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Headache and Facial Pain significant morbidity. In the presence of a normal neurologic examination, the following features increase the likelihood of e Head CT is indicated in the assessment of acute, severe a diagnosis of SAH: age 40 years or older, onset during exer- headaches. and brain MRI is used when indicated for tion, witnessed loss of consciousness, or concomitant neck subacute or chronic headaches, given its greater sensi- pain. Estimates vary widely, but many patients describe a past tivity and safety. headache consistent with a sentinel leak within the previous 1 ° Stable headaches meeting criteria for most primary to 2 weeks; these leaks typically involve abrupt, severe, tran- headaches usually do not require imaging. sient headaches that may be focal or global. Sensitivity of head CT is approximately 95% at 12 hours but decreases to 50% at 1 week. Lumbar puncture in SAH typically reveals a CSF eryth- Secondary Headache rocyte count greater than 10,000/uL (10,000 x 10°/L) and an Thunderclap Headache elevation in protein level. CSF xanthochromia may take Thunderclap headache is defined as a severe attack of head- 4 hours or more to develop but is nearly 100% sensitive ache pain developing abruptly and reaching maximum inten- between 12 hours and 7 days. Urgent neurosurgical consulta- sity within 1 minute. Although occasionally of primary origin, tion is required (see Stroke chapter). thunderclap headache is a medical emergency that warrants Headache is the most common symptom and may be the immediate diagnostic evaluation. Noncontrast head CT should only presenting manifestation with thrombosis of the intracra- be performed without delay. Lumbar puncture with measure- nial veins and sinuses. The pain is typically of sudden onset and ment of opening pressure, cell counts, and xanthochromia is unremitting. Clinical symptoms may be related to the result- should be performed if the head CT is nondiagnostic. Contrast- ing increase in intracranial pressure and include pain exacer- enhanced brain MRI and noninvasive vascular imaging (mag- bation with the Valsalva maneuver, pulsatile tinnitus, and netic resonance angiography [MRA] or CT angiography [CTA]) diplopia. Nonspecific findings of increased intracranial pres- of cranial and cervical vessels should be performed in patients sure include papilledema, decreased mentation, and seizures. with a normal CT scan and normal results of cerebrospinal Abducens nerve (cranial nerve VI) palsy secondary to increased fluid (CSF) analysis. Table 2 lists essential considerations in intracranial pressure may falsely indicate a focal lesion (falsely the differential diagnosis. localizing abducens nerve palsy). Specific findings may help Subarachnoid hemorrhage (SAH), the most common localize the thrombosis. Cavernous sinus thrombosis usually cause of thunderclap headache, is discovered in nearly 25% of involves extension of dental or sinus bacterial infection and affected patients. The headache associated with SAH may be manifests as acute-onset headache, proptosis, periorbital described as the worst the patient has ever experienced, edema, and ophthalmoplegia; this disorder requires immediate although the specificity of this finding is limited. Mortality is administration of antibiotics, often with surgical drainage. 50%, with an additional 25% of affected patients experiencing Other forms of cerebral venous thrombosis are not related to infection and often occur in the presence of hypercoagulable TABLE 2. Possible Causes of Thunderclap Headache states, thrombophilia, pregnancy (and the puerperium), oral | Secondary Headache Disorders contraceptive use, or dehydration. Anticoagulation is recom-

significant morbidity. In the presence of a normal neurologic examination, the following features increase the likelihood of e Head CT is indicated in the assessment of acute, severe a diagnosis of SAH: age 40 years or older, onset during exer- headaches. and brain MRI is used when indicated for tion, witnessed loss of consciousness, or concomitant neck subacute or chronic headaches, given its greater sensi- pain. Estimates vary widely, but many patients describe a past tivity and safety. headache consistent with a sentinel leak within the previous 1 ° Stable headaches meeting criteria for most primary to 2 weeks; these leaks typically involve abrupt, severe, tran- headaches usually do not require imaging. sient headaches that may be focal or global. Sensitivity of head CT is approximately 95% at 12 hours but decreases to 50% at 1 week. Lumbar puncture in SAH typically reveals a CSF eryth- Secondary Headache rocyte count greater than 10,000/uL (10,000 x 10°/L) and an Thunderclap Headache elevation in protein level. CSF xanthochromia may take Thunderclap headache is defined as a severe attack of head- 4 hours or more to develop but is nearly 100% sensitive ache pain developing abruptly and reaching maximum inten- between 12 hours and 7 days. Urgent neurosurgical consulta- sity within 1 minute. Although occasionally of primary origin, tion is required (see Stroke chapter). thunderclap headache is a medical emergency that warrants Headache is the most common symptom and may be the immediate diagnostic evaluation. Noncontrast head CT should only presenting manifestation with thrombosis of the intracra- be performed without delay. Lumbar puncture with measure- nial veins and sinuses. The pain is typically of sudden onset and ment of opening pressure, cell counts, and xanthochromia is unremitting. Clinical symptoms may be related to the result- should be performed if the head CT is nondiagnostic. Contrast- ing increase in intracranial pressure and include pain exacer- enhanced brain MRI and noninvasive vascular imaging (mag- bation with the Valsalva maneuver, pulsatile tinnitus, and netic resonance angiography [MRA] or CT angiography [CTA]) diplopia. Nonspecific findings of increased intracranial pres- of cranial and cervical vessels should be performed in patients sure include papilledema, decreased mentation, and seizures. with a normal CT scan and normal results of cerebrospinal Abducens nerve (cranial nerve VI) palsy secondary to increased fluid (CSF) analysis. Table 2 lists essential considerations in intracranial pressure may falsely indicate a focal lesion (falsely the differential diagnosis. localizing abducens nerve palsy). Specific findings may help Subarachnoid hemorrhage (SAH), the most common localize the thrombosis. Cavernous sinus thrombosis usually cause of thunderclap headache, is discovered in nearly 25% of involves extension of dental or sinus bacterial infection and affected patients. The headache associated with SAH may be manifests as acute-onset headache, proptosis, periorbital described as the worst the patient has ever experienced, edema, and ophthalmoplegia; this disorder requires immediate although the specificity of this finding is limited. Mortality is administration of antibiotics, often with surgical drainage. 50%, with an additional 25% of affected patients experiencing Other forms of cerebral venous thrombosis are not related to infection and often occur in the presence of hypercoagulable TABLE 2. Possible Causes of Thunderclap Headache states, thrombophilia, pregnancy (and the puerperium), oral | Secondary Headache Disorders contraceptive use, or dehydration. Anticoagulation is recom- | Intracranial hemorrhage mended even in those patients with hemorrhagic parenchymal lesions. Treatment with low-molecular-weight heparin results Cerebral venous sinus thrombosis in lower hospital mortality than treatment with unfractionated | Cervical artery dissection | heparin. Anticoagulation with warfarin or dabigatran is gener- Reversible cerebral vasoconstriction syndrome ally continued for a minimum of 3 to 6 months. Extended Posterior reversible encephalopathy syndrome anticoagulation should be considered in those with severe Ischemic stroke ortransient ischemic attack | hypercoagulable states, myeloproliferative disorders, or recur-

| Intracranial hemorrhage mended even in those patients with hemorrhagic parenchymal lesions. Treatment with low-molecular-weight heparin results Cerebral venous sinus thrombosis in lower hospital mortality than treatment with unfractionated | Cervical artery dissection | heparin. Anticoagulation with warfarin or dabigatran is gener- Reversible cerebral vasoconstriction syndrome ally continued for a minimum of 3 to 6 months. Extended Posterior reversible encephalopathy syndrome anticoagulation should be considered in those with severe Ischemic stroke ortransient ischemic attack | hypercoagulable states, myeloproliferative disorders, or recur- Spontaneous intracranial hypotension rence and perhaps in those with idiopathic thrombosis (see Subdural hematoma MKSAP 19 Hematology). Dissections of the cervical vessels (Figure 1) often present | Pituitary apoplexy with acute pain in the head and neck, typically occur in Pheochromocytoma younger patients, and may develop spontaneously or as a | Colloid cyst of third ventricle result of trauma, which may be minor. Risk factors include Acute hydrocephalus hypertension, migraine, polycystic kidney disease, and con- | Acute angle-closure glaucoma nective tissue disorders. Extracranial dissections are a com-

Subdural hematoma MKSAP 19 Hematology). Dissections of the cervical vessels (Figure 1) often present | Pituitary apoplexy with acute pain in the head and neck, typically occur in Pheochromocytoma younger patients, and may develop spontaneously or as a | Colloid cyst of third ventricle result of trauma, which may be minor. Risk factors include Acute hydrocephalus hypertension, migraine, polycystic kidney disease, and con- | Acute angle-closure glaucoma nective tissue disorders. Extracranial dissections are a com- | Primary Headache Disorders mon cause of stroke in persons younger than 50 years. Dissections of the carotid artery often present with orbital Primary thunderclap headache pain, partial Horner syndrome (ptosis and miosis only), and | Primary stabbing headache ipsilateral signs of cerebral or retinal ischemia. Those with

Headache and Facial Pain ~ DICA:s -178.Semés DICA:d 81.7¢cm/s FIGURE 1. Leftinternal carotid artery (ICA) dissection in a 32-year-old woman with a left frontal cerebral infarct. Top left, magnetic resonance angiogram of the neck showing mild irregularity in the distal extracranial ICA with a possible pseudoaneurysm (arrow). Top right, T1-weighted MRI of the soft tissues in the neck showing a crescent- shaped hematoma (hyperintense signal around the actual lumen) (arrow) within the ICA wall. Bottom, carotid Duplex ultrasounds from the same patient showing turbulent flow in the mid to distal ICA with associated accelerated systolic and diastolic velocities. DICA= distal ICA; LT= left; PICA= proximal ICA, dissections of the vertebral arteries may describe occipital and with an intramural hematoma. Noninvasive vessel imaging neck pain and posterior fossa symptoms, such as dysarthria, with MRA or CTA is typically sufficient. Comparative trials dysphagia, ataxia, and hemifield visual loss. Brain MRI may have not documented the superiority of anticoagulation in showa tapered arterial lumen or increased arterial diameter patients with dissections, and many are treated with aspirin. 3

Headache and Facial Pain TABLE 3. Predisposing Factors of Reversible Cerebral Vasoconstriction Syndrome e Thunderclap headache is a medical emergency that Vasoactive Drug Exposure | warrants immediate diagnostic evaluation; noncontrast Sympathomimetic agents (amphetamines, pseudoephedrine, | head CT should be performed without delay. phenylpropanolamine) e Subarachnoid hemorrhage is the most common cause Antidepressants (SSRIs, SNRIs, monoamine oxidase inhibitors) of thunderclap headache. Triptans or ergot alkaloid derivatives e Cavernous sinus thrombosis manifests as acute-onset Nicotine headache, proptosis, periorbital edema, and ophthal- Illicit drugs (cocaine, ecstasy, methamphetamines) moplegia; usually involves extension of dental or sinus

