycycline foot, and extension of the left big toe are observed. There Coa} (D) Discontinue vitamin D is mild sensory loss on the lateral border of the left foot. o w Result of a straight-leg-raise test is negative. Plantar -r responses are flexor. The rest of the neurologic examina- Item 6 tion is normal. Serum creatine kinase level is normal. A 44-year-old man is evaluated for a 15-year history of An electromyogram reveals slow velocity of peroneal headaches that have become increasingly frequent over nerve conduction around the fibular neck. Other lower the past 6 months, now occurring between 15 and 20 days extremity motor and sensory nerve conduction measure- per month. He describes the headache pain as a global feel- ments and needle examination results are normal. ing of heaviness or tightness that seems to improve with exercise or distraction. Headaches are often preceded and Which of the following is the most appropriate next step in accompanied by neck tightness. He has had no photopho- management? bia, phonophobia, nausea, aura, or neurologic symptoms. Acetaminophen, relaxation training, and cognitive behav- (A) Avoid leg crossing ioral therapy have been ineffective. NSAIDs caused gastro- (B) Avoid prolonged standing esophageal reflux symptoms. (C) Discontinue simvastatin All physical examination findings, including vital signs, (D) Pursue weight loss are normal. Which of the following is the most appropriate preventive treatment? Item 9 A 26-year-old man is evaluated for spells that began ( ) Amitriptyline > 4 months ago and occur twice monthly. He has no mem- (B Butalbital ory of the episodes, but family members report that he ( Cyclobenzaprine suddenly stops talking and sometimes has twitching of the ( Gos Onabotulinum toxin A right arm. He will not look at them, will not speak, and does (E a Topiramate not follow verbal commands. Family members also report stiffness and clenching of his right hand toward the end of an episode, which typically lasts 30 to 90 seconds. After Item 7 he regains speech and the twitching stops, he usually has

Which of the following is the most appropriate preventive treatment? Item 9 A 26-year-old man is evaluated for spells that began ( ) Amitriptyline > 4 months ago and occur twice monthly. He has no mem- (B Butalbital ory of the episodes, but family members report that he ( Cyclobenzaprine suddenly stops talking and sometimes has twitching of the ( Gos Onabotulinum toxin A right arm. He will not look at them, will not speak, and does (E a Topiramate not follow verbal commands. Family members also report stiffness and clenching of his right hand toward the end of an episode, which typically lasts 30 to 90 seconds. After Item 7 he regains speech and the twitching stops, he usually has A 38-year-old woman is hospitalized with a subarach slurred speech, right-arm weakness, and mild confusion noid hemorrhage. The patient’s medical history is oth for up to 30 minutes. He has no other medical problems and erwise unremarkable, and she takes no medications or takes no medications. illicit drugs. On physical examination, vital signs are normal. All On physical examination, blood pressure is 142/82 mm other examination findings, including those from a neuro- Hg; the remaining vital signs are normal. Nuchal rigidity logic examination, are unremarkable.

illicit drugs. On physical examination, vital signs are normal. All On physical examination, blood pressure is 142/82 mm other examination findings, including those from a neuro- Hg; the remaining vital signs are normal. Nuchal rigidity logic examination, are unremarkable. is present. The right pupil is 2 mm larger than the left; Which of the following is the most likely diagnosis? both pupils are reactive to light. Mental status examination shows somnolence but is otherwise normal. (A) Focal aware seizures CT scan of the head shows subarachnoid hemorrhage (B) Focal impaired awareness seizures in the basal cisterns with intraventricular hemorrhage. Dig- (C) Generalized absence seizures ital subtraction angiogram shows an 8-mm aneurysm in the (D) Generalized myoclonic seizures right posterior communicating artery; during angiography, this aneurysm was successfully treated with coiling. Which of the following is the most appropriate treatment? Item 10

is present. The right pupil is 2 mm larger than the left; Which of the following is the most likely diagnosis? both pupils are reactive to light. Mental status examination shows somnolence but is otherwise normal. (A) Focal aware seizures CT scan of the head shows subarachnoid hemorrhage (B) Focal impaired awareness seizures in the basal cisterns with intraventricular hemorrhage. Dig- (C) Generalized absence seizures ital subtraction angiogram shows an 8-mm aneurysm in the (D) Generalized myoclonic seizures right posterior communicating artery; during angiography, this aneurysm was successfully treated with coiling. Which of the following is the most appropriate treatment? Item 10 (A) Intravenous dexamethasone A 35-year-old woman is evaluated for a 5-year history of right- or occasionally left-sided throbbing headache (B) Intravenous labetalol with photophobia, phonophobia, and nausea. Recently, (C) Oral nimodipine her headaches have been associated with right-arm tin- (D) Oral verapamil gling lasting 30 minutes. The headaches occur twice a month and last 6 to 8 hours each. The patient is other- wise asymptomatic, and she has had no other neurologic Item 8 symptoms. A 52-year-old man is evaluated for 4-week history of left On physical examination, vital signs are normal. All lower extremity weakness that began with tripping on the other examination findings, including those from a neuro- left foot. He now must lift his knee to clear his foot off the logic examination, are unremarkable. 98

Self-Assessment Test ot CH MRI of the brain with contrast shows a homogenously Item 13 — P=) enhancing, dural-based lesion that has well-demarcated A 78-year-old man is evaluated for a 4-month history of i=] <7) borders and appears to be outside the brain parenchyma in increasing forgetfulness. He lives alone and maintains inde- [= the right hemisphere. No mass effect, hemorrhage, or cere- pendence in all of his instrumental and basic activities of wn wn <5) bral edema is noted. daily living. His daughter reports that he forgets appoint 77) rn Which of the following is the most appropriate next step in ments and his home is less organized than before with =< management? more clutter, including unopened bills on the dining room = 4) table. He does not drink alcohol. There is no history of 172) (A) Brain biopsy high-risk sexual behavior. He is otherwise well and takes (B) Dexamethasone no medications.

Which of the following is the most appropriate next step in ments and his home is less organized than before with =< management? more clutter, including unopened bills on the dining room = 4) table. He does not drink alcohol. There is no history of 172) (A) Brain biopsy high-risk sexual behavior. He is otherwise well and takes (B) Dexamethasone no medications. C) Levetiracetam On physical examination, vital signs. general screen- ing, and neurologic examination findings are normal. as ) Serial brain MRI He can recall two of three items on the Mini Cog test E) Surgical resection — but cannot correctly draw a clock face.

C) Levetiracetam On physical examination, vital signs. general screen- ing, and neurologic examination findings are normal. as ) Serial brain MRI He can recall two of three items on the Mini Cog test E) Surgical resection — but cannot correctly draw a clock face. Which of the following is the most appropriate test to per- Item 11 form next? A 70-year-old woman is hospitalized for progression of Cerebrospinal fluid analysis for tau protein (A) cognitive impairment to dementia and ataxia over the past (B) Depression screening 6 months. She has developed myoclonus, gait problems, and interruption of circadian rhythm. (C) Genetic testing On physical examination, vital signs are normal. She is (D) Serum VDRL testing alert but has difficulty with orientation to place and time. Cranial nerves and motor strength are normal. There are occasional myoclonic jerks of the trunk. Sensation is intact Item 14 throughout; cerebellar ataxia is seen on finger-nose-finger, A 47-year-old man is evaluated in the emergency depart- heel-to-shin, and gait testing. Deep tendon reflexes are ment for a prolonged seizure. According to the family, the increased throughout. initial seizure lasted for 3 minutes and stopped, but the Diffusion-weighted MRI of the brain shows hyperin- patient did not regain consciousness and a second gener- tensity in the basal ganglia and several regions of the cortex. alized tonic-clonic seizure started within 5 minutes and A lumbar puncture is performed. has continued. The patient received 4 mg intravenous lora- Which of the following is the most appropriate diagnostic zepam in the ambulance on the way to the hospital. In test to perform on the cerebrospinal fluid? the emergency department, the seizure continues and he receives an additional 4 mg of intravenous lorazepam fol- (A) 14-3-3 Protein assay lowed by another 2 mg 10 minutes later without benefit. (B) Polymerase chain reaction for herpes simplex virus The patient has a history of generalized tonic-clonic seizures (C) Real-time quaking-induced conversion assay treated with levetiracetam.

Which of the following is the most appropriate test to per- Item 11 form next? A 70-year-old woman is hospitalized for progression of Cerebrospinal fluid analysis for tau protein (A) cognitive impairment to dementia and ataxia over the past (B) Depression screening 6 months. She has developed myoclonus, gait problems, and interruption of circadian rhythm. (C) Genetic testing On physical examination, vital signs are normal. She is (D) Serum VDRL testing alert but has difficulty with orientation to place and time. Cranial nerves and motor strength are normal. There are occasional myoclonic jerks of the trunk. Sensation is intact Item 14 throughout; cerebellar ataxia is seen on finger-nose-finger, A 47-year-old man is evaluated in the emergency depart- heel-to-shin, and gait testing. Deep tendon reflexes are ment for a prolonged seizure. According to the family, the increased throughout. initial seizure lasted for 3 minutes and stopped, but the Diffusion-weighted MRI of the brain shows hyperin- patient did not regain consciousness and a second gener- tensity in the basal ganglia and several regions of the cortex. alized tonic-clonic seizure started within 5 minutes and A lumbar puncture is performed. has continued. The patient received 4 mg intravenous lora- Which of the following is the most appropriate diagnostic zepam in the ambulance on the way to the hospital. In test to perform on the cerebrospinal fluid? the emergency department, the seizure continues and he receives an additional 4 mg of intravenous lorazepam fol- (A) 14-3-3 Protein assay lowed by another 2 mg 10 minutes later without benefit. (B) Polymerase chain reaction for herpes simplex virus The patient has a history of generalized tonic-clonic seizures (C) Real-time quaking-induced conversion assay treated with levetiracetam. (D) Total tau protein measurement On physical examination, blood pressure is 147/92 mm Hg, pulse rate is 122/min, and respiration rate is 18/min. Oxygen saturation with the patient breathing ambient air Item 12 is 90%. Pupils are reactive. Clonic seizure activity persists. Twenty minutes have elapsed since admission to the A 48-year-old man is evaluated for a 25-year history of emergency department. The patient is intubated, an intra- headaches that have become increasingly frequent. Head- venous line is placed, a blood specimen is obtained, and aches previously occurred four to five times per month fingerstick glucose is obtained (126 mg/dL [7.0 mmol/L]). but now occur 16 to 20 times per month and last 12 to 24 hours. The headaches are bilateral, throbbing, moderate Which of the following is the most appropriate additional in intensity, aggravated by physical activity, and accom- management? panied by photophobia and phonophobia. He has had no other associated symptoms. The patient takes amitriptyline (A) Head CT and sumatriptan for the headaches and has been taking (B) Immediate continuous electroencephalography sumatriptan four to five times weekly for the past 3 months. (C) Intravenous fosphenytoin Physical examination findings, including vital signs, (D) Rectal administration of diazepam are all normal. A brain MRI with contrast is normal.

(D) Total tau protein measurement On physical examination, blood pressure is 147/92 mm Hg, pulse rate is 122/min, and respiration rate is 18/min. Oxygen saturation with the patient breathing ambient air Item 12 is 90%. Pupils are reactive. Clonic seizure activity persists. Twenty minutes have elapsed since admission to the A 48-year-old man is evaluated for a 25-year history of emergency department. The patient is intubated, an intra- headaches that have become increasingly frequent. Head- venous line is placed, a blood specimen is obtained, and aches previously occurred four to five times per month fingerstick glucose is obtained (126 mg/dL [7.0 mmol/L]). but now occur 16 to 20 times per month and last 12 to 24 hours. The headaches are bilateral, throbbing, moderate Which of the following is the most appropriate additional in intensity, aggravated by physical activity, and accom- management? panied by photophobia and phonophobia. He has had no other associated symptoms. The patient takes amitriptyline (A) Head CT and sumatriptan for the headaches and has been taking (B) Immediate continuous electroencephalography sumatriptan four to five times weekly for the past 3 months. (C) Intravenous fosphenytoin Physical examination findings, including vital signs, (D) Rectal administration of diazepam are all normal. A brain MRI with contrast is normal. Which of the following is the most appropriate next step in Item 15 treatment? A 29-year-old man is evaluated in the emergency depart- ment for pain and discomfort with movement of his left eye (A) Begin butalbital that began yesterday morning. He awoke this morning with (B) Begin verapamil blurry vision and a dark spot in the center of the visual field (C) Discontinue amitriptyline of his left eye. He has no other medical problems and takes (D) Discontinue sumatriptan no medications.

Which of the following is the most appropriate next step in Item 15 treatment? A 29-year-old man is evaluated in the emergency depart- ment for pain and discomfort with movement of his left eye (A) Begin butalbital that began yesterday morning. He awoke this morning with (B) Begin verapamil blurry vision and a dark spot in the center of the visual field (C) Discontinue amitriptyline of his left eye. He has no other medical problems and takes (D) Discontinue sumatriptan no medications. 99

Self-Assessment Test rn @ — On physical examination, vital signs are normal. Fun- findings, plantar response, and deep tendon reflexes are LS duscopic examination is normal. Pupils are reactive, with unremarkable. wn m) CONT. an afferent pupillary defect in the left eye. Visual acuity is oO wn Which of the following is the most likely diagnosis? my 20/20 in the right eye and 20/100 in the left eye with a cen- 3 tral scotoma. There is no proptosis, conjunctival erythema, ( A) Amyotrophic lateral sclerosis @ or pain on palpation of the orbit. = Coal ( B) Corticobasal degeneration Results of laboratory studies show a normal leukocyte o n count and erythrocyte sedimentation rate. ( C) Multiple system atrophy or ( D) Parkinson disease Which of the following is the most likely diagnosis? (E) Progressive supranuclear palsy (A) Central retinal artery occlusion (B) Giant cell arteritis Item 18 (C) Optic neuritis A 61-year-old man is evaluated for pain and weakness in his (D) Orbital cellulitis hands and feet. He has a history of bilateral carpal tunnel syndrome that improved after surgery 1 year ago, but symp- toms returned 2 months ago. He has noted progressive pain Item 16 and numbness below his knees and diminished sweating. A 60-year-old man is evaluated for a 3-year history of He also has episodic light-headedness on standing, dry eyes, gradual, progressive decline in his cognition, behavior, heart failure with preserved ejection fraction, left ventricu- and motor skills. Both he and his wife have noticed these lar hypertrophy by echocardiography, and erectile dysfunc- changes. He becomes agitated easily, and his wife reports tion. Current medications are amlodipine, metoclopramide, that his personality is much more subdued than it used to artificial tears, and sildenafil. be. He no longer enjoys socializing. Memory loss is not a On physical examination, vital signs are normal. The prominent symptom. The patient played professional foot- presence of orthostatic hypotension is confirmed. Pupils ball for 5 years after college graduation. He has hyperten- react sluggishly to light. Extraocular movements are pre- sion and hyperlipidemia. His medications are lisinopril, served. Weakness in right thumb abductor and bilateral hydrochlorothiazide, and atorvastatin. foot dorsiflexors is observed. Sensory loss to temperature On physical examination, vital signs are normal. There and pain below the knees and elbows are noted. Vibration is is slightly increased tone in the bilateral upper extremities. preserved at the ankles. Deep tendon reflexes are preserved He has a slow, shuffling gait. in upper extremities and diminished in lower extremities. MRI of the brain reveals global atrophy. Fasting plasma glucose level, glucose tolerance test Neuropsychological testing shows marked cognitive results, serum and urine protein electrophoresis and immu- slowing and a disorganized thought process. nofixation results, rapid plasma reagin results, and anti-Ro and anti-La antibody titers are normal. Which of the following is the most likely diagnosis? ECG is remarkable for low voltages. (A) Alzheimer disease Which of the following is the most appropriate diagnostic (B) Behavioral-variant frontotemporal dementia test to perform next? (C) Traumatic encephalopathy syndrome (A) Abdominal fat pad biopsy (D) Vascular cognitive impairment (B) Anti-muscle-specific kinase antibody measurement (C) Myotonic dystrophy gene test Item 17 (D) Serum GQ1B antibody measurement

rn @ — On physical examination, vital signs are normal. Fun- findings, plantar response, and deep tendon reflexes are LS duscopic examination is normal. Pupils are reactive, with unremarkable. wn m) CONT. an afferent pupillary defect in the left eye. Visual acuity is oO wn Which of the following is the most likely diagnosis? my 20/20 in the right eye and 20/100 in the left eye with a cen- 3 tral scotoma. There is no proptosis, conjunctival erythema, ( A) Amyotrophic lateral sclerosis @ or pain on palpation of the orbit. = Coal ( B) Corticobasal degeneration Results of laboratory studies show a normal leukocyte o n count and erythrocyte sedimentation rate. ( C) Multiple system atrophy or ( D) Parkinson disease Which of the following is the most likely diagnosis? (E) Progressive supranuclear palsy (A) Central retinal artery occlusion (B) Giant cell arteritis Item 18 (C) Optic neuritis A 61-year-old man is evaluated for pain and weakness in his (D) Orbital cellulitis hands and feet. He has a history of bilateral carpal tunnel syndrome that improved after surgery 1 year ago, but symp- toms returned 2 months ago. He has noted progressive pain Item 16 and numbness below his knees and diminished sweating. A 60-year-old man is evaluated for a 3-year history of He also has episodic light-headedness on standing, dry eyes, gradual, progressive decline in his cognition, behavior, heart failure with preserved ejection fraction, left ventricu- and motor skills. Both he and his wife have noticed these lar hypertrophy by echocardiography, and erectile dysfunc- changes. He becomes agitated easily, and his wife reports tion. Current medications are amlodipine, metoclopramide, that his personality is much more subdued than it used to artificial tears, and sildenafil. be. He no longer enjoys socializing. Memory loss is not a On physical examination, vital signs are normal. The prominent symptom. The patient played professional foot- presence of orthostatic hypotension is confirmed. Pupils ball for 5 years after college graduation. He has hyperten- react sluggishly to light. Extraocular movements are pre- sion and hyperlipidemia. His medications are lisinopril, served. Weakness in right thumb abductor and bilateral hydrochlorothiazide, and atorvastatin. foot dorsiflexors is observed. Sensory loss to temperature On physical examination, vital signs are normal. There and pain below the knees and elbows are noted. Vibration is is slightly increased tone in the bilateral upper extremities. preserved at the ankles. Deep tendon reflexes are preserved He has a slow, shuffling gait. in upper extremities and diminished in lower extremities. MRI of the brain reveals global atrophy. Fasting plasma glucose level, glucose tolerance test Neuropsychological testing shows marked cognitive results, serum and urine protein electrophoresis and immu- slowing and a disorganized thought process. nofixation results, rapid plasma reagin results, and anti-Ro and anti-La antibody titers are normal. Which of the following is the most likely diagnosis? ECG is remarkable for low voltages. (A) Alzheimer disease Which of the following is the most appropriate diagnostic (B) Behavioral-variant frontotemporal dementia test to perform next? (C) Traumatic encephalopathy syndrome (A) Abdominal fat pad biopsy (D) Vascular cognitive impairment (B) Anti-muscle-specific kinase antibody measurement (C) Myotonic dystrophy gene test Item 17 (D) Serum GQ1B antibody measurement A 73-year-old woman is evaluated for progressive left arm weakness. One year earlier, she noted difficulty with fine movements of her left hand. Within a few months, her Item 19 entire left upper extremity became stiff and slow, such that A 29-year-old woman is evaluated in the emergency depart she now cannot use this limb. Her left leg has also become ment for loss of consciousness. This morning her mother rigid. She notes brief jerky movements of her left arm. She found her unconscious in bed, drooling, and shaking all her takes no medication. limbs. The patient was unresponsive for 3 to 4 minutes, and On physical examination, vital signs are normal. Lan- was confused for about 5 minutes after regaining conscious- guage and memory are intact, but she speaks slowly and ness. By the time she reached the emergency department, has difficulty with multistep commands. Results of cra- she had recovered completely. She has had no recent illness, nial nerve testing, including extraocular movements, are including infection, head trauma, or history of prior similar normal. Muscle strength is preserved throughout. Her left episodes. upper and lower extremities are rigid and slow. Her left She has no history of alcohol or illicit drug use. She has hand is forced into a bent position and cannot be opened no other medical problems and her only medication is a by the examiner. She needs support during ambulation combined oral contraceptive. because of stiffness of her left leg. Her sensory perception is On physical examination, vital signs are normal. All preserved, but she cannot identify an object placed into her other examination findings, including those from a neuro- left hand when her eyes are closed. Cerebellar examination logic examination, are unremarkable.

A 73-year-old woman is evaluated for progressive left arm weakness. One year earlier, she noted difficulty with fine movements of her left hand. Within a few months, her Item 19 entire left upper extremity became stiff and slow, such that A 29-year-old woman is evaluated in the emergency depart she now cannot use this limb. Her left leg has also become ment for loss of consciousness. This morning her mother rigid. She notes brief jerky movements of her left arm. She found her unconscious in bed, drooling, and shaking all her takes no medication. limbs. The patient was unresponsive for 3 to 4 minutes, and On physical examination, vital signs are normal. Lan- was confused for about 5 minutes after regaining conscious- guage and memory are intact, but she speaks slowly and ness. By the time she reached the emergency department, has difficulty with multistep commands. Results of cra- she had recovered completely. She has had no recent illness, nial nerve testing, including extraocular movements, are including infection, head trauma, or history of prior similar normal. Muscle strength is preserved throughout. Her left episodes. upper and lower extremities are rigid and slow. Her left She has no history of alcohol or illicit drug use. She has hand is forced into a bent position and cannot be opened no other medical problems and her only medication is a by the examiner. She needs support during ambulation combined oral contraceptive. because of stiffness of her left leg. Her sensory perception is On physical examination, vital signs are normal. All preserved, but she cannot identify an object placed into her other examination findings, including those from a neuro- left hand when her eyes are closed. Cerebellar examination logic examination, are unremarkable. 100

Self-Assessment Test od Results of a comprehensive metabolic profile and toxi- (C) Functional disorder ® — hes! cology screen are normal. A CT of the head without contrast (D) Myoclonus =| E CONT. in the emergency department is normal. An electroenceph- (E) Tic alogram has been scheduled. wn wn wy Which of the following is the most appropriate management? wn Item 22 wn (A) Brain MRI without contrast = A 66-year-old woman is evaluated for a 6-month history of rl a (B) CT of the head with contrast slowed gait and difficulty lifting her feet off of the ground ~”

(A) Brain MRI without contrast = A 66-year-old woman is evaluated for a 6-month history of rl a (B) CT of the head with contrast slowed gait and difficulty lifting her feet off of the ground ~” (C) Levetiracetam when she first starts to walk. She becomes incontinent (D) Lumbar puncture without feeling the urge to urinate. Her family reports that she seems to be thinking more slowly. On physical examination, vital signs are normal. Gait is Item 20 magnetic, with hesitancy and shuffling. MRI of the brain shows ventriculomegaly in the lateral A 20-year-old woman is evaluated for intermittent double ventricles and third ventricle. There is no obstruction in the vision that occurs when she looks from the corner of her cerebral aqueduct. eyes and disappears when she covers one eye. She also reports fluctuating weakness and frequent nausea and diar- Which of the following is the most appropriate next step in rhea. She has had a history of exercise intolerance and management? intermittent muscle cramps since middle school. She also has postural orthostatic tachycardia syndrome, irritable (A) High-volume cerebrospinal fluid removal bowel syndrome, and fibromyalgia. Her sister, mother and (B) Lumboperitoneal shunt procedure maternal aunt have similar muscle, neurologic, and mul- (C) Placement of a temporary lumbar drain tiorgan symptoms but her father and two male siblings do (D) Ventriculoperitoneal shunt procedure not. Current medications are propranolol and duloxetine. On physical examination, vital signs are normal. BMI is 20. There is bilateral impairment of ocular adduction along Item 23 with bilateral ptosis. Lower facial muscles show weakness. A 21-year-old woman comes to the office requesting clear- Muscle tone is reduced, but deep tendon reflexes are pre- ance to resume playing collegiate soccer. Two weeks ago, served. Proximal weakness is noted in all extremities. There she sustained a mild traumatic brain injury in a head-to- is no delayed relaxation with grip or percussion. Mental head collision during a match and, for the next 3 days, had status, sensory examination, and coordination are intact. episodic dizziness and recurrent headaches lasting minutes Electromyogram shows myopathic changes. to hours; symptoms resolved spontaneously with a com- Which of the following is the most likely diagnosis? bination of physical and cognitive rest. She resumed nor- mal activities and has been free of symptoms for the past (A) Acid maltase deficiency 10 days. She has taken no medication. Neuroimaging was (B) Becker muscular dystrophy not performed at the time of injury. (C) Inclusion body myositis Physical examination findings, including vital signs (D) McArdle disease and neurologic examination results, are normal.

(C) Levetiracetam when she first starts to walk. She becomes incontinent (D) Lumbar puncture without feeling the urge to urinate. Her family reports that she seems to be thinking more slowly. On physical examination, vital signs are normal. Gait is Item 20 magnetic, with hesitancy and shuffling. MRI of the brain shows ventriculomegaly in the lateral A 20-year-old woman is evaluated for intermittent double ventricles and third ventricle. There is no obstruction in the vision that occurs when she looks from the corner of her cerebral aqueduct. eyes and disappears when she covers one eye. She also reports fluctuating weakness and frequent nausea and diar- Which of the following is the most appropriate next step in rhea. She has had a history of exercise intolerance and management? intermittent muscle cramps since middle school. She also has postural orthostatic tachycardia syndrome, irritable (A) High-volume cerebrospinal fluid removal bowel syndrome, and fibromyalgia. Her sister, mother and (B) Lumboperitoneal shunt procedure maternal aunt have similar muscle, neurologic, and mul- (C) Placement of a temporary lumbar drain tiorgan symptoms but her father and two male siblings do (D) Ventriculoperitoneal shunt procedure not. Current medications are propranolol and duloxetine. On physical examination, vital signs are normal. BMI is 20. There is bilateral impairment of ocular adduction along Item 23 with bilateral ptosis. Lower facial muscles show weakness. A 21-year-old woman comes to the office requesting clear- Muscle tone is reduced, but deep tendon reflexes are pre- ance to resume playing collegiate soccer. Two weeks ago, served. Proximal weakness is noted in all extremities. There she sustained a mild traumatic brain injury in a head-to- is no delayed relaxation with grip or percussion. Mental head collision during a match and, for the next 3 days, had status, sensory examination, and coordination are intact. episodic dizziness and recurrent headaches lasting minutes Electromyogram shows myopathic changes. to hours; symptoms resolved spontaneously with a com- Which of the following is the most likely diagnosis? bination of physical and cognitive rest. She resumed nor- mal activities and has been free of symptoms for the past (A) Acid maltase deficiency 10 days. She has taken no medication. Neuroimaging was (B) Becker muscular dystrophy not performed at the time of injury. (C) Inclusion body myositis Physical examination findings, including vital signs (D) McArdle disease and neurologic examination results, are normal. (E) Mitochondrial myopathy Which of the following is the most appropriate next step in management?

(C) Levetiracetam when she first starts to walk. She becomes incontinent (D) Lumbar puncture without feeling the urge to urinate. Her family reports that she seems to be thinking more slowly. On physical examination, vital signs are normal. Gait is Item 20 magnetic, with hesitancy and shuffling. MRI of the brain shows ventriculomegaly in the lateral A 20-year-old woman is evaluated for intermittent double ventricles and third ventricle. There is no obstruction in the vision that occurs when she looks from the corner of her cerebral aqueduct. eyes and disappears when she covers one eye. She also reports fluctuating weakness and frequent nausea and diar- Which of the following is the most appropriate next step in rhea. She has had a history of exercise intolerance and management? intermittent muscle cramps since middle school. She also has postural orthostatic tachycardia syndrome, irritable (A) High-volume cerebrospinal fluid removal bowel syndrome, and fibromyalgia. Her sister, mother and (B) Lumboperitoneal shunt procedure maternal aunt have similar muscle, neurologic, and mul- (C) Placement of a temporary lumbar drain tiorgan symptoms but her father and two male siblings do (D) Ventriculoperitoneal shunt procedure not. Current medications are propranolol and duloxetine. On physical examination, vital signs are normal. BMI is 20. There is bilateral impairment of ocular adduction along Item 23 with bilateral ptosis. Lower facial muscles show weakness. A 21-year-old woman comes to the office requesting clear- Muscle tone is reduced, but deep tendon reflexes are pre- ance to resume playing collegiate soccer. Two weeks ago, served. Proximal weakness is noted in all extremities. There she sustained a mild traumatic brain injury in a head-to- is no delayed relaxation with grip or percussion. Mental head collision during a match and, for the next 3 days, had status, sensory examination, and coordination are intact. episodic dizziness and recurrent headaches lasting minutes Electromyogram shows myopathic changes. to hours; symptoms resolved spontaneously with a com- Which of the following is the most likely diagnosis? bination of physical and cognitive rest. She resumed nor- mal activities and has been free of symptoms for the past (A) Acid maltase deficiency 10 days. She has taken no medication. Neuroimaging was (B) Becker muscular dystrophy not performed at the time of injury. (C) Inclusion body myositis Physical examination findings, including vital signs (D) McArdle disease and neurologic examination results, are normal. (E) Mitochondrial myopathy Which of the following is the most appropriate next step in management? Item 21 (A) Brain MRI