TABLE 3. Predisposing Factors of Reversible Cerebral Vasoconstriction Syndrome e Thunderclap headache is a medical emergency that Vasoactive Drug Exposure | warrants immediate diagnostic evaluation; noncontrast Sympathomimetic agents (amphetamines, pseudoephedrine, | head CT should be performed without delay. phenylpropanolamine) e Subarachnoid hemorrhage is the most common cause Antidepressants (SSRIs, SNRIs, monoamine oxidase inhibitors) of thunderclap headache. Triptans or ergot alkaloid derivatives e Cavernous sinus thrombosis manifests as acute-onset Nicotine headache, proptosis, periorbital edema, and ophthal- Illicit drugs (cocaine, ecstasy, methamphetamines) moplegia; usually involves extension of dental or sinus | Cannabis | bacterial infections; and requires immediate adminis- tration of antibiotics and frequently surgical drainage. Pregnancy and Puerperium | | Preeclampsia, eclampsia | e Dissections of the cervical vessels often present with acute pain in the head and neck and are a common | HELLP syndrome | cause of stroke in patients younger than 50 years. Head and Neck Trauma and Surgery | e Reversible cerebral vasoconstriction syndrome is Acute Cerebrovascular Disorders characterized by recurrent thunderclap headache and Cervical artery dissection is the second most frequent source of thunderclap Cerebral endovascular procedures or angiography headache.

| Cannabis | bacterial infections; and requires immediate adminis- tration of antibiotics and frequently surgical drainage. Pregnancy and Puerperium | | Preeclampsia, eclampsia | e Dissections of the cervical vessels often present with acute pain in the head and neck and are a common | HELLP syndrome | cause of stroke in patients younger than 50 years. Head and Neck Trauma and Surgery | e Reversible cerebral vasoconstriction syndrome is Acute Cerebrovascular Disorders characterized by recurrent thunderclap headache and Cervical artery dissection is the second most frequent source of thunderclap Cerebral endovascular procedures or angiography headache. Cerebral venous sinus thrombosis Miscellaneous Conditions Idiopathic Intracranial Hypertension ee

| Cannabis | bacterial infections; and requires immediate adminis- tration of antibiotics and frequently surgical drainage. Pregnancy and Puerperium | | Preeclampsia, eclampsia | e Dissections of the cervical vessels often present with acute pain in the head and neck and are a common | HELLP syndrome | cause of stroke in patients younger than 50 years. Head and Neck Trauma and Surgery | e Reversible cerebral vasoconstriction syndrome is Acute Cerebrovascular Disorders characterized by recurrent thunderclap headache and Cervical artery dissection is the second most frequent source of thunderclap Cerebral endovascular procedures or angiography headache. Cerebral venous sinus thrombosis Miscellaneous Conditions Idiopathic Intracranial Hypertension ee Increased intracranial pressure in the absence of any vascular Exposure to immunosuppressants or blood products or space-occupying lesion is the hallmark of idiopathic intra- Meningitis cranial hypertension (IIH) (also known as pseudotumor cere- Catecholamine-secreting tumors bri). Approximately 90% of affected persons are women of HELLP = Hemolysis, Elevated Liver enzymes, and Low Platelet count; SNRI = childbearing age with an elevated BMI. IIH is associated with serotonin-norepinephrine reuptake inhibitor; SSRI = selective serotonin reuptake inhibitor. the following conditions: ers

Increased intracranial pressure in the absence of any vascular Exposure to immunosuppressants or blood products or space-occupying lesion is the hallmark of idiopathic intra- Meningitis cranial hypertension (IIH) (also known as pseudotumor cere- Catecholamine-secreting tumors bri). Approximately 90% of affected persons are women of HELLP = Hemolysis, Elevated Liver enzymes, and Low Platelet count; SNRI = childbearing age with an elevated BMI. IIH is associated with serotonin-norepinephrine reuptake inhibitor; SSRI = selective serotonin reuptake inhibitor. the following conditions: ers e Hypervitaminosis A ¢ Use of the tetracycline class of antibiotics Reversible cerebral vasoconstriction syndrome is charac- terized by recurrent thunderclap headache and multifocal ¢ Retinoic acid use constriction of intracranial vessels normalizing within e Kidney failure 3 months of onset. Predisposing factors are listed in Table 3. It e Endocrine disorders (hypoparathyroidism, Addison disease) is the second most frequent source of thunderclap headache. ¢ Use of estrogen and progesterone supplements and preg- The headaches may be triggered by exertion, Valsalva maneu- nancy vers, emotion. or bathing. Focal deficits, encephalopathy, and seizures are seen in a minority of patients. Diagnostic evalua- e Glucocorticoid use or withdrawal

constriction of intracranial vessels normalizing within e Kidney failure 3 months of onset. Predisposing factors are listed in Table 3. It e Endocrine disorders (hypoparathyroidism, Addison disease) is the second most frequent source of thunderclap headache. ¢ Use of estrogen and progesterone supplements and preg- The headaches may be triggered by exertion, Valsalva maneu- nancy vers, emotion. or bathing. Focal deficits, encephalopathy, and seizures are seen in a minority of patients. Diagnostic evalua- e Glucocorticoid use or withdrawal tion should include noninvasive imaging of the brain and neck Headaches, visual symptoms, and intracranial noises vessels with MRA or CTA and CSF analysis. Digital subtraction (pulsatile tinnitus) are the most common presenting symp- angiography has been associated with transient neurologic toms of ITH. Head pain is nonspecific and may be like that of deficits and is typically avoided. Results of head CT and lumbar tension-type headache or migraine. Vision may be blurred, puncture are both typically normal. Brain MRI is more sensi- doubled, or periodically dim in brief episodes known as visual tive and may show areas of white matter edema primarily in obscurations. Visual field testing is essential in the initial and the occipital and parietal lobes compatible with posterior follow-up evaluations of IIH. Although only 25% of patients reversible encephalopathy syndrome. Areas of ischemia or may report visual symptoms, 90% have some abnormality on hemorrhage may he seen in the parenchyma, and a subdural visual perimetry. Typical findings include blind spot enlarge- or subarachnoid hemorrhage also may be found in some ment and peripheral field reduction. Patients with atypical patients. Management begins with resolution of predisposing presentations (men older than 50 years, patients with normal factors, avoidance of physical exertion, and management of BMIs) are less likely to report headaches and have a more blood pressure. Verapamil and nimodipine are the drugs of benign visual prognosis. choice. Glucocorticoids may worsen the clinical course and Findings on neurologic examination include papilledema should be avoided. Repeat MRA or CTA is necessary at and occasionally ophthalmoplegia. Enhanced brain MRI may 12 weeks to document resolution of vasospasm, at which time reveal widening of the optic nerve sheaths, an empty sella, or medication is tapered. flattening of the posterior optic globes. Magnetic resonance

tion should include noninvasive imaging of the brain and neck Headaches, visual symptoms, and intracranial noises vessels with MRA or CTA and CSF analysis. Digital subtraction (pulsatile tinnitus) are the most common presenting symp- angiography has been associated with transient neurologic toms of ITH. Head pain is nonspecific and may be like that of deficits and is typically avoided. Results of head CT and lumbar tension-type headache or migraine. Vision may be blurred, puncture are both typically normal. Brain MRI is more sensi- doubled, or periodically dim in brief episodes known as visual tive and may show areas of white matter edema primarily in obscurations. Visual field testing is essential in the initial and the occipital and parietal lobes compatible with posterior follow-up evaluations of IIH. Although only 25% of patients reversible encephalopathy syndrome. Areas of ischemia or may report visual symptoms, 90% have some abnormality on hemorrhage may he seen in the parenchyma, and a subdural visual perimetry. Typical findings include blind spot enlarge- or subarachnoid hemorrhage also may be found in some ment and peripheral field reduction. Patients with atypical patients. Management begins with resolution of predisposing presentations (men older than 50 years, patients with normal factors, avoidance of physical exertion, and management of BMIs) are less likely to report headaches and have a more blood pressure. Verapamil and nimodipine are the drugs of benign visual prognosis. choice. Glucocorticoids may worsen the clinical course and Findings on neurologic examination include papilledema should be avoided. Repeat MRA or CTA is necessary at and occasionally ophthalmoplegia. Enhanced brain MRI may 12 weeks to document resolution of vasospasm, at which time reveal widening of the optic nerve sheaths, an empty sella, or medication is tapered. flattening of the posterior optic globes. Magnetic resonance 4

Headache and Facial Pain venography may reveal unilateral or bilateral stenoses of the location of CSF leakage may be detected in 50% of patients by intracranial transverse sinuses. An elevation in CSF opening spinal MRI or CT myelography. Patients without identifiable pressure of greater than 250 mm H,O with normal CSF com- sites are empirically treated with “blind” lumbar EBP proce- position confirms the diagnosis. dures, whereas those with definable locations receive “tar- Treatment of ITH is aimed primarily at preservation of geted” EBP procedures at the appropriate spinal level. Relapses vision. Patients with BMI elevation require introduction of a may require repeat procedures. Symptomatic recovery may be weight-loss program. Bariatric procedures have been shown to delayed or complicated by “rebound” intracranial hyperten- be helpful, when necessary. Acetazolamide is the drug of sion. Those for whom the EBP approach is unsuccessful may choice. Topiramate may be less effective but carries the poten- require surgical repair. tial added benefit of weight loss. Paresthesia, kidney stones, and taste perversion are possible adverse effects of both e The headache associated with intracranial hypotension drugs. More immediate reduction in intracranial pressure caused by leakage of cerebrospinal fluid after a lumbar may be accomplished through repeated lumbar punctures. puncture is treated with an autologous epidural blood Cerebrospinal fluid diversion procedures, such as ventriculop- patch. eritoneal or lumboperitoneal shunting, should be considered in patients with medically refractory ITH. Endovascular stent e The diagnosis of spontaneous intracranial hypotension placement has been shown to be effective in patients with is associated with the contrast-enhanced brain-MRI

venography may reveal unilateral or bilateral stenoses of the location of CSF leakage may be detected in 50% of patients by intracranial transverse sinuses. An elevation in CSF opening spinal MRI or CT myelography. Patients without identifiable pressure of greater than 250 mm H,O with normal CSF com- sites are empirically treated with “blind” lumbar EBP proce- position confirms the diagnosis. dures, whereas those with definable locations receive “tar- Treatment of ITH is aimed primarily at preservation of geted” EBP procedures at the appropriate spinal level. Relapses vision. Patients with BMI elevation require introduction of a may require repeat procedures. Symptomatic recovery may be weight-loss program. Bariatric procedures have been shown to delayed or complicated by “rebound” intracranial hyperten- be helpful, when necessary. Acetazolamide is the drug of sion. Those for whom the EBP approach is unsuccessful may choice. Topiramate may be less effective but carries the poten- require surgical repair. tial added benefit of weight loss. Paresthesia, kidney stones, and taste perversion are possible adverse effects of both e The headache associated with intracranial hypotension drugs. More immediate reduction in intracranial pressure caused by leakage of cerebrospinal fluid after a lumbar may be accomplished through repeated lumbar punctures. puncture is treated with an autologous epidural blood Cerebrospinal fluid diversion procedures, such as ventriculop- patch. eritoneal or lumboperitoneal shunting, should be considered in patients with medically refractory ITH. Endovascular stent e The diagnosis of spontaneous intracranial hypotension placement has been shown to be effective in patients with is associated with the contrast-enhanced brain-MRI definable transverse sinus stenoses. finding of diffuse smooth dural thickening with enhancement in nearly 80% of patients.