(C) Levetiracetam when she first starts to walk. She becomes incontinent (D) Lumbar puncture without feeling the urge to urinate. Her family reports that she seems to be thinking more slowly. On physical examination, vital signs are normal. Gait is Item 20 magnetic, with hesitancy and shuffling. MRI of the brain shows ventriculomegaly in the lateral A 20-year-old woman is evaluated for intermittent double ventricles and third ventricle. There is no obstruction in the vision that occurs when she looks from the corner of her cerebral aqueduct. eyes and disappears when she covers one eye. She also reports fluctuating weakness and frequent nausea and diar- Which of the following is the most appropriate next step in rhea. She has had a history of exercise intolerance and management? intermittent muscle cramps since middle school. She also has postural orthostatic tachycardia syndrome, irritable (A) High-volume cerebrospinal fluid removal bowel syndrome, and fibromyalgia. Her sister, mother and (B) Lumboperitoneal shunt procedure maternal aunt have similar muscle, neurologic, and mul- (C) Placement of a temporary lumbar drain tiorgan symptoms but her father and two male siblings do (D) Ventriculoperitoneal shunt procedure not. Current medications are propranolol and duloxetine. On physical examination, vital signs are normal. BMI is 20. There is bilateral impairment of ocular adduction along Item 23 with bilateral ptosis. Lower facial muscles show weakness. A 21-year-old woman comes to the office requesting clear- Muscle tone is reduced, but deep tendon reflexes are pre- ance to resume playing collegiate soccer. Two weeks ago, served. Proximal weakness is noted in all extremities. There she sustained a mild traumatic brain injury in a head-to- is no delayed relaxation with grip or percussion. Mental head collision during a match and, for the next 3 days, had status, sensory examination, and coordination are intact. episodic dizziness and recurrent headaches lasting minutes Electromyogram shows myopathic changes. to hours; symptoms resolved spontaneously with a com- Which of the following is the most likely diagnosis? bination of physical and cognitive rest. She resumed nor- mal activities and has been free of symptoms for the past (A) Acid maltase deficiency 10 days. She has taken no medication. Neuroimaging was (B) Becker muscular dystrophy not performed at the time of injury. (C) Inclusion body myositis Physical examination findings, including vital signs (D) McArdle disease and neurologic examination results, are normal. (E) Mitochondrial myopathy Which of the following is the most appropriate next step in management? Item 21 (A) Brain MRI A 56-year-old woman is evaluated for changes in her voice. (B) Clearance to return to play Over the past year her voice has become strained and stran- (C) SS Head CT gled, and she has had problems with expressing certain (D) ee, Neuropsychological testing consonants. She reports no pain or shortness of breath but (E) ot Vestibular therapy has intermittent tightness in her throat. She notes no other symptoms or medical problems and takes no medications. On physical examination, vital signs are normal. Her Item 24 voice is hoarse, and she speaks with a strained and pres- A 55-year-old man is evaluated in the hospital for paralysis sured voice disrupted by sudden pauses, but she can sing present on awakening from anesthesia after surgical repair without any problems. The remainder of the neurologic ofan aortic dissection. He reports midthoracic back pain. He examination is normal. had been well without evidence of infection before surgery. Video laryngoscopy reveals slow, sustained, and non- Other medical problems include hypertension and dyslip- rhythmic pulling of vocal cords toward midline with inter- idemia. Current medications are atorvastatin, hydrochloro- mittent relaxations. thiazide, and losartan. Which of the following is the most likely diagnosis? On physical examination, vital signs are normal. Mus- cle tone is flaccid in the legs and normal in the arms. Muscle (A) Chorea strength is normal in both arms and no movement in both (B) Dystonia legs. Reflexes are 2+ in arms and 0 in legs. Sensation to

A 56-year-old woman is evaluated for changes in her voice. (B) Clearance to return to play Over the past year her voice has become strained and stran- (C) SS Head CT gled, and she has had problems with expressing certain (D) ee, Neuropsychological testing consonants. She reports no pain or shortness of breath but (E) ot Vestibular therapy has intermittent tightness in her throat. She notes no other symptoms or medical problems and takes no medications. On physical examination, vital signs are normal. Her Item 24 voice is hoarse, and she speaks with a strained and pres- A 55-year-old man is evaluated in the hospital for paralysis sured voice disrupted by sudden pauses, but she can sing present on awakening from anesthesia after surgical repair without any problems. The remainder of the neurologic ofan aortic dissection. He reports midthoracic back pain. He examination is normal. had been well without evidence of infection before surgery. Video laryngoscopy reveals slow, sustained, and non- Other medical problems include hypertension and dyslip- rhythmic pulling of vocal cords toward midline with inter- idemia. Current medications are atorvastatin, hydrochloro- mittent relaxations. thiazide, and losartan. Which of the following is the most likely diagnosis? On physical examination, vital signs are normal. Mus- cle tone is flaccid in the legs and normal in the arms. Muscle (A) Chorea strength is normal in both arms and no movement in both (B) Dystonia legs. Reflexes are 2+ in arms and 0 in legs. Sensation to 101

Self-Assessment Test — wn © ay pinprick is mildly impaired in the legs, and vibratory and examination is positive for dysphagia. The patient is incon > position sensation are intact. tinent of urine. wv wn CONT. © CT scan of the head shows an acute intracerebral hem wn Which of the following is the most likely diagnosis? orrhage in the right thalamus, which has remained stable 3 © (A) Guillain-Barré syndrome at 24 hours. = rr (B) Idiopathic transverse myelitis Which of the following is the most appropriate initial man- =i © (C) Lumbar disk herniation agement? wv - (D) Spinal cord infarction (A) Early feeding (B) Placement of bladder catheter Item 25 (C) Supplemental oxygen

= rr (B) Idiopathic transverse myelitis Which of the following is the most appropriate initial man- =i © (C) Lumbar disk herniation agement? wv - (D) Spinal cord infarction (A) Early feeding (B) Placement of bladder catheter Item 25 (C) Supplemental oxygen A 60-year-old woman comes to the office to discuss the best (D) Venous thromboembolism prophylaxis strategy to prevent cognitive impairment. She has no medical problems and takes no medications. She does not use tobacco, Item 28 has 1 to 2 alcoholic drinks per week, and is sedentary. On physical examination, vital signs and other findings A 78-year-old woman is evaluated for a 1-year history of are unremarkable. progressively worsening memory impairment. She can no Results of cognitive screening testing are normal. longer handle her finances, as she doesn’t remember how to fill out checks properly. She lives with her spouse and Which of the following is the most effective preventive is independent in all other basic activities of daily living. measure? On physical examination, vital signs are normal. Mon- treal Cognitive Assessment score is 21/30, with difficulty (A) Cognitive training copying the cube, recalling the five words, and naming the (B) Donepezil day, date, and month noted. (C) Physical exercise training Results of laboratory evaluation are normal. (D) Vitamin E supplementation Which of the following is the most appropriate imaging test to perform next? Item 26 (A) Amyloid PET scan A 39-year-old woman is evaluated for a 3-month history (B) Fluorodeoxyglucose PET scan of low energy, lack of interest in activities, and poor sleep. (C) Head CT with contrast She has relapsing-remitting multiple sclerosis and a history of vitamin D deficiency. Medications are glatiramer acetate (D) MRI of the brain without contrast and a vitamin D supplement. On physical examination, vital signs are normal. All Item 29 other examination findings, including those from a neuro- logic examination, are unremarkable. A 55-year-old man with relapsing-remitting multiple scle- Two weeks ago, a routine MRI of the brain showeda sta- rosis is evaluated for a slow, labored gait that began after a ble number of T2 lesions, and none of these lesions demon- relapse 1 year ago. The symptom has not worsened during strated contrast enhancement on Tl-weighted images. this time, but he reports that it interferes with his ability to keep up with the physical demands of his job. His only Which of the following is the most appropriate manage- medication is dimethyl fumarate. ment? Physical examination reveals increased tone in the left

A 60-year-old woman comes to the office to discuss the best (D) Venous thromboembolism prophylaxis strategy to prevent cognitive impairment. She has no medical problems and takes no medications. She does not use tobacco, Item 28 has 1 to 2 alcoholic drinks per week, and is sedentary. On physical examination, vital signs and other findings A 78-year-old woman is evaluated for a 1-year history of are unremarkable. progressively worsening memory impairment. She can no Results of cognitive screening testing are normal. longer handle her finances, as she doesn’t remember how to fill out checks properly. She lives with her spouse and Which of the following is the most effective preventive is independent in all other basic activities of daily living. measure? On physical examination, vital signs are normal. Mon- treal Cognitive Assessment score is 21/30, with difficulty (A) Cognitive training copying the cube, recalling the five words, and naming the (B) Donepezil day, date, and month noted. (C) Physical exercise training Results of laboratory evaluation are normal. (D) Vitamin E supplementation Which of the following is the most appropriate imaging test to perform next? Item 26 (A) Amyloid PET scan A 39-year-old woman is evaluated for a 3-month history (B) Fluorodeoxyglucose PET scan of low energy, lack of interest in activities, and poor sleep. (C) Head CT with contrast She has relapsing-remitting multiple sclerosis and a history of vitamin D deficiency. Medications are glatiramer acetate (D) MRI of the brain without contrast and a vitamin D supplement. On physical examination, vital signs are normal. All Item 29 other examination findings, including those from a neuro- logic examination, are unremarkable. A 55-year-old man with relapsing-remitting multiple scle- Two weeks ago, a routine MRI of the brain showeda sta- rosis is evaluated for a slow, labored gait that began after a ble number of T2 lesions, and none of these lesions demon- relapse 1 year ago. The symptom has not worsened during strated contrast enhancement on Tl-weighted images. this time, but he reports that it interferes with his ability to keep up with the physical demands of his job. His only Which of the following is the most appropriate manage- medication is dimethyl fumarate. ment? Physical examination reveals increased tone in the left (A) Initiate modafinil leg. Muscle strength is 4/5 with hip flexion and ankle dorsi- flexion. Gait is slow, and there is a slight outward swing of (B) Measure serum 25-hydroxyvitamin D level the left leg when ambulating. The remainder of the exam- (C) Screen for depression ination is normal. (D) Switch to natalizumab Which of the following interventions is most likely to improve this patient’s ambulation? Item 27 (A) Initiate dalfampridine A 72-year-old woman is hospitalized for an acute stroke: (B) Initiate modafinil symptoms have been present for 24 hours. She has chronic (C) Initiate tizanidine hypertension. Current medications are amlodipine and hydrochlorothiazide. (D) Switch to natalizumab On physical examination, temperature is normal and (E) Switch to ocrelizumab blood pressure is 136/78 mm Hg (decreased from 170/90 mm Hg at presentation): the remaining vital signs are normal.

(A) Initiate modafinil leg. Muscle strength is 4/5 with hip flexion and ankle dorsi- flexion. Gait is slow, and there is a slight outward swing of (B) Measure serum 25-hydroxyvitamin D level the left leg when ambulating. The remainder of the exam- (C) Screen for depression ination is normal. (D) Switch to natalizumab Which of the following interventions is most likely to improve this patient’s ambulation? Item 27 (A) Initiate dalfampridine A 72-year-old woman is hospitalized for an acute stroke: (B) Initiate modafinil symptoms have been present for 24 hours. She has chronic (C) Initiate tizanidine hypertension. Current medications are amlodipine and hydrochlorothiazide. (D) Switch to natalizumab On physical examination, temperature is normal and (E) Switch to ocrelizumab blood pressure is 136/78 mm Hg (decreased from 170/90 mm Hg at presentation): the remaining vital signs are normal. Hi) Oxygen saturation is 97% with the patient breathing ambi- Item 30 ent air. Left facial weakness, no movement in the left arm A 74-year-old woman is evaluated in the hospital at 2:00 AM and leg. and decreased pinprick sensation on the left side for agitation. She was admitted 3 days ago with acute are noted. Mental status is normal. A bedside swallow diverticulitis. Over the last 24 hours, the patient has been 102

Self-Assessment Test — alternately agitated or drowsy, sometimes confused when ECG shows atrial fibrillation. | cH awake, and tonight has experienced visual hallucinations. CT of the head shows no acute findings. nt < CONT. Her only other medical problem is long-standing depres ct) sion treated with paroxetine, which has been discontinued. Which of the following is the most appropriate next step £ 2) management? wn Medical records indicate she does not drink alcohol. Her <5) wn current medications include oxycodone, scheduled every (A) CT angiography of the brain “a

sion treated with paroxetine, which has been discontinued. Which of the following is the most appropriate next step £ 2) management? wn Medical records indicate she does not drink alcohol. Her <5) wn current medications include oxycodone, scheduled every (A) CT angiography of the brain “a 6 hours, and piperacillin tazobactam. (B) Intravenous heparin Ris On physical a examination, temperature is 37.2 °C (-F) (C) Intravenous labetalol (99.0 °F), and the remaining vital signs are normal. Oxygen (72) saturation is 97% with the patient breathing ambient air. The (D) No additional intervention or treatment abdomen has diminished but present bowel sounds, and there is mild tenderness to palpation without guarding in the Item 33 left lower quadrant. The remainder of the physical examina tion is normal. The patient is alert and vigilant but inattentive A 78-year-old man is evaluated in the emergency depart-

saturation is 97% with the patient breathing ambient air. The (D) No additional intervention or treatment abdomen has diminished but present bowel sounds, and there is mild tenderness to palpation without guarding in the Item 33 left lower quadrant. The remainder of the physical examina tion is normal. The patient is alert and vigilant but inattentive A 78-year-old man is evaluated in the emergency depart- to questioning and demonstrates disorganized thinking. ment after a fall. He has Parkinson disease treated with An order is written to hold the oxycodone. carbidopa levodopa. Since he started this treatment 2 months ago, tremor and walking have improved, but he Which of the following is the most appropriate next step in feels lightheaded upon standing. He also has type 2 diabetes management? and hyperlipidemia. Current medications are carbidopa- levodopa. metformin, and atorvastatin. (A) Diphenhydramine On physical examination, blood pressure is 140/80 mm (B) Lorazepam Hg sitting and 80/65 mm Hg standing. Pulse rate is 84/min. (C) Paroxetine A mild right-sided tremor at rest is noted. His gait is slow (D) Zolpidem with a narrow pace without shuffling or freezing. He can (E) No additional treatment walk in tandem and stabilizes himself against backward pull on his shoulders.

An order is written to hold the oxycodone. carbidopa levodopa. Since he started this treatment 2 months ago, tremor and walking have improved, but he Which of the following is the most appropriate next step in feels lightheaded upon standing. He also has type 2 diabetes management? and hyperlipidemia. Current medications are carbidopa- levodopa. metformin, and atorvastatin. (A) Diphenhydramine On physical examination, blood pressure is 140/80 mm (B) Lorazepam Hg sitting and 80/65 mm Hg standing. Pulse rate is 84/min. (C) Paroxetine A mild right-sided tremor at rest is noted. His gait is slow (D) Zolpidem with a narrow pace without shuffling or freezing. He can (E) No additional treatment walk in tandem and stabilizes himself against backward pull on his shoulders. Item 31 Which of the following is the most appropriate next step to prevent further falls? A 30-year-old man is evaluated for a 1-hour history of a severe headache. He has had similar headaches for the past (A) Add droxidopa 2 weeks that start in the evening, last 2 to 3 hours, and are (B) Add fludrocortisone characterized by intense left-sided pain that is periorbital (C) Add gabapentin and piercing. Associated features are photophobia, nausea, and ipsilateral tearing. Nasal sumatriptan, acetaminophen, (D) Add pramipexole and ibuprofen have not relieved the pain. He has a 7-pack- (E) Increase carbidopa dosage year smoking history. On physical examination, vital signs are within normal limits. Left ptosis and miosis are noted. Item 34 Results of laboratory studies and MRI of the brain with A 26-year-old man is evaluated for a 5-month history of contrast are normal. jerking episodes that occur three to four times monthly. The jerking lasts less than 1 second. He is aware during the Which of the following is the most appropriate next step in episodes and retains a complete memory of the event. He management? reports that sometimes the jerking occurs in clusters and (A) Carbamazepine he will have 10 to 20 jerks over a period of 30 to 90 seconds,

Item 31 Which of the following is the most appropriate next step to prevent further falls? A 30-year-old man is evaluated for a 1-hour history of a severe headache. He has had similar headaches for the past (A) Add droxidopa 2 weeks that start in the evening, last 2 to 3 hours, and are (B) Add fludrocortisone characterized by intense left-sided pain that is periorbital (C) Add gabapentin and piercing. Associated features are photophobia, nausea, and ipsilateral tearing. Nasal sumatriptan, acetaminophen, (D) Add pramipexole and ibuprofen have not relieved the pain. He has a 7-pack- (E) Increase carbidopa dosage year smoking history. On physical examination, vital signs are within normal limits. Left ptosis and miosis are noted. Item 34 Results of laboratory studies and MRI of the brain with A 26-year-old man is evaluated for a 5-month history of contrast are normal. jerking episodes that occur three to four times monthly. The jerking lasts less than 1 second. He is aware during the Which of the following is the most appropriate next step in episodes and retains a complete memory of the event. He management? reports that sometimes the jerking occurs in clusters and (A) Carbamazepine he will have 10 to 20 jerks over a period of 30 to 90 seconds, (B) Indomethacin often on awakening from sleep. He denies confusion or loss of muscle tone with either the single or clustered events. He (C) Magnetic resonance angiography of the head and has no other medical problems and takes no medications. neck On physical examination, vital signs are normal. The (D) Subcutaneous sumatriptan neurologic examination is normal.