placement has been shown to be effective in patients with is associated with the contrast-enhanced brain-MRI definable transverse sinus stenoses. finding of diffuse smooth dural thickening with enhancement in nearly 80% of patients. e Idiopathic intracranial hypertension, which is suggested Trigeminal Neuralgia by the presence of headache, visual symptoms, and pul- Trigeminal neuralgia is the most common and most intense satile tinnitus, occurs most commonly in women of child- cranial neuralgia. Incidence increases with advanced age. bearing age who have an elevated BMI. Multiple sclerosis should be considered in those with onset

e Idiopathic intracranial hypertension, which is suggested Trigeminal Neuralgia by the presence of headache, visual symptoms, and pul- Trigeminal neuralgia is the most common and most intense satile tinnitus, occurs most commonly in women of child- cranial neuralgia. Incidence increases with advanced age. bearing age who have an elevated BMI. Multiple sclerosis should be considered in those with onset e Treatment of idiopathic intracranial hypertension is before age 50 years. Pain is typically unilateral and localized aimed primarily at preservation of vision; weight loss in to the maxillary and mandibular branches of the trigeminal patients who have an elevated BMI and acetazolamide nerve (cranial nerve V). The pains are brief, “shock-like,” or are the treatments of choice. electric, lasting seconds to minutes. Paroxysms may be spon- taneous or triggered by innocuous stimulation of the face. Headaches from Intracranial Hypotension Refractory periods are common after a series of paroxysms. The most common source of intracranial hypotension is leak- Ipsilateral autonomic features are rare. Neurologic examina- age of CSF after a lumbar puncture. The cardinal feature is a tion is typically normal. Contrast-enhanced brain MRI detects postural headache, typically worsening within 20 seconds of nonvascular structural pathology (such as compressing and sitting or standing. Tinnitus, diplopia, neck pain, nausea, pho- demyelinating causes) in 15% of patients. In the other 85%, tophobia, and phonophobia are additional symptoms. Risk of brain MRA often identifies neurovascular contact between a developing symptoms may be reduced by using a small-caliber loop of the superior cerebellar artery and the trigeminal atraumatic needle but is unaffected by postprocedure bed rest. nerve. Contact causing displacement or atrophy of the nerve More than 50% of these headaches resolve spontaneously by has been associated with a greater likelihood of symptom day 4 after the procedure, and more than 75% by day 7. development. Persistent symptoms are treated with an autologous epidural Management of trigeminal neuralgia begins with carba- blood patch (EBP), which causes resolution of symptoms in mazepine administration. Pain may resolve within a few days, 80% to 90% of patients. although mild dizziness and drowsiness are common. Other Orthostatic headache is the most common of several adverse effects of the medication, including hyponatremia and clinical presentations of spontaneous intracranial hypoten- agranulocytosis, are less common but necessitate intermittent sion (SIH); the headache may be thunderclap or subacute in monitoring. Alternative drugs are oxcarbazepine, baclofen, nature. Associated symptoms are similar to those of post- gabapentin, and lamotrigine. Approximately 30% of patients dural-puncture headaches. No single diagnostic test detects do not respond to trials of monotherapy or combined therapy. SIH. A combination of brain and spine imaging is typically For those refractory to two-drug trials, surgical intervention required, and cerebrospinal fluid pressure measurement may should be considered. Nonsurgical options are effective in 50% be necessary. The contrast-enhanced brain MRI finding of dif- of patients and include percutaneous radiofrequency coagula- fuse, smooth dural thickening with enhancement is highly tion, glycerol injection, or focused stereotactic (Gamma Knife) specific for SIH. Cerebellar tonsillar descent (similar to that of radiation. Posterior fossa microvascular decompression of the a Chiari malformation), subdural fluid collections, decreased neurovascular contact zone (“Jannetta procedure”) is a more ventricular size, and engorgement of the pituitary gland are invasive and more effective option. It should be prioritized in other common findings. Leaks typically are spinal. The those with low surgical risk.

Headache and Facial Pain TABLE 4. International Headache Society Criteria for Migraine without Aura ¢ Contrast-enhanced brain MRI is the initial imaging test for patients with trigeminal neuralgia. | A. Atleast five attacks fulfilling criteria B-D | B. Headache attacks lasting 4-72 hours (untreated or e Initial management of trigeminal neuralgia is with unsuccessfully treated) carbamazepine; if medical therapy fails, focused stereo- C. Headache with at least two ofthe following four tactic (Gamma Knife) radiation, glycerol injection, or | ] characteristics: posterior fossa microvascular decompression may be 1. Unilateral location | effective. | 2. Pulsating quality 3. Moderate or severe pain intensity that inhibits or prohibits Medication-Induced Headache | | daily activities | Medication overuse headache (MOH), previously called | 4. Aggravation by walking up or down stairs or similar “rebound” headache, is a highly prevalent condition affecting | routine physical activity 1% of adults. This clinical syndrome may result from overtreat- D. During headache, occurrence of at least one of following | ment with acute medication in patients with underlying symptoms:

routine physical activity 1% of adults. This clinical syndrome may result from overtreat- D. During headache, occurrence of at least one of following | ment with acute medication in patients with underlying symptoms: migraine or tension-type headache. Use of triptans, ergot 1. Nausea/vomiting | alkaloids, opioids, or combination analgesics for 10 or more 2. Photophobia and phonophobia | E. Headache not better accounted for by another ICHD-3 | days per month or simple analgesics for 15 or more days per month constitutes medication overuse. Affected patients often | diagnosis | report daily or near-daily headache that is refractory to = ICHD-3 = International Classification of Headache Disorders, 3rd edition. | numerous treatment options. MOH is more common in midlife, in women, and in those with high baseline headache Adapted with permission of SAGE Publications LTD., London, Los Angeles, New | Delhi, Singapore and Washington DC, from Headache Classification Committee of | frequency. In those with migraine, opioids are associated with the International Headache Society (IHS). The International Classification of | Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):19. [29368949] a 44% increase and butalbital compounds with a 70% increase doi: 10.1177/0333102417738202.

ICHD-3 = International Classification of Headache Disorders, 3rd edition. | numerous treatment options. MOH is more common in midlife, in women, and in those with high baseline headache Adapted with permission of SAGE Publications LTD., London, Los Angeles, New | Delhi, Singapore and Washington DC, from Headache Classification Committee of | frequency. In those with migraine, opioids are associated with the International Headache Society (IHS). The International Classification of | Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):19. [29368949] a 44% increase and butalbital compounds with a 70% increase doi: 10.1177/0333102417738202. in the risk of headache progression. These medications should be avoided in patients with recurrent primary headache disor- ders. Management of MOH involves discontinuation of the presentations are misdiagnosed. Neck pain (75%) and “sinus” overused medication and the introduction of appropriate symptoms, such as tearing or nasal drainage (50%), are both preventive medication directed at the primary headache more common than features felt to be characteristic of

in the risk of headache progression. These medications should be avoided in patients with recurrent primary headache disor- ders. Management of MOH involves discontinuation of the presentations are misdiagnosed. Neck pain (75%) and “sinus” overused medication and the introduction of appropriate symptoms, such as tearing or nasal drainage (50%), are both preventive medication directed at the primary headache more common than features felt to be characteristic of disorder. migraine, such as vomiting or aura. In the presence of a stable clinical pattern of migraine and a normal neurologic examina- tion, brain imaging is not indicated. ¢ Medication overuse headache can result from use of Migraine without aura is the most prevalent migraine triptans, ergot alkaloids, opioids, or combination anal- subtype (Table 4). Although clinicians often emphasize unilat- gesic agents for 10 or more days per month or simple eral location or pulsatile characteristics, moderate to severe analgesic agents for 15 or more days per month; treat- pain is the most sensitive feature, and worsening by routine ment includes discontinuation of the overused medica- physical activity is the most specific element among the pain tion and use of appropriate preventive medication criteria. Photophobia, phonophobia, and nausea are each directed at the primary headache disorder. reported by approximately 75% of patients with migraine without aura. The diagnosis of migraine with aura is made after two Primary Headache discrete aura episodes have occurred (Table 5). Aura may occur Migraine in 20% to 30% of patients with migraine. It frequently precedes Diagnosis pain but may occur during or without head discomfort. Aura The International Classification of Headache Disorders (ICHD), symptoms involve positive and negative neurologic phenom- third edition (beta version), recognizes several subtypes of ena developing gradually and evolving over a period of 5 to migraine, including migraine without aura, migraine with 60 minutes. Resolution is gradual and complete. ICHD criteria aura, and chronic migraine. Each is characterized by episodes recognize several aura subtypes. Typical aura involves any of disabling headache lasting hours to days. Accuracy of diag- combination of homonymous visual, hemisensory, or lan- nosis and management is enhanced by the use of formal ICHD guage symptoms. Brainstem aura is defined by the presence of criteria, although the POUND mnemonic (Pulsatile quality of two of the following brainstem symptoms: dysarthria, vertigo, headache, One-day duration, Unilateral location, Nausea or tinnitus, hypacusis, diplopia, ataxia, or decreased level of con- vomiting, Disabling intensity) is a helpful means of recalling sciousness. Hemiplegic aura comprises any aura involving the symptoms typically associated with migraines. Because of motor weakness. Both hemiplegic and brainstem auras are the extensive phenotypic variation, nearly half of migraine listed as contraindications for triptan use. Retinal aura involves