(B) Indomethacin often on awakening from sleep. He denies confusion or loss of muscle tone with either the single or clustered events. He (C) Magnetic resonance angiography of the head and has no other medical problems and takes no medications. neck On physical examination, vital signs are normal. The (D) Subcutaneous sumatriptan neurologic examination is normal. Which of the following is the most likely diagnosis? Item 32 (A) Focal motor aware seizures A 76-year-old woman is evaluated in the emergency depart (B) Generalized tonic-clonic seizures ment for a stroke. She is given intravenous alteplase within 3 hours of onset. She also has hypertension. Her only outpa (C) Myoclonic seizures tient medication is amlodipine. (D) Tonic seizures On physical examination, temperature is 37.6 °C (99.7 °F). blood pressure is 160/76 mm Hg, pulse rate is 68/min and irregular, and oxygen saturation is 96% with Item 35 the patient breathing ambient air. She has global aphasia A 64-year-old man is hospitalized for right-sided weakness with no speech production. right facial weakness. and lack and slurred speech of 24 hours’ duration. He has hyperten of movement in right arm and leg. National Institutes of sion and dyslipidemia. Current medications are aspirin and Health Stroke Scale score is 18. high-intensity atorvastatin. 103

lled rn pes Which of the following is most likely the cause of this =h On physical examination, blood pressure is 190/88 mm Se wv Hg and oxygen saturation is 98% with the patient breath- patient’s symptoms? wn oO CONT. ing ambient air. The remaining vital signs are normal. (A) Immune-mediated necrotizing myopathy wn n Abnormal physical examination findings are confined to (B) Polymyalgia rheumatica =] right facial weakness and right arm and leg pronator drift. @ National Institutes of Health Stroke Scale score is 3. (C) Proximal lumbosacral radiculoneuropathy = -- ECG is normal. Serum creatinine level is 0.9 mg/dL (D) Statin-induced myopathy ry w (79.6 umol/L), and cardiac biomarkers are negative. CT sean of the head shows a left frontal infarction. or

lled rn pes Which of the following is most likely the cause of this =h On physical examination, blood pressure is 190/88 mm Se wv Hg and oxygen saturation is 98% with the patient breath- patient’s symptoms? wn oO CONT. ing ambient air. The remaining vital signs are normal. (A) Immune-mediated necrotizing myopathy wn n Abnormal physical examination findings are confined to (B) Polymyalgia rheumatica =] right facial weakness and right arm and leg pronator drift. @ National Institutes of Health Stroke Scale score is 3. (C) Proximal lumbosacral radiculoneuropathy = -- ECG is normal. Serum creatinine level is 0.9 mg/dL (D) Statin-induced myopathy ry w (79.6 umol/L), and cardiac biomarkers are negative. CT sean of the head shows a left frontal infarction. or CT angiogram shows 80% stenosis of middle cerebral artery. Item 38 An 85-year-old man is evaluated in the hospital after sur- Hi) gical fixation of a broken hip. He reports a mild level of Which of the following is the most appropriate immediate pain. He did not bring his glasses or hearing aids to the treatment? hospital. He has been making progress with physical ther- (A) Chlorthalidone apy and, when his pain is controlled, he will be transferred (B) Furosemide to an inpatient rehabilitation facility. His history includes high-frequency hearing loss, macular degeneration, and (C) Intracranial stenting hypertension. Medications are lisinopril and chlorthalidone. (D) Lisinopril On physical examination, vital signs and other findings (E) No additional intervention or treatment are normal.

CT angiogram shows 80% stenosis of middle cerebral artery. Item 38 An 85-year-old man is evaluated in the hospital after sur- Hi) gical fixation of a broken hip. He reports a mild level of Which of the following is the most appropriate immediate pain. He did not bring his glasses or hearing aids to the treatment? hospital. He has been making progress with physical ther- (A) Chlorthalidone apy and, when his pain is controlled, he will be transferred (B) Furosemide to an inpatient rehabilitation facility. His history includes high-frequency hearing loss, macular degeneration, and (C) Intracranial stenting hypertension. Medications are lisinopril and chlorthalidone. (D) Lisinopril On physical examination, vital signs and other findings (E) No additional intervention or treatment are normal. Which of the following is the most appropriate measure to ltem 36 prevent delirium?

CT angiogram shows 80% stenosis of middle cerebral artery. Item 38 An 85-year-old man is evaluated in the hospital after sur- Hi) gical fixation of a broken hip. He reports a mild level of Which of the following is the most appropriate immediate pain. He did not bring his glasses or hearing aids to the treatment? hospital. He has been making progress with physical ther- (A) Chlorthalidone apy and, when his pain is controlled, he will be transferred (B) Furosemide to an inpatient rehabilitation facility. His history includes high-frequency hearing loss, macular degeneration, and (C) Intracranial stenting hypertension. Medications are lisinopril and chlorthalidone. (D) Lisinopril On physical examination, vital signs and other findings (E) No additional intervention or treatment are normal. Which of the following is the most appropriate measure to ltem 36 prevent delirium? A 44-year-old man is evaluated for muscle cramps and (A) Bedrest except during physical therapy fatigue. He frequently experiences cramps after prolonged (B) Monitoring of vital signs every 2 hours exertion. Within the past few months he has developed (C) Quetiapine nightly difficulty climbing stairs and feels that his walking has (D) Scheduled morphine become unsteady. He has no family history of neuromus- cular disease. (E) Use of his glasses and hearing aids On physical examination, vital signs are normal. There is no muscle tenderness on palpation. Muscle strength is

A 44-year-old man is evaluated for muscle cramps and (A) Bedrest except during physical therapy fatigue. He frequently experiences cramps after prolonged (B) Monitoring of vital signs every 2 hours exertion. Within the past few months he has developed (C) Quetiapine nightly difficulty climbing stairs and feels that his walking has (D) Scheduled morphine become unsteady. He has no family history of neuromus- cular disease. (E) Use of his glasses and hearing aids On physical examination, vital signs are normal. There is no muscle tenderness on palpation. Muscle strength is Hi) reduced in bilateral hip flexors and arm abductors and Item 39 preserved distally. Deep tendon reflexes are preserved but A 38-year-old man is admitted to the hospital for surgi- exhibit delayed relaxation. Percussion over biceps muscles cal resection of a glioblastoma. Following resection, he is leads to formation of a raised muscular ridge. Gait has a expected to remain in intensive care for 2 days and dis waddling quality. Sensory examination and muscle tone are charged home by the third day. normal. Plantar responses are flexor. There are no involun- Which of the following is the most appropriate measure to tary movements. prevent venous thromboembolism immediately following Which of the following is the most likely cause of this surgery? patient’s findings? (A) Low-molecular-weight heparin (A) Copper deficiency (B) Mechanical prophylaxis (B) Hypokalemia (C) Unfractionated heparin (C) Hypothyroidism (D) No prophylaxis is needed ( ) Vitamin D deficiency o

Hi) reduced in bilateral hip flexors and arm abductors and Item 39 preserved distally. Deep tendon reflexes are preserved but A 38-year-old man is admitted to the hospital for surgi- exhibit delayed relaxation. Percussion over biceps muscles cal resection of a glioblastoma. Following resection, he is leads to formation of a raised muscular ridge. Gait has a expected to remain in intensive care for 2 days and dis waddling quality. Sensory examination and muscle tone are charged home by the third day. normal. Plantar responses are flexor. There are no involun- Which of the following is the most appropriate measure to tary movements. prevent venous thromboembolism immediately following Which of the following is the most likely cause of this surgery? patient’s findings? (A) Low-molecular-weight heparin (A) Copper deficiency (B) Mechanical prophylaxis (B) Hypokalemia (C) Unfractionated heparin (C) Hypothyroidism (D) No prophylaxis is needed ( ) Vitamin D deficiency o ( E) Vitamin E deficiency Item 40 A 31-year-old man is evaluated for a 5-mm saccular item 37 aneurysm in the anterior communicating artery found incidentally on a magnetic resonance angiogram of the A 63-year-old man is evaluated for myalgia and proximal head obtained to investigate new-onset trigeminal neu- weakness in both upper and lower extremities. He has dia- ralgia. The patient is otherwise asymptomatic and has betes, hypertension, and coronary artery disease. Current no additional medical problems. His only medication is medications include metformin, empagliflozin, lisinopril, carbamazepine. and atorvastatin. On physical examination, vital signs, general physical On physical examination, blood pressure is 132/82 mm and neurologic examinations are normal. Hg; other vital signs are normal. Proximal muscle tender- ness is noted. All other findings, including those from the Which of the following is the most appropriate manage- neurologic examination, are normal. ment? Laboratory studies show an erythrocyte sedimentation rate of 15 mm/h, hemoglobin A,, level of 6.8%, and serum (A) Aneurysm clipping creatine kinase level of 250 U/L. (B) Aneurysm coiling

( E) Vitamin E deficiency Item 40 A 31-year-old man is evaluated for a 5-mm saccular item 37 aneurysm in the anterior communicating artery found incidentally on a magnetic resonance angiogram of the A 63-year-old man is evaluated for myalgia and proximal head obtained to investigate new-onset trigeminal neu- weakness in both upper and lower extremities. He has dia- ralgia. The patient is otherwise asymptomatic and has betes, hypertension, and coronary artery disease. Current no additional medical problems. His only medication is medications include metformin, empagliflozin, lisinopril, carbamazepine. and atorvastatin. On physical examination, vital signs, general physical On physical examination, blood pressure is 132/82 mm and neurologic examinations are normal. Hg; other vital signs are normal. Proximal muscle tender- ness is noted. All other findings, including those from the Which of the following is the most appropriate manage- neurologic examination, are normal. ment? Laboratory studies show an erythrocyte sedimentation rate of 15 mm/h, hemoglobin A,, level of 6.8%, and serum (A) Aneurysm clipping creatine kinase level of 250 U/L. (B) Aneurysm coiling 104

Self-Assessment Test — cH (C) Catheter-based angiography episode of getting lost while driving on a familiar route, — fo) (D) CT angiography and repeated instances of losing her cell phone. She lives <= c.f) alone and is independent in all of her basic activities of daily (E) Serial magnetic resonance angiography = living, except that her daughter has automated her finances wn wn because of some errors. <8) wn item 41 On physical examination, vital signs are normal and wn

(D) CT angiography and repeated instances of losing her cell phone. She lives <= c.f) alone and is independent in all of her basic activities of daily (E) Serial magnetic resonance angiography = living, except that her daughter has automated her finances wn wn because of some errors. <8) wn item 41 On physical examination, vital signs are normal and wn other findings are unremarkable. The Mini-Mental State ow An 84-year-old man is evaluated for an 18-month history po Examination score is 23. wo of increasing forgetfulness and one episode of getting lost 2) An MRI of the brain shows significant hippocampal while driving on a familiar route. He lost his car keys and atrophy bilaterally with minimal white matter hyperinten- had to have a new set made. He lives with his spouse and is sities. independent in all of his instrumental and basic activities of daily living. He and his spouse report no signs and symptoms Which of the following is the most appropriate treatment? of depression. In high school and in college he was on the (A) Donepezil boxing team. He is otherwise well and takes no medications. On physical examination, vital signs are normal. His (B) Ginkgo biloba Montreal Cognitive Assessment score is 21/30, with diffi- (C) High-dose vitamin E culty copying the cube, recalling the five words, and naming (D) Memantine the day, date, and month noted. Depression screening is negative. Brain MRI shows significant hippocampal atrophy Item 44 bilaterally with minimal white matter hyperintensities. A 66-year-old man is evaluated for abnormal behavior during Which of the following is the most likely diagnosis? sleep. His wife reports he has a prolonged history of nocturnal flailing and jerking movements and of shouting, punching, (A) Alzheimer dementia and jumping out of bed. The patient does not recall these (B) Mild cognitive impairment movements and reports no discomfort. He also has a history (C) Traumatic encephalopathy syndrome of urinary incontinence and falls. He takes no medications. (D) Vascular cognitive impairment On physical examination, vital signs are normal. He has reduced facial expression and bilateral rigidity. His gait is unsteady, with postural imbalance during turns, and Item 42 he cannot walk in tandem. Other neurologic examination findings are unremarkable. A 48-year-old man is taken to the emergency department with A polysomnogram reveals complex movements a 3-month history of progressive headaches, double vision, associated with preserved muscle tone during rapid-eye- and slurred speech. On arrival, he has a sudden decline in movement sleep. mentation, becomes comatose, and is emergently intubated. On physical examination, blood pressure is 188/96 mm Which of the following is the most likely diagnosis? Hg, pulse rate is 48/min, and respiration rate is 10/min. The patient is comatose, with large unreactive pupils bilaterally, (A) Hypnic myoclonus has no spontaneous movements, and withdraws his upper (B) Periodic limb movement disorder and lower limbs from noxious stimuli. (C) Rapid eye movement sleep behavior disorder Head CT shows a midline thalamic mass extending (D) Restless legs syndrome downward into the upper brainstem with associated paren (E) Vivid dreams chymal and intraventricular hemorrhage. There is associ- ated edema and mass effect on the brainstem, leading to compression of brainstem and crowding of the foramen magnum. Item 45 The patient’s head of the bed is elevated to 30 degrees, A 68-year-old woman is evaluated for a diffuse pruritic rash hyperventilation with mechanical ventilation in initiated, that began 2 days ago and initially involved the torso. Today and intravenous dexamethasone is administered. the rash has spread to the upper and lower extremities and back. She began lamotrigine therapy 6 weeks ago for Which of the following is the most appropriate additional epilepsy and discontinued it yesterday. She denies ocular emergent management? symptoms, sores in her mouth, or fever. She is minimally (A) Bevacizumab symptomatic. She has no other medical problems and takes

other findings are unremarkable. The Mini-Mental State ow An 84-year-old man is evaluated for an 18-month history po Examination score is 23. wo of increasing forgetfulness and one episode of getting lost 2) An MRI of the brain shows significant hippocampal while driving on a familiar route. He lost his car keys and atrophy bilaterally with minimal white matter hyperinten- had to have a new set made. He lives with his spouse and is sities. independent in all of his instrumental and basic activities of daily living. He and his spouse report no signs and symptoms Which of the following is the most appropriate treatment? of depression. In high school and in college he was on the (A) Donepezil boxing team. He is otherwise well and takes no medications. On physical examination, vital signs are normal. His (B) Ginkgo biloba Montreal Cognitive Assessment score is 21/30, with diffi- (C) High-dose vitamin E culty copying the cube, recalling the five words, and naming (D) Memantine the day, date, and month noted. Depression screening is negative. Brain MRI shows significant hippocampal atrophy Item 44 bilaterally with minimal white matter hyperintensities. A 66-year-old man is evaluated for abnormal behavior during Which of the following is the most likely diagnosis? sleep. His wife reports he has a prolonged history of nocturnal flailing and jerking movements and of shouting, punching, (A) Alzheimer dementia and jumping out of bed. The patient does not recall these (B) Mild cognitive impairment movements and reports no discomfort. He also has a history (C) Traumatic encephalopathy syndrome of urinary incontinence and falls. He takes no medications. (D) Vascular cognitive impairment On physical examination, vital signs are normal. He has reduced facial expression and bilateral rigidity. His gait is unsteady, with postural imbalance during turns, and Item 42 he cannot walk in tandem. Other neurologic examination findings are unremarkable. A 48-year-old man is taken to the emergency department with A polysomnogram reveals complex movements a 3-month history of progressive headaches, double vision, associated with preserved muscle tone during rapid-eye- and slurred speech. On arrival, he has a sudden decline in movement sleep. mentation, becomes comatose, and is emergently intubated. On physical examination, blood pressure is 188/96 mm Which of the following is the most likely diagnosis? Hg, pulse rate is 48/min, and respiration rate is 10/min. The patient is comatose, with large unreactive pupils bilaterally, (A) Hypnic myoclonus has no spontaneous movements, and withdraws his upper (B) Periodic limb movement disorder and lower limbs from noxious stimuli. (C) Rapid eye movement sleep behavior disorder Head CT shows a midline thalamic mass extending (D) Restless legs syndrome downward into the upper brainstem with associated paren (E) Vivid dreams chymal and intraventricular hemorrhage. There is associ- ated edema and mass effect on the brainstem, leading to compression of brainstem and crowding of the foramen magnum. Item 45 The patient’s head of the bed is elevated to 30 degrees, A 68-year-old woman is evaluated for a diffuse pruritic rash hyperventilation with mechanical ventilation in initiated, that began 2 days ago and initially involved the torso. Today and intravenous dexamethasone is administered. the rash has spread to the upper and lower extremities and back. She began lamotrigine therapy 6 weeks ago for Which of the following is the most appropriate additional epilepsy and discontinued it yesterday. She denies ocular emergent management? symptoms, sores in her mouth, or fever. She is minimally (A) Bevacizumab symptomatic. She has no other medical problems and takes (B) Decompressive craniotomy no other medications. On physical examination, vital signs are normal. There (C) Intravenous mannitol is a patchy, erythematous maculopapular rash on the upper (D) Intravenous 0.9% saline and lower extremities, torso, and back. There is no involve- ment of the mucosa, palms, or soles. The remainder of the examination is normal. Item 43 Complete blood count with differential and a compre- An 82-year-old woman is evaluated for an 18-month his- hensive metabolic panel are normal. tory of progressively worsening repetitive questioning, one Diphenhydramine is prescribed.