disorder. migraine, such as vomiting or aura. In the presence of a stable clinical pattern of migraine and a normal neurologic examina- tion, brain imaging is not indicated. ¢ Medication overuse headache can result from use of Migraine without aura is the most prevalent migraine triptans, ergot alkaloids, opioids, or combination anal- subtype (Table 4). Although clinicians often emphasize unilat- gesic agents for 10 or more days per month or simple eral location or pulsatile characteristics, moderate to severe analgesic agents for 15 or more days per month; treat- pain is the most sensitive feature, and worsening by routine ment includes discontinuation of the overused medica- physical activity is the most specific element among the pain tion and use of appropriate preventive medication criteria. Photophobia, phonophobia, and nausea are each directed at the primary headache disorder. reported by approximately 75% of patients with migraine without aura. The diagnosis of migraine with aura is made after two Primary Headache discrete aura episodes have occurred (Table 5). Aura may occur Migraine in 20% to 30% of patients with migraine. It frequently precedes Diagnosis pain but may occur during or without head discomfort. Aura The International Classification of Headache Disorders (ICHD), symptoms involve positive and negative neurologic phenom- third edition (beta version), recognizes several subtypes of ena developing gradually and evolving over a period of 5 to migraine, including migraine without aura, migraine with 60 minutes. Resolution is gradual and complete. ICHD criteria aura, and chronic migraine. Each is characterized by episodes recognize several aura subtypes. Typical aura involves any of disabling headache lasting hours to days. Accuracy of diag- combination of homonymous visual, hemisensory, or lan- nosis and management is enhanced by the use of formal ICHD guage symptoms. Brainstem aura is defined by the presence of criteria, although the POUND mnemonic (Pulsatile quality of two of the following brainstem symptoms: dysarthria, vertigo, headache, One-day duration, Unilateral location, Nausea or tinnitus, hypacusis, diplopia, ataxia, or decreased level of con- vomiting, Disabling intensity) is a helpful means of recalling sciousness. Hemiplegic aura comprises any aura involving the symptoms typically associated with migraines. Because of motor weakness. Both hemiplegic and brainstem auras are the extensive phenotypic variation, nearly half of migraine listed as contraindications for triptan use. Retinal aura involves 6

Headache and Facial Pain TABLE 6. International Headache Society Criteria for Chronic Migraine | A. Headache (tension-type-like and/or migraine-like) on at B. One or more of the following fully reversible aura symptoms: | | least 15 days per month for greater than 3 months and | fulfilling criteria Band C | 1. Visual B. Occurring in a patient who has had at least five attacks | 2. Sensory | fulfilling criteria B-D for migraine without aura and/or criteria | B and C for migraine with aura 3. Speech and/or language | C. On at least 8 days per month for greater than 3 months, | |

2. Sensory | fulfilling criteria B-D for migraine without aura and/or criteria | B and C for migraine with aura 3. Speech and/or language | C. On at least 8 days per month for greater than 3 months, | | | 4. Motor | | fulfilling any of the following: | | 5. Brainstem | | 1. Criteria C and Dfor migraine without aura | | 6. Retinal | 2. Criteria B and C for migraine with aura | | C. At least three of the following six characteristics: | | 3. Believed by the patient to be a migraine at onset and | 1. At least one aura symptom spreads gradually over relieved by a triptan or ergot alkaloid derivative >5 minutes D. Headache not better accounted for by another ICHD-3 | NHN . Two or more aura symptoms occur in succession diagnosis

| 4. Motor | | fulfilling any of the following: | | 5. Brainstem | | 1. Criteria C and Dfor migraine without aura | | 6. Retinal | 2. Criteria B and C for migraine with aura | | C. At least three of the following six characteristics: | | 3. Believed by the patient to be a migraine at onset and | 1. At least one aura symptom spreads gradually over relieved by a triptan or ergot alkaloid derivative >5 minutes D. Headache not better accounted for by another ICHD-3 | NHN . Two or more aura symptoms occur in succession diagnosis Ww . Each individual aura symptom lasts 5-60 minutes | ICHD-3 = International Classification of Headache Disorders, 3rd edition. | ff Adapted with permission of SAGE Publications LTD., London, Los Angeles, New . At least one aura symptom is unilateral | Delhi, Singapore and Washington DC, from Headache Classification Committee of oa the International Headache Society (IHS). The International Classification of . At least one aura symptom is positive Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):24. [29368949]

Ww . Each individual aura symptom lasts 5-60 minutes | ICHD-3 = International Classification of Headache Disorders, 3rd edition. | ff Adapted with permission of SAGE Publications LTD., London, Los Angeles, New . At least one aura symptom is unilateral | Delhi, Singapore and Washington DC, from Headache Classification Committee of oa the International Headache Society (IHS). The International Classification of . At least one aura symptom is positive Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):24. [29368949] Lo a _| a . The aura is accompanied, orfollowed within 60 minutes, doi: 10.1177/0333102417738202. by headache |

Ww . Each individual aura symptom lasts 5-60 minutes | ICHD-3 = International Classification of Headache Disorders, 3rd edition. | ff Adapted with permission of SAGE Publications LTD., London, Los Angeles, New . At least one aura symptom is unilateral | Delhi, Singapore and Washington DC, from Headache Classification Committee of oa the International Headache Society (IHS). The International Classification of . At least one aura symptom is positive Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):24. [29368949] Lo a _| a . The aura is accompanied, orfollowed within 60 minutes, doi: 10.1177/0333102417738202. by headache | D. Headache not better accounted for by another ICHD-3 | diagnosis, and transient ischemic attack has been excluded intraindividual variability, different treatment options and strategies are required (Table 7). Guidelines recommend tailor- ij ICHD-3 = International Classification of Headache Disorders, 3rd edition. ing the treatments to the severity and symptomatology of the Adapted with permission of SAGE Publications LTD., London, Los Angeles, New | Delhi, Singapore and Washington DC, from Headache Classification Committee of | attack. Migraines awakening a patient from sleep or those the International Headache Society (IHS). The International Classification of associated with nausea and vomiting may require medication Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):20. [29368949] | doi: 10.1177/0333102417738202. offered in parenteral or nasal formulations. Because consist- ency of response to any treatment rarely approaches 100%, monocular visual compromise. These auras need to be distin- most patients benefit from availability of two or more acute guished from ocular pathologies, such as retinal ischemia or migraine therapies. Administration of medication at the time detachment. Given an associated increased risk of stroke, of mild pain has been shown to improve therapeutic outcomes estrogen-containing oral contraceptives should be avoided in when compared to treating moderate to severe headache; women with migraine aura of all subtypes. therefore, treatment should be started as early as possible in Chronic migraine is defined as headache for 15 or more the disease course. Treatment should be limited to 10 days per days per month (Table 6). Transformation from acute to month to avoid MOH. chronic migraine occurs in the general population at an annual Evidence-based guidelines recommend several simple rate of 3%. Older age, female sex, head trauma, major life and combination analgesic agents as first-line therapies for changes or stressors, obesity, chronic pain, mood and anxiety acute migraine. Acetaminophen has established efficacy only disorders, and inadequate acute migraine management are in migraine of mild to moderate intensity; data suggest the risk factors. Acute migraine medication or caffeine overuse response may be enhanced by coadministration with metoclo- and exposure to nicotine may also raise the risk for or exacer- pramide. Aspirin administered alone or in combination with bate chronic migraine; some patients with chronic migraine acetaminophen and caffeine also has established efficacy in may have a secondary diagnosis of MOH. Patients with chronic acute migraine. The effectiveness of the NSAIDs ibuprofen, migraine are more disabled and more likely to report migraine- naproxen sodium, and diclofenac potassium is supported by related comorbidities (mood or anxiety disorders, sleep dys- strong evidence. Special formulations of these products have function, irritable bowel syndrome, fibromyalgia) than are been shown to be more rapidly absorbed and effective than those with episodic migraine. their standard tablet counterparts. These formulations include effervescent aspirin, solubilized ibuprofen, and diclofenac Acute Migraine Management powder for oral solution. The acute treatment of migraine aims to eliminate pain and Triptans are migraine-specific selective agonists at restore function. Goals of care involve freedom from pain, 5-hydroxytryptamine 1B and 1D receptors. These drugs inhibit nausea, and sensory sensitivities within 1 to 2 hours and main- the release of calcitonin gene-related peptide, which mediates tenance of such control through at least 24 hours. Because cerebral vasodilation, neurogenic inflammation, and pain attack characteristics show significant interindividual and sensitization of migraine. Guidelines recommend the use of

D. Headache not better accounted for by another ICHD-3 | diagnosis, and transient ischemic attack has been excluded intraindividual variability, different treatment options and strategies are required (Table 7). Guidelines recommend tailor- ij ICHD-3 = International Classification of Headache Disorders, 3rd edition. ing the treatments to the severity and symptomatology of the Adapted with permission of SAGE Publications LTD., London, Los Angeles, New | Delhi, Singapore and Washington DC, from Headache Classification Committee of | attack. Migraines awakening a patient from sleep or those the International Headache Society (IHS). The International Classification of associated with nausea and vomiting may require medication Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):20. [29368949] | doi: 10.1177/0333102417738202. offered in parenteral or nasal formulations. Because consist- ency of response to any treatment rarely approaches 100%, monocular visual compromise. These auras need to be distin- most patients benefit from availability of two or more acute guished from ocular pathologies, such as retinal ischemia or migraine therapies. Administration of medication at the time detachment. Given an associated increased risk of stroke, of mild pain has been shown to improve therapeutic outcomes estrogen-containing oral contraceptives should be avoided in when compared to treating moderate to severe headache; women with migraine aura of all subtypes. therefore, treatment should be started as early as possible in Chronic migraine is defined as headache for 15 or more the disease course. Treatment should be limited to 10 days per days per month (Table 6). Transformation from acute to month to avoid MOH. chronic migraine occurs in the general population at an annual Evidence-based guidelines recommend several simple rate of 3%. Older age, female sex, head trauma, major life and combination analgesic agents as first-line therapies for changes or stressors, obesity, chronic pain, mood and anxiety acute migraine. Acetaminophen has established efficacy only disorders, and inadequate acute migraine management are in migraine of mild to moderate intensity; data suggest the risk factors. Acute migraine medication or caffeine overuse response may be enhanced by coadministration with metoclo- and exposure to nicotine may also raise the risk for or exacer- pramide. Aspirin administered alone or in combination with bate chronic migraine; some patients with chronic migraine acetaminophen and caffeine also has established efficacy in may have a secondary diagnosis of MOH. Patients with chronic acute migraine. The effectiveness of the NSAIDs ibuprofen, migraine are more disabled and more likely to report migraine- naproxen sodium, and diclofenac potassium is supported by related comorbidities (mood or anxiety disorders, sleep dys- strong evidence. Special formulations of these products have function, irritable bowel syndrome, fibromyalgia) than are been shown to be more rapidly absorbed and effective than those with episodic migraine. their standard tablet counterparts. These formulations include effervescent aspirin, solubilized ibuprofen, and diclofenac Acute Migraine Management powder for oral solution. The acute treatment of migraine aims to eliminate pain and Triptans are migraine-specific selective agonists at restore function. Goals of care involve freedom from pain, 5-hydroxytryptamine 1B and 1D receptors. These drugs inhibit nausea, and sensory sensitivities within 1 to 2 hours and main- the release of calcitonin gene-related peptide, which mediates tenance of such control through at least 24 hours. Because cerebral vasodilation, neurogenic inflammation, and pain attack characteristics show significant interindividual and sensitization of migraine. Guidelines recommend the use of 7

Headache and Facial Pain headaches. Different formulations of one triptan may be used TABLE 7. Acute Migraine Therapies safely within the same day, but the use of a different triptan or Drug Recommended Dose | an ergot alkaloid should be delayed by 24 hours. Despite labe- | NSAIDs? | ling precautions, the concurrent use of triptans and selective Aspirin 325-900 mg | serotonin reuptake inhibitors or serotonin-norepinephrine Ibuprofen 400-800 mg reuptake inhibitor antidepressants is safe in most cases. Newer Naproxen sodium 250-1000 mg migraine-specific acute therapies have no significant vascular risks based on different mechanisms of action. “Ditans” are Combination of 7 2 tablets | acetaminophen-aspirin-caffeine | selective agonists at the 5-hydroxytryptamine 1F receptor.

Naproxen sodium 250-1000 mg migraine-specific acute therapies have no significant vascular risks based on different mechanisms of action. “Ditans” are Combination of 7 2 tablets | acetaminophen-aspirin-caffeine | selective agonists at the 5-hydroxytryptamine 1F receptor. Diclofenac potassium (oral 50 mg Lasmiditan is first in its class. The “gepants,” ubrogepant and solution) | rimegepant, are direct antagonists at the calcitonin gene- related peptide (CGRP) receptor. Migraine-Specific Oral Agents? | Other agents also are effective in the management of | Almotriptan 6.25-12.5 mg | acute migraine. Ergot alkaloids have been used for years but | Eletriptan 20-40 mg have been largely replaced by triptans, which have preferable Frovatriptan 2.5mg safety and tolerability profiles; parenteral and nasal formula- Lasmiditan 50-100 mg | tions of dihydroergotamine are the most available and effective

Diclofenac potassium (oral 50 mg Lasmiditan is first in its class. The “gepants,” ubrogepant and solution) | rimegepant, are direct antagonists at the calcitonin gene- related peptide (CGRP) receptor. Migraine-Specific Oral Agents? | Other agents also are effective in the management of | Almotriptan 6.25-12.5 mg | acute migraine. Ergot alkaloids have been used for years but | Eletriptan 20-40 mg have been largely replaced by triptans, which have preferable Frovatriptan 2.5mg safety and tolerability profiles; parenteral and nasal formula- Lasmiditan 50-100 mg | tions of dihydroergotamine are the most available and effective Naratriptan 1-2.5mg | options among the ergot alkaloids. Dopamine D, receptor antagonists (metoclopramide, prochlorperazine) are often Rizatriptan 5-10 mg used as adjuncts to analgesics or triptans. In addition to Sumatriptan 25-100 mg | antiemetic properties, these agents can reduce migraine pain Sumatriptan-naproxen 85-500 mg when delivered parenterally. Guidelines recommend avoiding Zolmitriptan 2.5-5 mg | the use of opioids and butalbital-containing compounds for

Naratriptan 1-2.5mg | options among the ergot alkaloids. Dopamine D, receptor antagonists (metoclopramide, prochlorperazine) are often Rizatriptan 5-10 mg used as adjuncts to analgesics or triptans. In addition to Sumatriptan 25-100 mg | antiemetic properties, these agents can reduce migraine pain Sumatriptan-naproxen 85-500 mg when delivered parenterally. Guidelines recommend avoiding Zolmitriptan 2.5-5 mg | the use of opioids and butalbital-containing compounds for Nonoral Agents? acute migraine. In addition to the potential for dependence or addiction, use of these agents has been linked to an increased Dihydroergotamine 1 mg nasally | Dihydroergotamine 1 mg subcutaneously | risk of transformation from episodic to chronic migraine. They should be used sparingly and only when more appropriate Prochlorperazine 10 mg intravenously | acute therapies are contraindicated. Sumatriptan 5-20 mg nasally | Patients not responding to multiple acute migraine treat-

| Dihydroergotamine 1 mg subcutaneously | risk of transformation from episodic to chronic migraine. They should be used sparingly and only when more appropriate Prochlorperazine 10 mg intravenously | acute therapies are contraindicated. Sumatriptan 5-20 mg nasally | Patients not responding to multiple acute migraine treat- Sumatriptan 4-6 mg subcutaneously | | ments may be candidates for referral. Neuromodulatory devices | shown to be effective in the acute treatment of migraine may be Zolmitriptan 5 mg nasally |

Sumatriptan 4-6 mg subcutaneously | | ments may be candidates for referral. Neuromodulatory devices | shown to be effective in the acute treatment of migraine may be Zolmitriptan 5 mg nasally | | used by specialists in this setting. | *Doses listed may be administered once or twice daily. Migraine with a duration lasting longer than 72 hours is known as status migrainosus. Many patients with this condi- triptans in patients with moderate to severe migraine who tion can be treated with several days of glucocorticoids. Severe have not responded to NSAID therapy over a series of at least cases of acute migraine or status migrainosus may require three migraine attacks. Current evidence suggests that all oral emergency department or inpatient management. The corner- triptans possess nearly similar clinical efficacy. Orally dissolv- stone of care in these settings is intravenous delivery of a able tablets are intestinally absorbed; their only advantage is dopamine antagonist. These are typically combined with use without the need to drink liquids. Nasal spray options may intravenous diphenhydramine (to limit dystonic reactions), have more rapid onset, bypassing the gastrointestinal tract. intravenous ketorolac, and hydration. Opioids may be associ- Outcomes are like those of oral agents, but unpleasant taste, ated with prolonged length of hospital stay and should be nasal congestion, or burning may be limiting factors. avoided. A more extended course of treatment involving repet- Subcutaneous sumatriptan provides the most rapid onset itive intravenous dihydroergotamine with antiemetics over 2 and achieves the highest response rates among all triptan to 3 days is very effective in the management of refractory agents and formulations. Local site reactions and more status migrainosus. prominent “triptan” sensations characteristic of the class (flushing, chest or throat tightness, paresthesia) may be Migraine Prevention noted. Because of vasoconstricting properties, all triptans are The goals of migraine prevention are the reduction of migraine contraindicated in the presence of coronary, cerebral, or frequency, intensity, and duration. None of the medications peripheral vascular disease; uncontrolled hypertension; or used for migraine prevention were designed for this specific migraine with brainstem or hemiplegic auras. Lasmiditan, a purpose. The best agents may reduce migraine frequency highly selective 5-HT1F receptor agonist, and ubrogepant, a by half in approximately half of the patients treated. calcitonin-gene related peptide antagonist, lack vasoconstric- Nonpharmacologic preventive measures are not only optimal tor properties for migraine and are now approved for migraine but required. Trigger identification and avoidance are often

| used by specialists in this setting. | *Doses listed may be administered once or twice daily. Migraine with a duration lasting longer than 72 hours is known as status migrainosus. Many patients with this condi- triptans in patients with moderate to severe migraine who tion can be treated with several days of glucocorticoids. Severe have not responded to NSAID therapy over a series of at least cases of acute migraine or status migrainosus may require three migraine attacks. Current evidence suggests that all oral emergency department or inpatient management. The corner- triptans possess nearly similar clinical efficacy. Orally dissolv- stone of care in these settings is intravenous delivery of a able tablets are intestinally absorbed; their only advantage is dopamine antagonist. These are typically combined with use without the need to drink liquids. Nasal spray options may intravenous diphenhydramine (to limit dystonic reactions), have more rapid onset, bypassing the gastrointestinal tract. intravenous ketorolac, and hydration. Opioids may be associ- Outcomes are like those of oral agents, but unpleasant taste, ated with prolonged length of hospital stay and should be nasal congestion, or burning may be limiting factors. avoided. A more extended course of treatment involving repet- Subcutaneous sumatriptan provides the most rapid onset itive intravenous dihydroergotamine with antiemetics over 2 and achieves the highest response rates among all triptan to 3 days is very effective in the management of refractory agents and formulations. Local site reactions and more status migrainosus. prominent “triptan” sensations characteristic of the class (flushing, chest or throat tightness, paresthesia) may be Migraine Prevention noted. Because of vasoconstricting properties, all triptans are The goals of migraine prevention are the reduction of migraine contraindicated in the presence of coronary, cerebral, or frequency, intensity, and duration. None of the medications peripheral vascular disease; uncontrolled hypertension; or used for migraine prevention were designed for this specific migraine with brainstem or hemiplegic auras. Lasmiditan, a purpose. The best agents may reduce migraine frequency highly selective 5-HT1F receptor agonist, and ubrogepant, a by half in approximately half of the patients treated. calcitonin-gene related peptide antagonist, lack vasoconstric- Nonpharmacologic preventive measures are not only optimal tor properties for migraine and are now approved for migraine but required. Trigger identification and avoidance are often 8

Headache and Facial Pain helpful. Stress management techniques, such as relaxation therapy or biofeedback, have established efficacy. Regulation of e Triptans are migraine-specific agents that are useful in sleep patterns, intake of small frequent meals, adequate hydra- moderate to severe migraine that has not responded to tion, and daily aerobic exercise are all extremely helpful. Regular NSAID therapy; they are contraindicated in the pres- work and school schedules should be encouraged. Stimulants ence of coronary, cerebral, or peripheral vascular dis- (such as caffeine and nicotine) must be eliminated or limited. ease; uncontrolled hypertension; or migraine with Diet should be modified to avoid additives or preservatives, such brainstem or hemiplegic auras. as monosodium glutamate and artificial sweeteners. There is e Pharmacologic prophylaxis for migraine should be con- good evidence to support the use of certain supplements, such sidered when headache frequency reaches 4 days per as petasites (butterbur), magnesium, riboflavin, and feverfew. Pharmacologic prophylaxis should be considered when month and almost always is initiated when the frequency the headache frequency reaches 4 days per month and almost exceeds 10 days per month.