(B) Decompressive craniotomy no other medications. On physical examination, vital signs are normal. There (C) Intravenous mannitol is a patchy, erythematous maculopapular rash on the upper (D) Intravenous 0.9% saline and lower extremities, torso, and back. There is no involve- ment of the mucosa, palms, or soles. The remainder of the examination is normal. Item 43 Complete blood count with differential and a compre- An 82-year-old woman is evaluated for an 18-month his- hensive metabolic panel are normal. tory of progressively worsening repetitive questioning, one Diphenhydramine is prescribed. 105

Self-Assessment Test 72) wk = Which of the following is the most appropriate seizure no changes in extremity strength but sometimes finds it > management? hard to lift his head. He takes no medications. wn wn @o On physical examination, vital signs are normal. Lower wn (A) Carbamazepine %) facial muscles are weak. Tongue is atrophied and moves = (B) Gabapentin slowly; fasciculations are present. Neck flexion and right- @o | (C) Phenobarbital hand finger flexion are mildly weak. Jaw jerk and patellar Ed (D) Phenytoin reflexes are brisk, and right ankle clonus is present. Extra- oD wn ocular movements are intact. oF Creatine kinase level is 250 U/L. Item 46 MRI of the brain and cervical spine and chest radio- graph are normal. A 62-year-old man is evaluated in the emergency depart ment for right arm weakness that lasted 20 minutes and Which of the following is the most likely diagnosis? occurred 6 hours ago. He has hypertension and type 2 diabe (A) Amyotrophic lateral sclerosis tes mellitus. Current medications are metformin, lisinopril, and hydrochlorothiazide. (B) Chronic inflammatory demyelinating polyradiculo- On physical examination, blood pressure is 142/82 mm neuropathy Hg; the remainder of the vital signs and neurologic exam (C) Inclusion body myositis ination are normal. (D) Myasthenia gravis Hemoglobin A,,. value is 6.5%, and LDL cholesterol level (E) Neuromyelitis optica is 120 mg/dL (3.1 mmol/L). Basic metabolic profile and ami notransferase levels are normal. CT scan of the head shows no acute ischemic changes. Item 49 Carotid duplex ultrasound shows less than 40% stenosis A 70-year-old woman is evaluated in the hospital for the in both carotid arteries and normal flow in the vertebral recent onset of severe back pain and gait ataxia. Extensive arteries. stage small cell lung cancer was diagnosed 1 month ago. High-intensity rosuvastatin is initiated. Metastases to brain, liver, and adrenal glands were noted. Which of the following is the most appropriate additional She has decided to forego active therapy and has opted treatment? for comfort care. Until the past week she has experienced only modest pain. Her other medical problems include (A) Aspirin and apixaban chronic kidney disease, diabetes mellitus, hypertension, (B) Aspirin and clopidogrel and oxygen-dependent COPD. Medications are oral mor (C) Aspirin, clopidogrel, and dipyridamole phine, losartan, hydrochlorothiazide, metformin, albuterol, and tiotropium inhalers. Her predicted life expectancy is (D) Ticagrelor 3 months. On physical examination, vital signs are normal. She has a wide- based gait and poor vibratory and position sense in the Item 47 feet. Muscle strength testing shows 4/5 bilateral hip flexion. A 49-year-old woman is evaluated in the emergency MRI ofthe thoracic spine reveals two contrast-enhancing department for sudden-onset severe headache of masses involving the posterior vertebra and dura at T3 and 24 hours’ duration. The headache reached its peak inten T8. Both lesions cause cord compression with increased T2 sity within 1 minute. She is otherwise well and takes no signal in the cord. medications. On physical examination, vital signs are normal. Which of the following is the most appropriate treatment? Nuchal rigidity is present. Findings on mental status and (A) Glucocorticoids, surgical decompression, and radio funduscopic examinations are normal. There are no cranial therapy nerve deficits. ACT scan of the head is normal. (B) Glucocorticoids and radiotherapy (C) Glucocorticoids and surgical decompression Which of the following is the most appropriate next step in (D) Radiotherapy and surgical decompression management?

72) wk = Which of the following is the most appropriate seizure no changes in extremity strength but sometimes finds it > management? hard to lift his head. He takes no medications. wn wn @o On physical examination, vital signs are normal. Lower wn (A) Carbamazepine %) facial muscles are weak. Tongue is atrophied and moves = (B) Gabapentin slowly; fasciculations are present. Neck flexion and right- @o | (C) Phenobarbital hand finger flexion are mildly weak. Jaw jerk and patellar Ed (D) Phenytoin reflexes are brisk, and right ankle clonus is present. Extra- oD wn ocular movements are intact. oF Creatine kinase level is 250 U/L. Item 46 MRI of the brain and cervical spine and chest radio- graph are normal. A 62-year-old man is evaluated in the emergency depart ment for right arm weakness that lasted 20 minutes and Which of the following is the most likely diagnosis? occurred 6 hours ago. He has hypertension and type 2 diabe (A) Amyotrophic lateral sclerosis tes mellitus. Current medications are metformin, lisinopril, and hydrochlorothiazide. (B) Chronic inflammatory demyelinating polyradiculo- On physical examination, blood pressure is 142/82 mm neuropathy Hg; the remainder of the vital signs and neurologic exam (C) Inclusion body myositis ination are normal. (D) Myasthenia gravis Hemoglobin A,,. value is 6.5%, and LDL cholesterol level (E) Neuromyelitis optica is 120 mg/dL (3.1 mmol/L). Basic metabolic profile and ami notransferase levels are normal. CT scan of the head shows no acute ischemic changes. Item 49 Carotid duplex ultrasound shows less than 40% stenosis A 70-year-old woman is evaluated in the hospital for the in both carotid arteries and normal flow in the vertebral recent onset of severe back pain and gait ataxia. Extensive arteries. stage small cell lung cancer was diagnosed 1 month ago. High-intensity rosuvastatin is initiated. Metastases to brain, liver, and adrenal glands were noted. Which of the following is the most appropriate additional She has decided to forego active therapy and has opted treatment? for comfort care. Until the past week she has experienced only modest pain. Her other medical problems include (A) Aspirin and apixaban chronic kidney disease, diabetes mellitus, hypertension, (B) Aspirin and clopidogrel and oxygen-dependent COPD. Medications are oral mor (C) Aspirin, clopidogrel, and dipyridamole phine, losartan, hydrochlorothiazide, metformin, albuterol, and tiotropium inhalers. Her predicted life expectancy is (D) Ticagrelor 3 months. On physical examination, vital signs are normal. She has a wide- based gait and poor vibratory and position sense in the Item 47 feet. Muscle strength testing shows 4/5 bilateral hip flexion. A 49-year-old woman is evaluated in the emergency MRI ofthe thoracic spine reveals two contrast-enhancing department for sudden-onset severe headache of masses involving the posterior vertebra and dura at T3 and 24 hours’ duration. The headache reached its peak inten T8. Both lesions cause cord compression with increased T2 sity within 1 minute. She is otherwise well and takes no signal in the cord. medications. On physical examination, vital signs are normal. Which of the following is the most appropriate treatment? Nuchal rigidity is present. Findings on mental status and (A) Glucocorticoids, surgical decompression, and radio funduscopic examinations are normal. There are no cranial therapy nerve deficits. ACT scan of the head is normal. (B) Glucocorticoids and radiotherapy (C) Glucocorticoids and surgical decompression Which of the following is the most appropriate next step in (D) Radiotherapy and surgical decompression management? (A) Cerebral catheter angiography Item 50 (B) Lumbar puncture A 67-year-old man comes to the office for a follow-up eval- (C) Magnetic resonance venography of the brain uation of secondary progressive multiple sclerosis (MS). He (D) Oral sumatriptan was diagnosed with relapsing-remitting MS 20 years ago and had his last relapse 6 years ago. His disease converted to a secondary progressive course 4 years ago, and 2.5 years Item 48 ago, he became nonambulatory. His only medication is A 54-year-old man is evaluated for difficulty swallowing interferon-beta 1b. of 6 months’ duration. He initially had occasional choking On physical examination, all vital signs are normal. with liquids, which has progressed to dysphagia with both Marked spasticity is noted in the lower extremities. Muscle liquids and solids. His voice has become hoarse. He reports strength is grade 1/5 with hip and knee flexion, bilaterally,

(A) Cerebral catheter angiography Item 50 (B) Lumbar puncture A 67-year-old man comes to the office for a follow-up eval- (C) Magnetic resonance venography of the brain uation of secondary progressive multiple sclerosis (MS). He (D) Oral sumatriptan was diagnosed with relapsing-remitting MS 20 years ago and had his last relapse 6 years ago. His disease converted to a secondary progressive course 4 years ago, and 2.5 years Item 48 ago, he became nonambulatory. His only medication is A 54-year-old man is evaluated for difficulty swallowing interferon-beta 1b. of 6 months’ duration. He initially had occasional choking On physical examination, all vital signs are normal. with liquids, which has progressed to dysphagia with both Marked spasticity is noted in the lower extremities. Muscle liquids and solids. His voice has become hoarse. He reports strength is grade 1/5 with hip and knee flexion, bilaterally, 106

; Self-Assessment Test es o and grade 2/5 with ankle dorsiflexion, bilaterally. An Item 53 — vine indwelling suprapubic catheter is noted. A 72-year-old man is evaluated for dementia with fluctua- = °F) A recent MRI of the brain shows multiple lesions tions in attentiveness, visual hallucinations, and slowness = consistent with MS that are unchanged from 2 years ago; of movements over the past year. He has also acted out his w wn gadolinium does not enhance the lesions. dreams in his sleep a few times over the last 2 years. <P) wn wn

gadolinium does not enhance the lesions. dreams in his sleep a few times over the last 2 years. <P) wn wn Which of the following changes to the patient’s immuno- On physical examination, vital signs are normal. Mild = modulatory therapy is most appropriate? hypomimia, symmetrically increased tone in bilateral upper — a extremities, and bradykinesia on rapid alternating move- “2) (A) Administer intravenous glucocorticoids ments are observed. (B) Discontinue interferon beta-1b Which of the following is the most appropriate (C) Substitute natalizumab for interferon beta-1b treatment? (D) Substitute ocrelizumab for interferon beta-1b (A) Clonazepam (B) Haloperidol Item 51 (C) Memantine An 18-year-old woman is evaluated for a 5-year history (D) Rivastigmine of recurrent headaches. Headaches occur approximately (E) Zolpidem 12 days per month; three of these episodes are severe, occur- ring without a trigger or premonitory symptoms, and last for a full day, followed by profound fatigue the next day. She Item 54 describes the pain as bilateral, frontotemporal, steady and A 43-year-old woman is seen in a follow-up evaluation of throbbing, associated with sensitivities to light and noise, myasthenia gravis diagnosed 2 years ago. Current treatment worsening with movement, and causing her to miss 2 or with daily prednisone and pyridostigmine partially controls 3 days of school per month. The severe headaches have not her symptoms. She is experiencing weakness with pro- responded to ibuprofen or naproxen. The patient also has longed activity and in the evening. She wishes to minimize seasonal allergies. Other medications are fexofenadine and long-term need for glucocorticoids and other medications a combination estrogen-progestin oral contraceptive. that may suppress her immune system. All physical examination findings, including vital signs, On physical examination, vital signs are normal. She are normal. has mild bilateral ptosis and impaired left eye adduction Which of the following is the most appropriate next step in and bilateral eye abduction. Speech is nasal. Proximal upper management? and lower extremity muscle strength and neck flexion are mildly weak. (A) Begin oral zolmitriptan Acetylcholine receptor-binding antibody is positive. (B) Discontinue the oral contraceptive CT of the chest is normal. (C) Obtain a brain MRI Which of the following is the most appropriate (D) Substitute loratadine for fexofenadine treatment?

Which of the following changes to the patient’s immuno- On physical examination, vital signs are normal. Mild = modulatory therapy is most appropriate? hypomimia, symmetrically increased tone in bilateral upper — a extremities, and bradykinesia on rapid alternating move- “2) (A) Administer intravenous glucocorticoids ments are observed. (B) Discontinue interferon beta-1b Which of the following is the most appropriate (C) Substitute natalizumab for interferon beta-1b treatment? (D) Substitute ocrelizumab for interferon beta-1b (A) Clonazepam (B) Haloperidol Item 51 (C) Memantine An 18-year-old woman is evaluated for a 5-year history (D) Rivastigmine of recurrent headaches. Headaches occur approximately (E) Zolpidem 12 days per month; three of these episodes are severe, occur- ring without a trigger or premonitory symptoms, and last for a full day, followed by profound fatigue the next day. She Item 54 describes the pain as bilateral, frontotemporal, steady and A 43-year-old woman is seen in a follow-up evaluation of throbbing, associated with sensitivities to light and noise, myasthenia gravis diagnosed 2 years ago. Current treatment worsening with movement, and causing her to miss 2 or with daily prednisone and pyridostigmine partially controls 3 days of school per month. The severe headaches have not her symptoms. She is experiencing weakness with pro- responded to ibuprofen or naproxen. The patient also has longed activity and in the evening. She wishes to minimize seasonal allergies. Other medications are fexofenadine and long-term need for glucocorticoids and other medications a combination estrogen-progestin oral contraceptive. that may suppress her immune system. All physical examination findings, including vital signs, On physical examination, vital signs are normal. She are normal. has mild bilateral ptosis and impaired left eye adduction Which of the following is the most appropriate next step in and bilateral eye abduction. Speech is nasal. Proximal upper management? and lower extremity muscle strength and neck flexion are mildly weak. (A) Begin oral zolmitriptan Acetylcholine receptor-binding antibody is positive. (B) Discontinue the oral contraceptive CT of the chest is normal. (C) Obtain a brain MRI Which of the following is the most appropriate (D) Substitute loratadine for fexofenadine treatment? (A) Eculizumab Item 52 ) Ipilimumab A 55-year-old man is evaluated for a 1-year history of wors- (C) Plasma exchange ening personality and behavioral changes, compulsive ten- (D) Reduction in prednisone dose dencies, a loss of empathy, and poor insight. Although he (E) Thymectomy previously was neither a drinker nor asmokKer, he now drinks heavily and smokes two packs of cigarettes daily. According to his son, the patient lost more than half of his retirement Item 55 savings in the past month while gambling, and 2 weeks ago, he was arrested for breaking into a neighbor’s house. Results A 29-year-old man is hospitalized for suicidal ideation. He of recent cognitive screening have been normal. He has no was recently diagnosed with bipolar disorder without psy- other medical problems and takes no medication. chotic features. He has a history of medication-resistant All physical examination findings, including vital signs, generalized tonic-clonic seizures. He has no other medical are unremarkable. problems. His antiepileptic drugs are valproic acid, levetir An MRI of the brain shows atrophy of the right frontal acetam, and lamotrigine. In addition, he is taking queti lobe. apine. The patient has no other medical problems.

(A) Eculizumab Item 52 ) Ipilimumab A 55-year-old man is evaluated for a 1-year history of wors- (C) Plasma exchange ening personality and behavioral changes, compulsive ten- (D) Reduction in prednisone dose dencies, a loss of empathy, and poor insight. Although he (E) Thymectomy previously was neither a drinker nor asmokKer, he now drinks heavily and smokes two packs of cigarettes daily. According to his son, the patient lost more than half of his retirement Item 55 savings in the past month while gambling, and 2 weeks ago, he was arrested for breaking into a neighbor’s house. Results A 29-year-old man is hospitalized for suicidal ideation. He of recent cognitive screening have been normal. He has no was recently diagnosed with bipolar disorder without psy- other medical problems and takes no medication. chotic features. He has a history of medication-resistant All physical examination findings, including vital signs, generalized tonic-clonic seizures. He has no other medical are unremarkable. problems. His antiepileptic drugs are valproic acid, levetir An MRI of the brain shows atrophy of the right frontal acetam, and lamotrigine. In addition, he is taking queti lobe. apine. The patient has no other medical problems. Which of the following classes of drugs is most appropriate Which of the following drugs should be discontinued in the for the pharmacologic treatment of this patient? acute setting for this patient? (A) Acetylcholinesterase inhibitors (A) Lamotrigine (B) Atypical antipsychotic agents (B) Levetiracetam (C) N-Methyl-p-aspartate receptor antagonists (C) Quetiapine (D) Selective serotonin reuptake inhibitors (D) Valproic acid 107

Self-Assessment Test 2) ee scotoma and ipsilateral numbness in the face and hand. => Item 56 bp Photophobia and nausea are intense, and he typically vom- wn A 70-year-old man is evaluated for a 2-day history of agita- wa its within an hour of awakening on migraine days. Oral @ tion. He has not slept and has been rummaging about the wn sumatriptan, oral rizatriptan, and nasal zolmitriptan have wn house. He has dementia due to Alzheimer disease. His care- 3 not relieved the pain. giver reports that he has not started any new medications © All physical examination findings, including vital signs, —] and has not shown any obvious signs of infection or injury. -r are normal. He has not been constipated and has been able to urinate o wn - regularly. The patient is unable to give a symptom history. Which of the following is the most appropriate next step in His only medication is donepezil. management? On physical examination, vital signs are normal and (A Brain MRI other findings are unremarkable. ee A complete blood count and measurement of serum (B) Head CT electrolytes, serum creatinine, and blood urea nitrogen will (C) Hydrocodone be obtained. (D) Subcutaneous sumatriptan

2) ee scotoma and ipsilateral numbness in the face and hand. => Item 56 bp Photophobia and nausea are intense, and he typically vom- wn A 70-year-old man is evaluated for a 2-day history of agita- wa its within an hour of awakening on migraine days. Oral @ tion. He has not slept and has been rummaging about the wn sumatriptan, oral rizatriptan, and nasal zolmitriptan have wn house. He has dementia due to Alzheimer disease. His care- 3 not relieved the pain. giver reports that he has not started any new medications © All physical examination findings, including vital signs, —] and has not shown any obvious signs of infection or injury. -r are normal. He has not been constipated and has been able to urinate o wn - regularly. The patient is unable to give a symptom history. Which of the following is the most appropriate next step in His only medication is donepezil. management? On physical examination, vital signs are normal and (A Brain MRI other findings are unremarkable. ee A complete blood count and measurement of serum (B) Head CT electrolytes, serum creatinine, and blood urea nitrogen will (C) Hydrocodone be obtained. (D) Subcutaneous sumatriptan Which of the following is the most appropriate additional diagnostic test? Item 59 (A) Arterial blood gas analysis A 64-year-old man is evaluated for recent emergence of (B) Head CT scan involuntary twisting movements of his neck and extremi- (C) Lumbar puncture with cerebrospinal fluid analysis ties. Parkinson disease was diagnosed 8 years ago. Mobility (D) Urinalysis and fatigue improve after each dose of carbidopa-levodopa. A few months ago, he started to feel excessive slowness and anxiety 1 hour before each dose of medication and was Item 57 treated with an increased dose of his carbidopa-levodopa. A 27-year-old woman is evaluated for a 10-day history Now, he has developed involuntary nonrhythmic move- of involuntary movements involving the face, arms, ments that peak after each dose. Current medication is and trunk. Symptoms began as occasional twitching of carbidopa-levodopa. the limbs and then became more generalized and per- On physical examination, vital signs are normal. He sistent to the point that now she has difficulty sitting is examined half an hour after taking a dose of levodopa. still. She cannot suppress the movements voluntarily. He exhibits cogwheel rigidity, shuffling of gait, and The patient has no personal or family medical history large-amplitude ballistic and flowing movements of the of involuntary movements. She is otherwise well and neck and extremities. Other examination findings are takes no medications. unremarkable. On physical examination, vital signs are normal. Vari- Which of the following is the most appropriate next step in able and random movements that flow from one body part treatment? to another are noted; the movements are not distractible. Gait has a prancing quality with flailing arms. Cognition (A) Amantadine is intact and motor strength and deep tendon reflexes are (B) Deep-brain stimulation normal. (C) Entacapone A complete blood count and comprehensive metabolic (D) Ropinirole profile are normal. (E) Selegiline Which of the following is the most appropriate diagnostic test?

Which of the following is the most appropriate additional diagnostic test? Item 59 (A) Arterial blood gas analysis A 64-year-old man is evaluated for recent emergence of (B) Head CT scan involuntary twisting movements of his neck and extremi- (C) Lumbar puncture with cerebrospinal fluid analysis ties. Parkinson disease was diagnosed 8 years ago. Mobility (D) Urinalysis and fatigue improve after each dose of carbidopa-levodopa. A few months ago, he started to feel excessive slowness and anxiety 1 hour before each dose of medication and was Item 57 treated with an increased dose of his carbidopa-levodopa. A 27-year-old woman is evaluated for a 10-day history Now, he has developed involuntary nonrhythmic move- of involuntary movements involving the face, arms, ments that peak after each dose. Current medication is and trunk. Symptoms began as occasional twitching of carbidopa-levodopa. the limbs and then became more generalized and per- On physical examination, vital signs are normal. He sistent to the point that now she has difficulty sitting is examined half an hour after taking a dose of levodopa. still. She cannot suppress the movements voluntarily. He exhibits cogwheel rigidity, shuffling of gait, and The patient has no personal or family medical history large-amplitude ballistic and flowing movements of the of involuntary movements. She is otherwise well and neck and extremities. Other examination findings are takes no medications. unremarkable. On physical examination, vital signs are normal. Vari- Which of the following is the most appropriate next step in able and random movements that flow from one body part treatment? to another are noted; the movements are not distractible. Gait has a prancing quality with flailing arms. Cognition (A) Amantadine is intact and motor strength and deep tendon reflexes are (B) Deep-brain stimulation normal. (C) Entacapone A complete blood count and comprehensive metabolic (D) Ropinirole profile are normal. (E) Selegiline Which of the following is the most appropriate diagnostic test? (A) Genetic testing for Huntington disease Item 60 So B) Peripheral blood smear A 78-year-old woman is evaluated in the hospital fol- lowing treatment with intravenous alteplase 8 hours C) Pregnancy test ago for acute ischemic stroke. CT angiogram showed no o Serum ceruloplasmin measurement large vessel occlusion. The National Institutes of Health =

(A) Genetic testing for Huntington disease Item 60 So B) Peripheral blood smear A 78-year-old woman is evaluated in the hospital fol- lowing treatment with intravenous alteplase 8 hours C) Pregnancy test ago for acute ischemic stroke. CT angiogram showed no o Serum ceruloplasmin measurement large vessel occlusion. The National Institutes of Health = aaa E) Spinal fluid analysis Stroke Scale (NIHSS) score before and 1 hour after treat ment was 9. She has atrial fibrillation and hypertension. Outpatient medications were warfarin, enalapril, and Item 58 amlodipine. At the time of hospital admission the INR A 43-year-old man is evaluated for a 6-year history of was 1.0. recurrent, severe migraine occurring every 3 months and On physical examination, vital signs are normal. lasting 2 to 3 days. He describes the migraine pain, which Oxygen saturation is 97% with the patient breathing typically awakens him from sleep, as debilitating, intense, ambient air. She has left facial weakness and dysarthria. throbbing, and unilateral in location (although alternating The raised left arm and leg have a downward drift that sides). The migraine prohibits normal physical activity, contacts the bed, and there is a decreased sensitivity to such as going to work. For 30 to 45 minutes during a pinprick on the left side. Heart rhythm is irregularly migraine episode, he may experience hemifield visual irregular. 108

Self-Assessment Test ot CH Which of the following is the most appropriate next step in (C) Serum aquaporin-4 antibody testing _ _ management? (D) Serum copper level i CONT. = Atorvastatin 5 2) wn Intravenous heparin Item 63 @ wn Oxygen by nasal cannula wn A 64-year-old woman is evaluated for a 3-month history of = Repeat head CT daily headaches of sudden, spontaneous onset. Although = ® SSCRe Swallow evaluation the patient awakens each morning without any pain, a rn

management? (D) Serum copper level i CONT. = Atorvastatin 5 2) wn Intravenous heparin Item 63 @ wn Oxygen by nasal cannula wn A 64-year-old woman is evaluated for a 3-month history of = Repeat head CT daily headaches of sudden, spontaneous onset. Although = ® SSCRe Swallow evaluation the patient awakens each morning without any pain, a rn headache typically develops within 30 minutes after assuming an upright posture; the pain is dull and localized Item 61 at the top of the head but becomes more intense and global throughout the day. Bedrest will ease her symptoms, but A 67-year-old woman is evaluated in the ICU. She is unre- the headaches typically return when assuming the upright sponsive. Three days ago, she was admitted to the hospi- position. tal with acute respiratory distress syndrome secondary to Vital signs and physical examination findings are community-acquired pneumonia and was intubated and normal. mechanically ventilated. Prior to intubation, neurologic A contrast-enhanced MRI of the brain reveals 2 mm of examination was normal. Antibiotics and propofol were cerebellar tonsillar descent and enhanced diffuse smooth initiated. Twelve hours ago, the propofol was discontinued, dural thickening. MRIs of the cervical, thoracic, and lumbar but the patient remains unresponsive. Medical history is spines are normal. significant for hypertension and previous right hemispheric stroke without residual deficits. Prehospitalization medica- Which of the following is the most appropriate treatment of tions were aspirin, atorvastatin, and hydrochlorothiazide;: this patient’s headaches? current medications include ceftriaxone, azithromycin. (A) Acetazolamide On physical examination, vital signs are normal. The patient does not respond to verbal stimuli and does not open (B) Epidural blood patch her eyes or move her limbs when asked, but there is inter- (C) Methylprednisolone mittent twitching of the left upper and lower extremities. (D) Surgical suboccipital decompression She withdraws all extremities to noxious stimuli. A recent metabolic profile is normal, including liver and kidney function. Item 64 A head CT scan shows evidence of remote hemispheric A 60-year-old woman is evaluated for constant movements stroke but no additional findings. of her tongue and lips that she cannot suppress. She also has Which of the following is the most appropriate next step in difficulty with keeping her jaw closed, and food spills out management? of her mouth during meals. The movements started 2 years ago following treatment with metoclopramide for diabetic (A) Brain MRI gastroparesis. She stopped the medication, but movements (B) Continuous electroencephalography persisted. Medical history is also significant for panic disor- (C) Intravenous fosphenytoin der. She has no history of depression and no family history (D) Reinitiation of intravenous propofol of abnormal movements or dementia. Current medications are metformin, sitagliptin, canagliflozin, and clonazepam. On physical examination, vital signs are normal. She displays frequent, fast, and variable twitching of the lips and Item 62 facial muscles and intermittent tongue protrusion. She can- A 53-year-old woman was admitted to the hospital for not keep her mouth open more than a few seconds because bilateral optic neuritis. She is receiving intravenous meth- of forced closure of the jaw, with associated hyperactivity ylprednisolone. of the masseter muscles. Minimal writhing finger flexion On physical examination, vital signs are normal. Visual movements are noted during ambulation. acuity is 20/200 in both eyes. Reflexes are 3+ in the lower MRI of the brain is unremarkable. extremities, and plantar response is extensor. The remain- der of the examination is normal. Which of the following is the most appropriate treatment? An MRI ofthe thoracic spine shows an extensive hyperin- (A) Add aripiprazole tense lesion in the thoracic cord from T3 to T7 with mild con- (B) Add ropinirole trast enhancement. An MRI of the orbits shows hyperintensity in the anterior portion of the optic nerve bilaterally with con- ( C) Add valbenazine trast enhancement. Cerebrospinal fluid testing shows leuko- ( ) Begin cognitive behavioral therapy o

headache typically develops within 30 minutes after assuming an upright posture; the pain is dull and localized Item 61 at the top of the head but becomes more intense and global throughout the day. Bedrest will ease her symptoms, but A 67-year-old woman is evaluated in the ICU. She is unre- the headaches typically return when assuming the upright sponsive. Three days ago, she was admitted to the hospi- position. tal with acute respiratory distress syndrome secondary to Vital signs and physical examination findings are community-acquired pneumonia and was intubated and normal. mechanically ventilated. Prior to intubation, neurologic A contrast-enhanced MRI of the brain reveals 2 mm of examination was normal. Antibiotics and propofol were cerebellar tonsillar descent and enhanced diffuse smooth initiated. Twelve hours ago, the propofol was discontinued, dural thickening. MRIs of the cervical, thoracic, and lumbar but the patient remains unresponsive. Medical history is spines are normal. significant for hypertension and previous right hemispheric stroke without residual deficits. Prehospitalization medica- Which of the following is the most appropriate treatment of tions were aspirin, atorvastatin, and hydrochlorothiazide;: this patient’s headaches? current medications include ceftriaxone, azithromycin. (A) Acetazolamide On physical examination, vital signs are normal. The patient does not respond to verbal stimuli and does not open (B) Epidural blood patch her eyes or move her limbs when asked, but there is inter- (C) Methylprednisolone mittent twitching of the left upper and lower extremities. (D) Surgical suboccipital decompression She withdraws all extremities to noxious stimuli. A recent metabolic profile is normal, including liver and kidney function. Item 64 A head CT scan shows evidence of remote hemispheric A 60-year-old woman is evaluated for constant movements stroke but no additional findings. of her tongue and lips that she cannot suppress. She also has Which of the following is the most appropriate next step in difficulty with keeping her jaw closed, and food spills out management? of her mouth during meals. The movements started 2 years ago following treatment with metoclopramide for diabetic (A) Brain MRI gastroparesis. She stopped the medication, but movements (B) Continuous electroencephalography persisted. Medical history is also significant for panic disor- (C) Intravenous fosphenytoin der. She has no history of depression and no family history (D) Reinitiation of intravenous propofol of abnormal movements or dementia. Current medications are metformin, sitagliptin, canagliflozin, and clonazepam. On physical examination, vital signs are normal. She displays frequent, fast, and variable twitching of the lips and Item 62 facial muscles and intermittent tongue protrusion. She can- A 53-year-old woman was admitted to the hospital for not keep her mouth open more than a few seconds because bilateral optic neuritis. She is receiving intravenous meth- of forced closure of the jaw, with associated hyperactivity ylprednisolone. of the masseter muscles. Minimal writhing finger flexion On physical examination, vital signs are normal. Visual movements are noted during ambulation. acuity is 20/200 in both eyes. Reflexes are 3+ in the lower MRI of the brain is unremarkable. extremities, and plantar response is extensor. The remain- der of the examination is normal. Which of the following is the most appropriate treatment? An MRI ofthe thoracic spine shows an extensive hyperin- (A) Add aripiprazole tense lesion in the thoracic cord from T3 to T7 with mild con- (B) Add ropinirole trast enhancement. An MRI of the orbits shows hyperintensity in the anterior portion of the optic nerve bilaterally with con- ( C) Add valbenazine trast enhancement. Cerebrospinal fluid testing shows leuko- ( ) Begin cognitive behavioral therapy o cytes (180/uL [180 x 10°/L) composed mostly of neutrophils. ( E) Discontinue clonazepam An MRI of the brain is normal.