helpful. Stress management techniques, such as relaxation therapy or biofeedback, have established efficacy. Regulation of e Triptans are migraine-specific agents that are useful in sleep patterns, intake of small frequent meals, adequate hydra- moderate to severe migraine that has not responded to tion, and daily aerobic exercise are all extremely helpful. Regular NSAID therapy; they are contraindicated in the pres- work and school schedules should be encouraged. Stimulants ence of coronary, cerebral, or peripheral vascular dis- (such as caffeine and nicotine) must be eliminated or limited. ease; uncontrolled hypertension; or migraine with Diet should be modified to avoid additives or preservatives, such brainstem or hemiplegic auras. as monosodium glutamate and artificial sweeteners. There is e Pharmacologic prophylaxis for migraine should be con- good evidence to support the use of certain supplements, such sidered when headache frequency reaches 4 days per as petasites (butterbur), magnesium, riboflavin, and feverfew. Pharmacologic prophylaxis should be considered when month and almost always is initiated when the frequency the headache frequency reaches 4 days per month and almost exceeds 10 days per month. always is initiated when the frequency exceeds 10 days per month. Data suggest preventive medication can reduce attack Tension-Type Headache frequency and intensity, patient disability, and medical cost. The most prevalent primary headache condition, tension-type Several weeks or months are often required before maximum headache is defined by clinical criteria as a headache disorder benefit is achieved. Once a response occurs, the medications that, unlike migraine, is mild to moderate in intensity and is should be continued for a period of 6 to 12 months, at which not associated with nausea, severe sensory sensitivities, or point dose reduction or drug elimination may be considered. neurologic symptoms (Table 8). Imaging is not indicated. Evidence-based guidelines for pharmacologic prevention of Subclassification is based on monthly headache frequency: episodic migraine have established Level A evidence support- infrequent episodic (<1 day), frequent episodic (1-14 days), and ing the use of five medications: three B-adrenergic blockers chronic (15 or more days). Acetaminophen, aspirin, NSAIDs, (propranolol, timolol, metoprolol) and two antiepileptic drugs and caffeine-containing compounds are effective acute treat- (divalproex sodium and topiramate). Level B evidence is avail- ments for tension-type headache. Amitriptyline is the only able for atenolol, two antidepressants (amitriptyline and ven- agent shown to be effective in controlled trials for tension-type lafaxine), and several NSAIDs. No evidence supports the use of headache prevention; venlafaxine and mirtazapine have more calcium channel blockers, selective serotonin reuptake inhibi- limited data. Muscle relaxants, benzodiazepines, opioids, and tor antidepressants, or onabotulinum toxin A in episodic onabotulinum toxin A have no role in the management of migraine prevention. Studies involving patients with chronic tension-type headache. Psychological treatment strategies, migraine are more limited, but efficacy in prevention has been shown with topiramate and onabotulinum toxin A. Migraine- TABLE 8. International Headache Society Criteria for specific biologic therapies have been developed and shown to Episodic Tension-Type Headache? be effective in the prevention of both episodic and chronic | A. At least 10 episodes of headache fulfilling criteria B-D | migraine. The monoclonal antibodies erenumab, fremane-

always is initiated when the frequency exceeds 10 days per month. Data suggest preventive medication can reduce attack Tension-Type Headache frequency and intensity, patient disability, and medical cost. The most prevalent primary headache condition, tension-type Several weeks or months are often required before maximum headache is defined by clinical criteria as a headache disorder benefit is achieved. Once a response occurs, the medications that, unlike migraine, is mild to moderate in intensity and is should be continued for a period of 6 to 12 months, at which not associated with nausea, severe sensory sensitivities, or point dose reduction or drug elimination may be considered. neurologic symptoms (Table 8). Imaging is not indicated. Evidence-based guidelines for pharmacologic prevention of Subclassification is based on monthly headache frequency: episodic migraine have established Level A evidence support- infrequent episodic (<1 day), frequent episodic (1-14 days), and ing the use of five medications: three B-adrenergic blockers chronic (15 or more days). Acetaminophen, aspirin, NSAIDs, (propranolol, timolol, metoprolol) and two antiepileptic drugs and caffeine-containing compounds are effective acute treat- (divalproex sodium and topiramate). Level B evidence is avail- ments for tension-type headache. Amitriptyline is the only able for atenolol, two antidepressants (amitriptyline and ven- agent shown to be effective in controlled trials for tension-type lafaxine), and several NSAIDs. No evidence supports the use of headache prevention; venlafaxine and mirtazapine have more calcium channel blockers, selective serotonin reuptake inhibi- limited data. Muscle relaxants, benzodiazepines, opioids, and tor antidepressants, or onabotulinum toxin A in episodic onabotulinum toxin A have no role in the management of migraine prevention. Studies involving patients with chronic tension-type headache. Psychological treatment strategies, migraine are more limited, but efficacy in prevention has been shown with topiramate and onabotulinum toxin A. Migraine- TABLE 8. International Headache Society Criteria for specific biologic therapies have been developed and shown to Episodic Tension-Type Headache? be effective in the prevention of both episodic and chronic | A. At least 10 episodes of headache fulfilling criteria B-D | migraine. The monoclonal antibodies erenumab, fremane- zumab, galcanezumab, and eptinezumab target calcitonin | B. Headache attacks (untreated or unsuccessfully treated) | lasting from 30 minutes to 7 days gene-related peptide or its receptor. Guidelines recommend C. Headache with at least two of the following four integrating these agents into prevention of episodic or chronic characteristics: migraine after two or three adequate but unsuccessful trials of | 1. Bilateral location oral preventive medication. Treatment selection is informed by previous therapeutic trials, the presence of coexisting med- | 2. Pressing/tightening (nonpulsating) quality ical conditions, and patient preference. 3. Mild or moderate intensity

zumab, galcanezumab, and eptinezumab target calcitonin | B. Headache attacks (untreated or unsuccessfully treated) | lasting from 30 minutes to 7 days gene-related peptide or its receptor. Guidelines recommend C. Headache with at least two of the following four integrating these agents into prevention of episodic or chronic characteristics: migraine after two or three adequate but unsuccessful trials of | 1. Bilateral location oral preventive medication. Treatment selection is informed by previous therapeutic trials, the presence of coexisting med- | 2. Pressing/tightening (nonpulsating) quality ical conditions, and patient preference. 3. Mild or moderate intensity 4. Not aggravated by walking stairs or similar routine | physical activity HVC ¢ The POUND mnemonic (Pulsatile quality of headache, | D. Headache characterized by both of the following: One-day duration, Unilateral location, Nausea or vomit- 1. No nausea/vomiting ing, Disabling intensity) is a helpful means of recalling 2. No more than one of photophobia or phonophobia the symptoms typically associated with migraines. | E. Not better accounted for by another ICHD-3 diagnosis HVC ¢ In the presence ofa stable clinical pattern of migraine and a normal neurologic examination, brain imaging is | ICHD-3 = International Classification of Headache Disorders, 3rd edition.

4. Not aggravated by walking stairs or similar routine | physical activity HVC ¢ The POUND mnemonic (Pulsatile quality of headache, | D. Headache characterized by both of the following: One-day duration, Unilateral location, Nausea or vomit- 1. No nausea/vomiting ing, Disabling intensity) is a helpful means of recalling 2. No more than one of photophobia or phonophobia the symptoms typically associated with migraines. | E. Not better accounted for by another ICHD-3 diagnosis HVC ¢ In the presence ofa stable clinical pattern of migraine and a normal neurologic examination, brain imaging is | ICHD-3 = International Classification of Headache Disorders, 3rd edition. not indicated. | Adapted with permission of SAGE Publications LTD., London, Los Angeles, New | Delhi, Singapore and Washington DC, from Headache Classification Committee of ¢ Given an associated increased risk of stroke, estrogen- _ the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):36-7. [29368949] containing oral contraceptives should be avoided in doi: 10.1177/0333102417738202. women with migraine aura of all subtypes. “Episodic tension-type headache is considered infrequent if it occurs <1 day/month | on average (<12 days/year) and frequent if it occurs 1-14 days/month on average (Continued) for >3 months (212 and <180 days/year).

Headache and Facial Pain such as relaxation training and cognitive behavioral therapies, are both effective in the treatment of attacks. Less effective have reasonable scientific support for effectiveness. Physical alternatives include nasal sumatriptan and zolmitriptan. A therapies have only modest effect, acupuncture possible effect, 2-week course of glucocorticoids may help reduce attack and spinal manipulation no effect. frequency at the onset of the cycle. Verapamil and galcane- zumab have been shown to reduce cluster headache fre- quency. Several neuromodulatory devices also have been ¢ Imaging is not indicated for tension-type headache. shown to be effective in the acute or preventive treatment of ¢ Acetaminophen, aspirin, NSAIDs, caffeine-containing cluster headache. compounds, and amitriptyline are effective acute and Other primary headache syndromes seen occasionally in preventive treatments for tension-type headache, but clinical practice are outlined in Table 10. muscle relaxants, benzodiazepines, opioids, and onabotulinum toxin A have no role in the management of tension-type headache. TABLE 10. Other Primary Headache Syndromes

such as relaxation training and cognitive behavioral therapies, are both effective in the treatment of attacks. Less effective have reasonable scientific support for effectiveness. Physical alternatives include nasal sumatriptan and zolmitriptan. A therapies have only modest effect, acupuncture possible effect, 2-week course of glucocorticoids may help reduce attack and spinal manipulation no effect. frequency at the onset of the cycle. Verapamil and galcane- zumab have been shown to reduce cluster headache fre- quency. Several neuromodulatory devices also have been ¢ Imaging is not indicated for tension-type headache. shown to be effective in the acute or preventive treatment of ¢ Acetaminophen, aspirin, NSAIDs, caffeine-containing cluster headache. compounds, and amitriptyline are effective acute and Other primary headache syndromes seen occasionally in preventive treatments for tension-type headache, but clinical practice are outlined in Table 10. muscle relaxants, benzodiazepines, opioids, and onabotulinum toxin A have no role in the management of tension-type headache. TABLE 10. Other Primary Headache Syndromes Cc hronic paroxysmal hemicrania Trigeminal Autonomic Cephalalgias Symptoms like cluster headache, episodes shorter (2-30 minutes) TACs are the most severe and stereotypic primary headache | Responsive to indomethacin disorders. Pain is severe, localized to the periorbital or temporal Short-lasting unilateral neuralgiform headache attacks with areas, and associated with pronounced ipsilateral cranial auto- | conjunctival injection and tearing (SUNCT) | nomic features, such as nasal congestion or rhinorrhea and Symptoms like cluster headache, episodes shorter ptosis or miosis. Cluster headache is the most common TAC. (1-600 seconds) Cluster headache may last 15 to 180 minutes and recur one Medically refractory, reports of response to lamotrigine to eight times daily over a span of weeks to months; the shorter duration distinguishes cluster headache from migraine (Table 9). | Hemicrania continua | This headache is characterized by a cyclical nature in which Persistent strictly unilateral headache with autonomic features | periods of recurrent headache activity are interrupted by | Responsive to indomethacin months to years of headache remission. Many of the attacks are | Primary cough headache nocturnal, and some may be provoked by alcohol ingestion. | Sudden onset headache triggered by cough or Valsalva Cluster headache has several acute and preventive | Duration of severe pain 1 second to 2 hours options. Oxygen inhalation and subcutaneous sumatriptan | Brain MRI required to exclude secondary origins such as | Chiari malformation