headache typically develops within 30 minutes after assuming an upright posture; the pain is dull and localized Item 61 at the top of the head but becomes more intense and global throughout the day. Bedrest will ease her symptoms, but A 67-year-old woman is evaluated in the ICU. She is unre- the headaches typically return when assuming the upright sponsive. Three days ago, she was admitted to the hospi- position. tal with acute respiratory distress syndrome secondary to Vital signs and physical examination findings are community-acquired pneumonia and was intubated and normal. mechanically ventilated. Prior to intubation, neurologic A contrast-enhanced MRI of the brain reveals 2 mm of examination was normal. Antibiotics and propofol were cerebellar tonsillar descent and enhanced diffuse smooth initiated. Twelve hours ago, the propofol was discontinued, dural thickening. MRIs of the cervical, thoracic, and lumbar but the patient remains unresponsive. Medical history is spines are normal. significant for hypertension and previous right hemispheric stroke without residual deficits. Prehospitalization medica- Which of the following is the most appropriate treatment of tions were aspirin, atorvastatin, and hydrochlorothiazide;: this patient’s headaches? current medications include ceftriaxone, azithromycin. (A) Acetazolamide On physical examination, vital signs are normal. The patient does not respond to verbal stimuli and does not open (B) Epidural blood patch her eyes or move her limbs when asked, but there is inter- (C) Methylprednisolone mittent twitching of the left upper and lower extremities. (D) Surgical suboccipital decompression She withdraws all extremities to noxious stimuli. A recent metabolic profile is normal, including liver and kidney function. Item 64 A head CT scan shows evidence of remote hemispheric A 60-year-old woman is evaluated for constant movements stroke but no additional findings. of her tongue and lips that she cannot suppress. She also has Which of the following is the most appropriate next step in difficulty with keeping her jaw closed, and food spills out management? of her mouth during meals. The movements started 2 years ago following treatment with metoclopramide for diabetic (A) Brain MRI gastroparesis. She stopped the medication, but movements (B) Continuous electroencephalography persisted. Medical history is also significant for panic disor- (C) Intravenous fosphenytoin der. She has no history of depression and no family history (D) Reinitiation of intravenous propofol of abnormal movements or dementia. Current medications are metformin, sitagliptin, canagliflozin, and clonazepam. On physical examination, vital signs are normal. She displays frequent, fast, and variable twitching of the lips and Item 62 facial muscles and intermittent tongue protrusion. She can- A 53-year-old woman was admitted to the hospital for not keep her mouth open more than a few seconds because bilateral optic neuritis. She is receiving intravenous meth- of forced closure of the jaw, with associated hyperactivity ylprednisolone. of the masseter muscles. Minimal writhing finger flexion On physical examination, vital signs are normal. Visual movements are noted during ambulation. acuity is 20/200 in both eyes. Reflexes are 3+ in the lower MRI of the brain is unremarkable. extremities, and plantar response is extensor. The remain- der of the examination is normal. Which of the following is the most appropriate treatment? An MRI ofthe thoracic spine shows an extensive hyperin- (A) Add aripiprazole tense lesion in the thoracic cord from T3 to T7 with mild con- (B) Add ropinirole trast enhancement. An MRI of the orbits shows hyperintensity in the anterior portion of the optic nerve bilaterally with con- ( C) Add valbenazine trast enhancement. Cerebrospinal fluid testing shows leuko- ( ) Begin cognitive behavioral therapy o cytes (180/uL [180 x 10°/L) composed mostly of neutrophils. ( E) Discontinue clonazepam An MRI of the brain is normal. Which of the following is the most appropriate management? Item 65 (A) Interferon beta A 41-year-old woman is evaluated for a 2-day history of (B) Oligoclonal band testing worsening imbalance and clumsiness of the left arm. She

Which of the following is the most appropriate management? Item 65 (A) Interferon beta A 41-year-old woman is evaluated for a 2-day history of (B) Oligoclonal band testing worsening imbalance and clumsiness of the left arm. She 109

Self-Assessment Test w 2 = has relapsing-remitting multiple sclerosis. Medications are (D) Surgical resection D> teriflunomide and a vitamin D supplement. wn (E) Whole-brain radiation n CONT. ) On physical examination, vital signs are normal. Neu- wn rologic examination shows new findings of dysmetria and 3 rs) tremor of the left arm. The patient has a wide-based, ataxic Item 68 = gait and is unable to perform tandem gait. oe A 45-year-old woman is evaluated for sleeping difficulties. An MRI of the brain shows multiple periventricular and =i Her main symptom is an uncomfortable urge to move her o juxtacortical lesions and one lesion in the left cerebellum. wn [on al legs when she is at rest or in bed at night. This may result in The cerebellar lesion enhances with contrast administration. difficulty falling asleep or sometimes in maintaining sleep. Which of the following is the most appropriate treatment? She may wake up three to five times a night and then walk around the house for a few minutes to relieve her symp- (A) Intramuscular adrenocorticotropin hormone gel toms before attempting to go back to sleep. Her husband B) Intravenous glucocorticoids reports no snoring or witnessed apneic episodes or daytime ) Intravenous immunoglobulin sleepiness.

w 2 = has relapsing-remitting multiple sclerosis. Medications are (D) Surgical resection D> teriflunomide and a vitamin D supplement. wn (E) Whole-brain radiation n CONT. ) On physical examination, vital signs are normal. Neu- wn rologic examination shows new findings of dysmetria and 3 rs) tremor of the left arm. The patient has a wide-based, ataxic Item 68 = gait and is unable to perform tandem gait. oe A 45-year-old woman is evaluated for sleeping difficulties. An MRI of the brain shows multiple periventricular and =i Her main symptom is an uncomfortable urge to move her o juxtacortical lesions and one lesion in the left cerebellum. wn [on al legs when she is at rest or in bed at night. This may result in The cerebellar lesion enhances with contrast administration. difficulty falling asleep or sometimes in maintaining sleep. Which of the following is the most appropriate treatment? She may wake up three to five times a night and then walk around the house for a few minutes to relieve her symp- (A) Intramuscular adrenocorticotropin hormone gel toms before attempting to go back to sleep. Her husband B) Intravenous glucocorticoids reports no snoring or witnessed apneic episodes or daytime ) Intravenous immunoglobulin sleepiness. D) Ocrelizumab infusion On physical examination, vital signs and the remainder of the physical examination are normal. (E) Plasmapheresis Which of the following is the most appropriate manage- ment? Item 66 (A) Cognitive behavioral therapy for insomnia A 34-year-old woman is hospitalized for right-sided visual (B) Ferritin measurement loss of 2 days’ duration. She has no headache, eye pain, or neck pain. She has had two prior pregnancies without com (C) Polysomnography plications. She has no other symptoms and is otherwise well. (D) Zolpidem On physical examination, vital signs are normal. Right visual field deficit in both eyes is observed. Pupils are equal in size and are reactive to light; she has full eye movements. Item 69 Complete blood count, coagulation profile, and C- A 49-year-old woman is evaluated for a 3-year history reactive protein level are normal. of slowly worsening bilateral lower extremity weakness, CT scan of the head showsa left occipital infarct. Mag- fatigue, and numbness in the hands. She had a single epi- netic resonance angiogram of the head and neck and ECG sode of unilateral optic neuritis 8 years ago that resolved are normal. following administration of glucocorticoids. She has no other medical problems and takes no medications. Which of the following is the most appropriate diagnostic On physical examination, vital signs are normal. Mus- test to perform next? cle strength testing shows 4/5 bilateral lower extremity (A) Catheter angiography weakness. There is 3+ hyperreflexia throughout in both (B) Lumbar puncture lower extremities and extensor plantar response. MRI of the brain shows five periventricular white mat- (C) Transesophageal echocardiography ter hyperintensities, two with contrast enhancement. A (D) Transthoracic echocardiography with agitated saline T2-weighted MRI of the cervical and thoracic spine shows multiple hyperintensities without contrast enhancement. A diagnosis of multiple sclerosis is established. Item 67 An 82-year-old woman is evaluated in the emergency Which of the following best describes the status of this department for a 10-week history of a gradual and steady patient’s multiple sclerosis? progression of new-onset headaches. She is otherwise well (A) Primary progressive, with progression but without and takes no mediations. activity On physical examination, vital signs are normal. Neur- ologic examination is normal. (B) Relapsing remitting, with activity A T2-weighted MRI of the brain with contrast shows (C) Relapsing remitting, without activity a well-demarcated, hyperintense lesion arising from lat- (D) Secondary progressive, with progression and activity eral ventricles anteriorly and extending bilaterally into the (E) Secondary progressive, with progression but without frontal lobes. The lesion shows gadolinium enhancement on activity Tl-weighted imaging. Vitreous and cerebrospinal fluid sampling are negative for lymphomatous cells. Item 70 Which of the following is the most appropriate next step in A 51-year-old woman is evaluated for a 5-year history of management? increasingly frequent and disabling migraine episodes. Whereas she previously had migraine on the first 2 days of (A) Brain biopsy menses, migraine episodes now have increased to 10 days (B) Dexamethasone monthly; once monthly, she experiences a scotoma lasting (C) Methotrexate 20 minutes before migraine onset. Oral and subcutaneous

D) Ocrelizumab infusion On physical examination, vital signs and the remainder of the physical examination are normal. (E) Plasmapheresis Which of the following is the most appropriate manage- ment? Item 66 (A) Cognitive behavioral therapy for insomnia A 34-year-old woman is hospitalized for right-sided visual (B) Ferritin measurement loss of 2 days’ duration. She has no headache, eye pain, or neck pain. She has had two prior pregnancies without com (C) Polysomnography plications. She has no other symptoms and is otherwise well. (D) Zolpidem On physical examination, vital signs are normal. Right visual field deficit in both eyes is observed. Pupils are equal in size and are reactive to light; she has full eye movements. Item 69 Complete blood count, coagulation profile, and C- A 49-year-old woman is evaluated for a 3-year history reactive protein level are normal. of slowly worsening bilateral lower extremity weakness, CT scan of the head showsa left occipital infarct. Mag- fatigue, and numbness in the hands. She had a single epi- netic resonance angiogram of the head and neck and ECG sode of unilateral optic neuritis 8 years ago that resolved are normal. following administration of glucocorticoids. She has no other medical problems and takes no medications. Which of the following is the most appropriate diagnostic On physical examination, vital signs are normal. Mus- test to perform next? cle strength testing shows 4/5 bilateral lower extremity (A) Catheter angiography weakness. There is 3+ hyperreflexia throughout in both (B) Lumbar puncture lower extremities and extensor plantar response. MRI of the brain shows five periventricular white mat- (C) Transesophageal echocardiography ter hyperintensities, two with contrast enhancement. A (D) Transthoracic echocardiography with agitated saline T2-weighted MRI of the cervical and thoracic spine shows multiple hyperintensities without contrast enhancement. A diagnosis of multiple sclerosis is established. Item 67 An 82-year-old woman is evaluated in the emergency Which of the following best describes the status of this department for a 10-week history of a gradual and steady patient’s multiple sclerosis? progression of new-onset headaches. She is otherwise well (A) Primary progressive, with progression but without and takes no mediations. activity On physical examination, vital signs are normal. Neur- ologic examination is normal. (B) Relapsing remitting, with activity A T2-weighted MRI of the brain with contrast shows (C) Relapsing remitting, without activity a well-demarcated, hyperintense lesion arising from lat- (D) Secondary progressive, with progression and activity eral ventricles anteriorly and extending bilaterally into the (E) Secondary progressive, with progression but without frontal lobes. The lesion shows gadolinium enhancement on activity Tl-weighted imaging. Vitreous and cerebrospinal fluid sampling are negative for lymphomatous cells. Item 70 Which of the following is the most appropriate next step in A 51-year-old woman is evaluated for a 5-year history of management? increasingly frequent and disabling migraine episodes. Whereas she previously had migraine on the first 2 days of (A) Brain biopsy menses, migraine episodes now have increased to 10 days (B) Dexamethasone monthly; once monthly, she experiences a scotoma lasting (C) Methotrexate 20 minutes before migraine onset. Oral and subcutaneous 110

Self-Assessment Test ent cH sumatriptan have been effective in controlling symptoms, (C) Labetalol po ant but because of the increased frequency of her headaches she (D) Nitroprusside = <P) sometimes runs out of medication during the month. Pre- £ ventive trials of propranolol, amitriptyline, and topiramate wn wn C) over the past 2 years have been unsuccessful. Item 73 <8) wn All physical examination findings, including vital signs, 2) A 32-year-old woman is hospitalized for recent-onset leg = are normal. = weakness. She reports development of pins-and-needles Results of laboratory studies are within normal limits. C.F) sensation in her upper and lower extremities 10 days ago, ”

C) over the past 2 years have been unsuccessful. Item 73 <8) wn All physical examination findings, including vital signs, 2) A 32-year-old woman is hospitalized for recent-onset leg = are normal. = weakness. She reports development of pins-and-needles Results of laboratory studies are within normal limits. C.F) sensation in her upper and lower extremities 10 days ago, ” Which of the following is the most appropriate next step in followed by low back pain and progressive weakness in her preventive treatment? lower extremities. She has otherwise been well and reports no other symptoms. She received an influenza vaccination = Erenumab a month ago. She takes no medications. Ethinyl estradiol On physical examination, temperature is 37.2 °C Oo. Onabotulinum toxin A (98.9 °F), blood pressure is 105/70 mm Hg, pulse rate is Sertraline 115/min, respiration rate is 18/min. Moderate weakness in hip flexors and mild weakness in foot dorsiflexors and plan- mo Verapamil tar flexors bilaterally are observed. Deep tendon reflexes are —