Cc hronic paroxysmal hemicrania Trigeminal Autonomic Cephalalgias Symptoms like cluster headache, episodes shorter (2-30 minutes) TACs are the most severe and stereotypic primary headache | Responsive to indomethacin disorders. Pain is severe, localized to the periorbital or temporal Short-lasting unilateral neuralgiform headache attacks with areas, and associated with pronounced ipsilateral cranial auto- | conjunctival injection and tearing (SUNCT) | nomic features, such as nasal congestion or rhinorrhea and Symptoms like cluster headache, episodes shorter ptosis or miosis. Cluster headache is the most common TAC. (1-600 seconds) Cluster headache may last 15 to 180 minutes and recur one Medically refractory, reports of response to lamotrigine to eight times daily over a span of weeks to months; the shorter duration distinguishes cluster headache from migraine (Table 9). | Hemicrania continua | This headache is characterized by a cyclical nature in which Persistent strictly unilateral headache with autonomic features | periods of recurrent headache activity are interrupted by | Responsive to indomethacin months to years of headache remission. Many of the attacks are | Primary cough headache nocturnal, and some may be provoked by alcohol ingestion. | Sudden onset headache triggered by cough or Valsalva Cluster headache has several acute and preventive | Duration of severe pain 1 second to 2 hours options. Oxygen inhalation and subcutaneous sumatriptan | Brain MRI required to exclude secondary origins such as | Chiari malformation TABLE 9. International Headache Society Criteria for | Primary exercise headache Cluster Headache | Headache precipitated by any form of exercise in the |

TABLE 9. International Headache Society Criteria for | Primary exercise headache Cluster Headache | Headache precipitated by any form of exercise in the | A. At least five attacks fulfilling criteria B-D absence of any intracranial disorder B. Severe or very severe unilateral orbital, supraorbital, and/or | Duration <48 hours | temporal pain lasting 15-180 minutes (when untreated) | Primary headache associated with sexual activity | C. Either or both of the following: | | | Headache brought on by and occurring only during sexual | | 1. At least one of the following symptoms or signs, ipsilateral | activity in the absence of any intracranial disorder |

| Headache brought on by and occurring only during sexual | | 1. At least one of the following symptoms or signs, ipsilateral | activity in the absence of any intracranial disorder | to the headache: | Either or both of the following: | a) conjunctival injection and/or lacrimation | | 1. increasing in intensity with increasing sexual excitement | | b) nasal congestion and/or rhinorrhea 2. abrupt explosive intensity just before or with orgasm | c) eyelid edema | | d) forehead and facial sweating | High intensity for 1 minute to 48 hours, up to 72 hours mild | intensity | | |

to the headache: | Either or both of the following: | a) conjunctival injection and/or lacrimation | | 1. increasing in intensity with increasing sexual excitement | | b) nasal congestion and/or rhinorrhea 2. abrupt explosive intensity just before or with orgasm | c) eyelid edema | | d) forehead and facial sweating | High intensity for 1 minute to 48 hours, up to 72 hours mild | intensity | | | e) miosis and/or ptosis | Primary stabbing headache | ] 2. Asense of restlessness or agitation | Head pain occurring spontaneously as a single stab or series | of stabs | D. Attack frequency from one every other day to eight per day when the disorder is active | Each stab lasts for up to a few seconds and recur with | irregular frequency, from one to many per day | E. Headache not better accounted for by another ICHD-3 | diagnosis | No cranial autonomic symptoms

e) miosis and/or ptosis | Primary stabbing headache | ] 2. Asense of restlessness or agitation | Head pain occurring spontaneously as a single stab or series | of stabs | D. Attack frequency from one every other day to eight per day when the disorder is active | Each stab lasts for up to a few seconds and recur with | irregular frequency, from one to many per day | E. Headache not better accounted for by another ICHD-3 | diagnosis | No cranial autonomic symptoms | ICHD-3 = International Classification of Headache Disorders, 3rd edition. | No evidence for underlying secondary headache disorder | | Adapted with permission of SAGE Publications LTD., London, Los Angeles, New Adapted with permission of SAGE Publications LTD., London, Los Angeles, New | Delhi, Singapore and Washington DC, from Headache Classification Committee of Delhi, Singapore and Washington DC, from Headache Classification Committee of the International Headache Society (IHS). The International Classification of | the International Headache Society (IHS). The International Classification of | | Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):41. [29368949] Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):42-6, 48-51, 53-4. | doi: 10.1177/0333102417738202. [29368949] doi: 10.1177/0333102417738202. |

| ICHD-3 = International Classification of Headache Disorders, 3rd edition. | No evidence for underlying secondary headache disorder | | Adapted with permission of SAGE Publications LTD., London, Los Angeles, New Adapted with permission of SAGE Publications LTD., London, Los Angeles, New | Delhi, Singapore and Washington DC, from Headache Classification Committee of Delhi, Singapore and Washington DC, from Headache Classification Committee of the International Headache Society (IHS). The International Classification of | the International Headache Society (IHS). The International Classification of | | Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):41. [29368949] Headache Disorders, 3rd edition. Cephalalgia. 2018 Jan;38(1):42-6, 48-51, 53-4. | doi: 10.1177/0333102417738202. [29368949] doi: 10.1177/0333102417738202. | 10

Head Injury TABLE 11. Glasgow Coma Scale e Cluster headache and the other trigeminal autonomic || Eye Opening Score cephalalgias are the most severe and stereotypic primary Spontaneous 4 | headache disorders and are characterized by trigeminal Response to verbal command WwW pain and ipsilateral cranial autonomic features. | Response to pain NY ¢ Cluster headache is treated with oxygen inhalation and | No eye opening = subcutaneous sumatriptan. Best Verbal Response Oriented om fk Confused Head Injury | Inappropriate words [ i] . Traumatic Brain Injury Incomprehensible sounds ow

fk Confused Head Injury | Inappropriate words [ i] . Traumatic Brain Injury Incomprehensible sounds ow No verbal response 1 Traumatic brain injury (TBI) results from biomechanical forces applied to the structures of the head and neck. Damage may be Best Motor Response temporary or permanent and may arise from functional or Obeys commands DO | structural alterations in the central nervous system. Severity is Oo Localizing response to pain determined by clinical findings and imaging results. When | Withdrawal response to pain fF | indicated, noncontrast CT is the imaging modality of choice | Flexion to pain ww because of its wide availability to quickly assess for hemor- | rhage, high sensitivity for bony injuries, and relatively low Extension to pain we | expense. Mild TBI is associated with Glasgow Coma Scale No motor response 1 (GCS) scores of 13 to 15 with no or only a brief initial loss of Total | consciousness; results of head CT are typically normal. Moderate TBI is associated with GCS scores of 9 to 12 and/or an Data from Teasdale G, Jennett B. Assessment of coma and impaired consciousness. A practical scale. Lancet 1974 Jul 13;2(7872):81-4. [PMID: 4136544] | a initial loss of consciousness of 30 minutes to 24 hours. Severe TBI is associated with GCS scores of 3 to 8 and/or an initial loss new headache or worsen a preexisting headache condition. of consciousness of more than 24 hours (Table 11). In moderate Risk factors for postconcussion headaches include a history of and severe TBI, head CT may reveal a skull fracture, cerebral previous concussion, young age, female sex, and family or per- contusions and edema, and intracranial hemorrhage. sonal history of migraine. Posttraumatic headaches most com- monly have the characteristics of migraine and tension-type Mild Traumatic Brain Injury Mild TBI, also known as concussion, typically causes tempo- TABLE 12. Indications for Head CT in Mild Traumatic Brain rary neurologic impairment without evidence of structural Injury? damage on conventional neuroimaging. In most patients, the Age >60 years with loss of consciousness or age 265 years with | physical examination is normal and diagnostic investigation is no loss of consciousness unnecessary. Guidelines for the use of head CT in mild TBI Vomiting | have been published by the CDC (Table 12); CT in patients with Severe headache | mild TBI and any of the findings listed in the table has nearly 100% sensitivity for identifying patients with clinically impor- Posttraumatic seizure | tant findings but low specificity. Patients with mild TBI, a Drug or alcohol intoxication

No verbal response 1 Traumatic brain injury (TBI) results from biomechanical forces applied to the structures of the head and neck. Damage may be Best Motor Response temporary or permanent and may arise from functional or Obeys commands DO | structural alterations in the central nervous system. Severity is Oo Localizing response to pain determined by clinical findings and imaging results. When | Withdrawal response to pain fF | indicated, noncontrast CT is the imaging modality of choice | Flexion to pain ww because of its wide availability to quickly assess for hemor- | rhage, high sensitivity for bony injuries, and relatively low Extension to pain we | expense. Mild TBI is associated with Glasgow Coma Scale No motor response 1 (GCS) scores of 13 to 15 with no or only a brief initial loss of Total | consciousness; results of head CT are typically normal. Moderate TBI is associated with GCS scores of 9 to 12 and/or an Data from Teasdale G, Jennett B. Assessment of coma and impaired consciousness. A practical scale. Lancet 1974 Jul 13;2(7872):81-4. [PMID: 4136544] | a initial loss of consciousness of 30 minutes to 24 hours. Severe TBI is associated with GCS scores of 3 to 8 and/or an initial loss new headache or worsen a preexisting headache condition. of consciousness of more than 24 hours (Table 11). In moderate Risk factors for postconcussion headaches include a history of and severe TBI, head CT may reveal a skull fracture, cerebral previous concussion, young age, female sex, and family or per- contusions and edema, and intracranial hemorrhage. sonal history of migraine. Posttraumatic headaches most com- monly have the characteristics of migraine and tension-type Mild Traumatic Brain Injury Mild TBI, also known as concussion, typically causes tempo- TABLE 12. Indications for Head CT in Mild Traumatic Brain rary neurologic impairment without evidence of structural Injury? damage on conventional neuroimaging. In most patients, the Age >60 years with loss of consciousness or age 265 years with | physical examination is normal and diagnostic investigation is no loss of consciousness unnecessary. Guidelines for the use of head CT in mild TBI Vomiting | have been published by the CDC (Table 12); CT in patients with Severe headache | mild TBI and any of the findings listed in the table has nearly 100% sensitivity for identifying patients with clinically impor- Posttraumatic seizure | tant findings but low specificity. Patients with mild TBI, a Drug or alcohol intoxication normal neurologic examination, and (when necessary) a nor- Persistent drowsiness or short-term memory deficit mal head CT scan may be safely followed as outpatients. “Dangerous” mechanisms of injury (fall from height greater Symptoms following a mild TBI may be divided into four than 3 feet or 5 steps, ejection from a vehicle, being struck by a vehicle as a pedestrian) domains: physical, cognitive, psychological, and sleep-associ- ated (Table 13). Most symptoms resolve spontaneously within Glasgow Coma Scale score <15