Which of the following is the most appropriate next step in followed by low back pain and progressive weakness in her preventive treatment? lower extremities. She has otherwise been well and reports no other symptoms. She received an influenza vaccination = Erenumab a month ago. She takes no medications. Ethinyl estradiol On physical examination, temperature is 37.2 °C Oo. Onabotulinum toxin A (98.9 °F), blood pressure is 105/70 mm Hg, pulse rate is Sertraline 115/min, respiration rate is 18/min. Moderate weakness in hip flexors and mild weakness in foot dorsiflexors and plan- mo Verapamil tar flexors bilaterally are observed. Deep tendon reflexes are — absent throughout. Cranial nerves, including extraocular movements, are intact. Plantar responses are flexor. Sen Item 71 sory perception is intact to all modalities. A 25-year-old woman is evaluated during a follow-up visit Cerebrospinal fluid cell count and protein and glucose for a generalized tonic-clonic seizure diagnosed yesterday levels are normal. after a comprehensive evaluation. She reports having a sim- Electromyogram shows no neuropathy, radiculopathy, ilar seizure 1 year ago for which she did not seek medical or myopathy. attention. She also reports that for the past year she has had Cervical, thoracic, and lumbar MRI findings are unre brief episodes of muscle twitches on awakening that cause markable. her to drop her coffee cup. She has no other medical prob- lems and takes no medications. She is planning a pregnancy. Which of the following is the most appropriate treatment? CT scan of the head without contrast and follow-up (A) Doxycycline MRI obtained yesterday were normal. An electroenceph- (B) Intravenous glucocorticoids alogram showed 4- to 6-Hz spike-and-wave discharges consistent with generalized epilepsy. (C) Plasma exchange Pregnancy test is negative. (D) No additional treatment

absent throughout. Cranial nerves, including extraocular movements, are intact. Plantar responses are flexor. Sen Item 71 sory perception is intact to all modalities. A 25-year-old woman is evaluated during a follow-up visit Cerebrospinal fluid cell count and protein and glucose for a generalized tonic-clonic seizure diagnosed yesterday levels are normal. after a comprehensive evaluation. She reports having a sim- Electromyogram shows no neuropathy, radiculopathy, ilar seizure 1 year ago for which she did not seek medical or myopathy. attention. She also reports that for the past year she has had Cervical, thoracic, and lumbar MRI findings are unre brief episodes of muscle twitches on awakening that cause markable. her to drop her coffee cup. She has no other medical prob- lems and takes no medications. She is planning a pregnancy. Which of the following is the most appropriate treatment? CT scan of the head without contrast and follow-up (A) Doxycycline MRI obtained yesterday were normal. An electroenceph- (B) Intravenous glucocorticoids alogram showed 4- to 6-Hz spike-and-wave discharges consistent with generalized epilepsy. (C) Plasma exchange Pregnancy test is negative. (D) No additional treatment Which of the following is the most appropriate manage- ment? Item 74 (A) Gabapentin A 78-year-old man is evaluated for two generalized tonic- (B) Levetiracetam clonic seizures that occurred 2 weeks apart during the past (C) Topiramate month. Medical history includes hypertension and type 2 diabetes mellitus and a recent diagnosis of mild cognitive (D) Valproic acid impairment. Medications are hydrochlorothiazide and met- formin. On physical examination, vital signs are normal. All Item 72 other examination findings, including those from a neuro- A 57-year-old woman is evaluated in the emergency depart logic examination, are unremarkable. ment for a 70-minute history of right-sided weakness Serum creatinine, electrolytes, and liver chemistry involving the face, arm, and leg. She has had no surgeries or tests are normal. recent gastrointestinal or genitourinary bleeding. Medical Findings on brain MRI and electroencephalography history is significant for hypertension and type 2 diabetes are normal. mellitus. Medications are metformin and lisinopril. On physical examination, blood pressure is 192/122 mm Which of the following is the most appropriate treatment? Hg; the remaining vital signs are normal. She has left facial (A) Lamotrigine weakness, dysarthria, and left arm and leg weakness with a (B) Oxcarbazepine downward drift, with the leg not touching the examination table. (C) Valproic acid ECG is normal. CT of the head shows no sign of hemor (D) No seizure medication is necessary rhage or early infarcts.

Which of the following is the most appropriate manage- ment? Item 74 (A) Gabapentin A 78-year-old man is evaluated for two generalized tonic- (B) Levetiracetam clonic seizures that occurred 2 weeks apart during the past (C) Topiramate month. Medical history includes hypertension and type 2 diabetes mellitus and a recent diagnosis of mild cognitive (D) Valproic acid impairment. Medications are hydrochlorothiazide and met- formin. On physical examination, vital signs are normal. All Item 72 other examination findings, including those from a neuro- A 57-year-old woman is evaluated in the emergency depart logic examination, are unremarkable. ment for a 70-minute history of right-sided weakness Serum creatinine, electrolytes, and liver chemistry involving the face, arm, and leg. She has had no surgeries or tests are normal. recent gastrointestinal or genitourinary bleeding. Medical Findings on brain MRI and electroencephalography history is significant for hypertension and type 2 diabetes are normal. mellitus. Medications are metformin and lisinopril. On physical examination, blood pressure is 192/122 mm Which of the following is the most appropriate treatment? Hg; the remaining vital signs are normal. She has left facial (A) Lamotrigine weakness, dysarthria, and left arm and leg weakness with a (B) Oxcarbazepine downward drift, with the leg not touching the examination table. (C) Valproic acid ECG is normal. CT of the head shows no sign of hemor (D) No seizure medication is necessary rhage or early infarcts. Which of the following is the most appropriate initial intra- Item 75 venous treatment? A 58-year-old man is evaluated for left lower extremity (A) Abciximab weakness. Three months ago, severe pain in the left buttock (B) Alteplase developed. The pain has since diminished, but progressive

Which of the following is the most appropriate initial intra- Item 75 venous treatment? A 58-year-old man is evaluated for left lower extremity (A) Abciximab weakness. Three months ago, severe pain in the left buttock (B) Alteplase developed. The pain has since diminished, but progressive 111

Self-Assessment Test rn @ oss left-thigh weakness and muscle wasting are now present. has hypertension and dyslipidemia. She has had no recent b= surgery or history of gastrointestinal, genitourinary, or wn He has hypothyroidism, type 2 diabetes, and alcohol use dis- wn Oo order. Current medications are levothyroxine, metformin, intracranial bleeding. Medications are amlodipine and ator- wn wn and canagliflozin. vastatin. 3 On physical examination, vital signs are normal. BMI On physical examination, blood pressure is 168/92 mm @ 1] is 20. There is tenderness to touch over the left buttock and Hg; the remaining vital signs are normal. Oxygen satu- ration is 97% with the patient breathing ambient air. Left Cal inner thigh. Sensory perception to light touch and pin- o vn prick is diminished over the left medial thigh and dorsum facial weakness, dysarthria, decreased sensation to pin- - of the left foot. Left anterior thigh muscles are weak and prick on the left side of the body, and left arm and leg drift wasted. Fasciculation is noted in left quadriceps and lumbar are noted. A bedside swallow examination is positive for paraspinal muscles. Examination of the toes reveals a flexor dysphagia. Her score on the National Institutes of Health plantar response. Patellar and Achilles reflexes are absent Stroke Scale is 6. Neurologic examination reveals a normal on the left side and diminished on the right side. The rest of mental status. The remainder of the physical examination the neurologic examination is normal. is normal. Laboratory studies show a hemoglobin A,,. level of ACT scan of the head without contrast shows no intra- 6.9% and thyroid-stimulating hormone level of 5.2 wU/mL cerebral hemorrhages or early hypodensities. (5.2 mU/L). Which of the following is the most appropriate treatment? Electromyogram reveals multifocal sensorimotor axon loss and denervation affecting the proximal more than the (A) Intravenous alteplase distal left lower extremity. (B) Intravenous low-molecular-weight heparin MRI of lumbar spine and CT scan of abdomen and pel- (C) Intravenous nicardipine vis are unremarkable. (D) Oral aspirin Which of the following is the most likely diagnosis?

rn @ oss left-thigh weakness and muscle wasting are now present. has hypertension and dyslipidemia. She has had no recent b= surgery or history of gastrointestinal, genitourinary, or wn He has hypothyroidism, type 2 diabetes, and alcohol use dis- wn Oo order. Current medications are levothyroxine, metformin, intracranial bleeding. Medications are amlodipine and ator- wn wn and canagliflozin. vastatin. 3 On physical examination, vital signs are normal. BMI On physical examination, blood pressure is 168/92 mm @ 1] is 20. There is tenderness to touch over the left buttock and Hg; the remaining vital signs are normal. Oxygen satu- ration is 97% with the patient breathing ambient air. Left Cal inner thigh. Sensory perception to light touch and pin- o vn prick is diminished over the left medial thigh and dorsum facial weakness, dysarthria, decreased sensation to pin- - of the left foot. Left anterior thigh muscles are weak and prick on the left side of the body, and left arm and leg drift wasted. Fasciculation is noted in left quadriceps and lumbar are noted. A bedside swallow examination is positive for paraspinal muscles. Examination of the toes reveals a flexor dysphagia. Her score on the National Institutes of Health plantar response. Patellar and Achilles reflexes are absent Stroke Scale is 6. Neurologic examination reveals a normal on the left side and diminished on the right side. The rest of mental status. The remainder of the physical examination the neurologic examination is normal. is normal. Laboratory studies show a hemoglobin A,,. level of ACT scan of the head without contrast shows no intra- 6.9% and thyroid-stimulating hormone level of 5.2 wU/mL cerebral hemorrhages or early hypodensities. (5.2 mU/L). Which of the following is the most appropriate treatment? Electromyogram reveals multifocal sensorimotor axon loss and denervation affecting the proximal more than the (A) Intravenous alteplase distal left lower extremity. (B) Intravenous low-molecular-weight heparin MRI of lumbar spine and CT scan of abdomen and pel- (C) Intravenous nicardipine vis are unremarkable. (D) Oral aspirin Which of the following is the most likely diagnosis? (A) Alcoholic polyneuropathy Item 78 (B) Autoimmune ganglionopathy A 48-year-old man is seen following a self-referred carotid (C) Diabetic amyotrophy ultrasonography. He has type 2 diabetes mellitus. Current (D) Hypothyroid plexopathy medications are atorvastatin and metformin. On physical examination, blood pressure is 134/68 mm Hg; the remaining vital signs are normal. General physical Item 76 examination reveals a right carotid bruit. The screening A 58-year-old man is evaluated for disabling rhythmic neurologic examination is normal. movements involving his upper extremities. These move- A carotid duplex ultrasound shows right internal ments started 8 years ago and now interfere with his job as carotid artery stenosis of 60% to 80%, without acoustic a chemist. He reports that his handwriting has become illeg- shadowing. ible. Previous treatment with propranolol, primidone, and Which of the following is the most appropriate manage- clonazepam did not improve these symptoms. He also has a ment? history of kidney stones. Family history includes tremor in his brother and Parkinson disease in his uncle. He takes no (A) Carotid endarterectomy medications. (B) Carotid stenting On physical examination, vital signs are normal. The (C) CT angiography of neck patient’s voice quivers during prolonged vowels. Bilat- eral upper extremities exhibit a high-amplitude rhythmic (D) No further testing or intervention movement during the finger-to-nose task and with out- stretched position. There are no involuntary movements when hands are placed on the chair arms. Rapid alternative Item 79 movements, tone, and gait are normal. A 20-year-old man is evaluated for a 2-year history of

(A) Alcoholic polyneuropathy Item 78 (B) Autoimmune ganglionopathy A 48-year-old man is seen following a self-referred carotid (C) Diabetic amyotrophy ultrasonography. He has type 2 diabetes mellitus. Current (D) Hypothyroid plexopathy medications are atorvastatin and metformin. On physical examination, blood pressure is 134/68 mm Hg; the remaining vital signs are normal. General physical Item 76 examination reveals a right carotid bruit. The screening A 58-year-old man is evaluated for disabling rhythmic neurologic examination is normal. movements involving his upper extremities. These move- A carotid duplex ultrasound shows right internal ments started 8 years ago and now interfere with his job as carotid artery stenosis of 60% to 80%, without acoustic a chemist. He reports that his handwriting has become illeg- shadowing. ible. Previous treatment with propranolol, primidone, and Which of the following is the most appropriate manage- clonazepam did not improve these symptoms. He also has a ment? history of kidney stones. Family history includes tremor in his brother and Parkinson disease in his uncle. He takes no (A) Carotid endarterectomy medications. (B) Carotid stenting On physical examination, vital signs are normal. The (C) CT angiography of neck patient’s voice quivers during prolonged vowels. Bilat- eral upper extremities exhibit a high-amplitude rhythmic (D) No further testing or intervention movement during the finger-to-nose task and with out- stretched position. There are no involuntary movements when hands are placed on the chair arms. Rapid alternative Item 79 movements, tone, and gait are normal. A 20-year-old man is evaluated for a 2-year history of Which of the following is the most appropriate treat- migraine that began when he started college. Migraine

(A) Alcoholic polyneuropathy Item 78 (B) Autoimmune ganglionopathy A 48-year-old man is seen following a self-referred carotid (C) Diabetic amyotrophy ultrasonography. He has type 2 diabetes mellitus. Current (D) Hypothyroid plexopathy medications are atorvastatin and metformin. On physical examination, blood pressure is 134/68 mm Hg; the remaining vital signs are normal. General physical Item 76 examination reveals a right carotid bruit. The screening A 58-year-old man is evaluated for disabling rhythmic neurologic examination is normal. movements involving his upper extremities. These move- A carotid duplex ultrasound shows right internal ments started 8 years ago and now interfere with his job as carotid artery stenosis of 60% to 80%, without acoustic a chemist. He reports that his handwriting has become illeg- shadowing. ible. Previous treatment with propranolol, primidone, and Which of the following is the most appropriate manage- clonazepam did not improve these symptoms. He also has a ment? history of kidney stones. Family history includes tremor in his brother and Parkinson disease in his uncle. He takes no (A) Carotid endarterectomy medications. (B) Carotid stenting On physical examination, vital signs are normal. The (C) CT angiography of neck patient’s voice quivers during prolonged vowels. Bilat- eral upper extremities exhibit a high-amplitude rhythmic (D) No further testing or intervention movement during the finger-to-nose task and with out- stretched position. There are no involuntary movements when hands are placed on the chair arms. Rapid alternative Item 79 movements, tone, and gait are normal. A 20-year-old man is evaluated for a 2-year history of Which of the following is the most appropriate treat- migraine that began when he started college. Migraine ment? episodes occur 6 to 12 days per month, with more frequent episodes associated with increased stress from difficult (A) Botulinum toxin injection assignments or final examinations. ) Deep-brain stimulation The migraine pain is typically bifrontal and steady, (C) Levodopa becoming severe when he bends forward. When the pain is severe, he also experiences sensitivities to light and noise (D) Occupational therapy but has no visual aura, nausea, or vomiting; neck pain and (E) Topiramate a slight vertiginous sensation may accompany the most intense episodes. Ibuprofen, naproxen, or diclofenac pro- vides only limited pain relief. He has had no other medical Item 77 problems or symptoms. A 72-year-old woman is evaluated in the emergency depart All physical examination findings, including vital signs, ment for a 1-hour history of left-sided weakness. The patient are unremarkable.

ment? episodes occur 6 to 12 days per month, with more frequent episodes associated with increased stress from difficult (A) Botulinum toxin injection assignments or final examinations. ) Deep-brain stimulation The migraine pain is typically bifrontal and steady, (C) Levodopa becoming severe when he bends forward. When the pain is severe, he also experiences sensitivities to light and noise (D) Occupational therapy but has no visual aura, nausea, or vomiting; neck pain and (E) Topiramate a slight vertiginous sensation may accompany the most intense episodes. Ibuprofen, naproxen, or diclofenac pro- vides only limited pain relief. He has had no other medical Item 77 problems or symptoms. A 72-year-old woman is evaluated in the emergency depart All physical examination findings, including vital signs, ment for a 1-hour history of left-sided weakness. The patient are unremarkable. 112