normal neurologic examination, and (when necessary) a nor- Persistent drowsiness or short-term memory deficit mal head CT scan may be safely followed as outpatients. “Dangerous” mechanisms of injury (fall from height greater Symptoms following a mild TBI may be divided into four than 3 feet or 5 steps, ejection from a vehicle, being struck by a vehicle as a pedestrian) domains: physical, cognitive, psychological, and sleep-associ- ated (Table 13). Most symptoms resolve spontaneously within Glasgow Coma Scale score <15 7 to 10 days. Although traditional management has mandated | Focal neurologic deficit complete physical and cognitive rest, studies show that earlier Physical evidence of open, depressed, or basilar skull fracture resumption of activity results in improved outcomes. After 1 or Coagulopathy 2 days, patients should be advised to exercise as tolerated and, CT scan should be obtained if any of the following findings are present. | after 3 to 5 days of cognitive rest, they should begin to escalate Data from Centers for Disease Control and Prevention and American College of their mental activities. Emergency Physicians. Mild TBI pocket guide: guideline for adult patients: a part Headache is consistently the most common symptom and of CDC's “Heads Up" series. Available at: https://www.cdc.gov/traumaticbraininjury/ pdf/TBI_Pocket_Card-a.pdf. Accessed September 27, 2019. | is among the most disabling sequelae. Head trauma may cause 11

Head Injury TABLE 13. Symptoms of Mild Traumatic Brain Injury Physical | | Headache | Nausea/vomiting | ‘ Dizziness/vertigo Gait disturbance | Photophobia or phonophobia | Visual blurring | | Dysarthria | Seizures | { al | Cognitive Poor concentration | Mental “fogginess” or confusion | Poor memory | FIGURE 2. This patient demonstrates the characteristic findings of Battle sign, Learning impairment a retroauricular or mastoid ecchymosis appearing 1 to 3 days after sustaining a basilar skull fracture. Slowed reaction times |

| Mental “fogginess” or confusion | Poor memory | FIGURE 2. This patient demonstrates the characteristic findings of Battle sign, Learning impairment a retroauricular or mastoid ecchymosis appearing 1 to 3 days after sustaining a basilar skull fracture. Slowed reaction times | Psychological Severe Traumatic Brain Injury Irritability Severe TBI may present with an alteration in consciousness, | Depression seizures, repeated vomiting, or focal neurologic deficits. Anxiety Bilateral periorbital or mastoid bruising (Figure 2) and hemo- tympanum may be signs of basilar skull fracture. Sleep Associated The primary goal of initial management is prevention of Fatigue hypotension and hypoxia. Normalization of blood oxygenation Insomnia (arterial Po, > 60 mm Hg [8.0 kPa]) and blood pressure (systolic Hypersomnia >90 mm Hg) results in improved outcomes. Mannitol or hyper- Drowsiness tonic saline and mechanical hyperventilation are recommended as temporizing measures in the presence of elevated intracranial Vivid dreams pressure. Monitoring and controlling intracranial pressure and cerebral perfusion pressure result in reduced mortality. headaches, and treatment is matched to the headache pheno- Medical management includes deep venous thrombosis type. Acetaminophen, aspirin, NSAIDs, and triptans are effec- prevention, glycemic control, and treatment of infectious or tive acute therapies; opioids and butalbital products should be gastrointestinal complications. Nutritional support through avoided. $-Blockers, antidepressants, and antiepileptic drugs transgastric jejunal feeding by day 7 postinjury is recom- may be useful for headache prevention in patients with persis- mended. Fever should be controlled aggressively with aceta- tent posttraumatic headache. A seizure occurring within the minophen and cooling blankets. Either phenytoin or first week of a traumatic brain event is felt to be symptomatic levetiracetam can be used to reduce early posttraumatic sei- (closely related to neurologic or systemic insults) and does not zures. Glucocorticoids worsen the prognosis of severe TBI and warrant antiepileptic therapy. Visual, vestibular, and cognitive should be avoided. rehabilitation therapies may be helpful in certain settings. Referral to a healthcare professional who is expert in the man- agement of concussion is recommended for those patients with ¢ Medical management in the treatment of severe trau- symptoms extending beyond 2 weeks. matic brain injury includes normalization of blood oxy- genation and blood pressure, reduction of elevated intracranial pressure, thromboprophylaxis for deep e The symptoms of mild traumatic brain injury, which venous thrombosis, glycemic control, seizure preven- usually involves brief or no loss of consciousness, typi- tion, and aggressive control of fever; glucocorticoids cally resolve spontaneously within 7 to 10 days. should be avoided. ¢ Posttraumatic headaches most commonly have the characteristics of migraine and tension-type headaches, Epidural and Subdural Hematoma and treatment is matched to the headache phenotype; An epidural hematoma arises when an arterial structure is opioids and butalbital products should be avoided. breached and blood collects between the dura mater and skull

Psychological Severe Traumatic Brain Injury Irritability Severe TBI may present with an alteration in consciousness, | Depression seizures, repeated vomiting, or focal neurologic deficits. Anxiety Bilateral periorbital or mastoid bruising (Figure 2) and hemo- tympanum may be signs of basilar skull fracture. Sleep Associated The primary goal of initial management is prevention of Fatigue hypotension and hypoxia. Normalization of blood oxygenation Insomnia (arterial Po, > 60 mm Hg [8.0 kPa]) and blood pressure (systolic Hypersomnia >90 mm Hg) results in improved outcomes. Mannitol or hyper- Drowsiness tonic saline and mechanical hyperventilation are recommended as temporizing measures in the presence of elevated intracranial Vivid dreams pressure. Monitoring and controlling intracranial pressure and cerebral perfusion pressure result in reduced mortality. headaches, and treatment is matched to the headache pheno- Medical management includes deep venous thrombosis type. Acetaminophen, aspirin, NSAIDs, and triptans are effec- prevention, glycemic control, and treatment of infectious or tive acute therapies; opioids and butalbital products should be gastrointestinal complications. Nutritional support through avoided. $-Blockers, antidepressants, and antiepileptic drugs transgastric jejunal feeding by day 7 postinjury is recom- may be useful for headache prevention in patients with persis- mended. Fever should be controlled aggressively with aceta- tent posttraumatic headache. A seizure occurring within the minophen and cooling blankets. Either phenytoin or first week of a traumatic brain event is felt to be symptomatic levetiracetam can be used to reduce early posttraumatic sei- (closely related to neurologic or systemic insults) and does not zures. Glucocorticoids worsen the prognosis of severe TBI and warrant antiepileptic therapy. Visual, vestibular, and cognitive should be avoided. rehabilitation therapies may be helpful in certain settings. Referral to a healthcare professional who is expert in the man- agement of concussion is recommended for those patients with ¢ Medical management in the treatment of severe trau- symptoms extending beyond 2 weeks. matic brain injury includes normalization of blood oxy- genation and blood pressure, reduction of elevated intracranial pressure, thromboprophylaxis for deep e The symptoms of mild traumatic brain injury, which venous thrombosis, glycemic control, seizure preven- usually involves brief or no loss of consciousness, typi- tion, and aggressive control of fever; glucocorticoids cally resolve spontaneously within 7 to 10 days. should be avoided. ¢ Posttraumatic headaches most commonly have the characteristics of migraine and tension-type headaches, Epidural and Subdural Hematoma and treatment is matched to the headache phenotype; An epidural hematoma arises when an arterial structure is opioids and butalbital products should be avoided. breached and blood collects between the dura mater and skull 12

Head Injury FIGURE 3. CTscan of an epidural hematoma. Note the biconvex lens appearance as blood under arterial pressure collects between the skull and outer margin of the FIGURE 4. CTscan of a subdural hematoma. Note the crescent shape as blood dura (arrow). under venous pressure separates the dura from the arachnoid membrane (arrow).

FIGURE 3. CTscan of an epidural hematoma. Note the biconvex lens appearance as blood under arterial pressure collects between the skull and outer margin of the FIGURE 4. CTscan of a subdural hematoma. Note the crescent shape as blood dura (arrow). under venous pressure separates the dura from the arachnoid membrane (arrow). in a “lentiform” fashion (Figure 3). Most epidural hematomas involve a laceration of the middle meningeal artery from a tem- poral bone fracture. Lateral extension is limited by dural attach- e In patients with epidural hematoma, stupor or coma ments at the skull sutures, and expansion occurs inward toward with ipsilateral oculomotor nerve palsy and contralat- the brain parenchyma. Many patients with an epidural hema- eral hemiparesis may signal transtentorial herniation. toma have a “lucid interval” followed by rapid neurologic com- e Chronic subdural hematoma may be associated with promise. Headache, vomiting, and declining mental status may nonspecific symptoms, including headache, altered occur early. Stupor or coma with ipsilateral oculomotor nerve mental status, or somnolence, in addition to focal neu- (cranial nerve III) palsy and contralateral hemiparesis may sig- rologic findings. nal transtentorial (uncal) herniation. Urgent surgical evacua- tion is recommended for those with a GCS score less than 9, anisocoria, or a hematoma greater than 30 mL in volume. A subdural hematoma (Figure 4) is a collection of blood Head Injury in Special Populations between the brain and dura mater. Rupture of bridging veins Athletes within this space is typically responsible. This may occur Any athlete suspected of sustaining a mild TBI should be spontaneously, as a complication of anticoagulation, or after immediately removed from play and assessed by a health care trauma. Because of cerebral atrophy and subsequent tension provider trained in the evaluation and management of concus- on the subdural bridging veins, older persons and those with sion. Guidelines recommend initial screening with a symptom alcoholism are particularly susceptible. Presentations may be checklist and neurologic examination involving specific cogni- acute, subacute, or chronic. Whereas acute subdural hema- tive evaluation and balance testing. Specific instruments, such toma typically presents with coma or neurologic compromise, as the Sport Concussion Assessment Tool 5th edition (SCATS) subacute and chronic hematoma (which develops days to (http://dx.doi.org/10.1136/bjsports-2017-097506SCATS), are weeks after injury) may be associated with nonspecific symp- useful in the acute detection of sports-related concussion. toms-including headache, altered mental status, or somno- Neuropsychological testing provides an objective and more lence—in addition to focal neurologic findings. In patients with sensitive measure of cognitive function; it should be employed acute subdural hematomas, a hematoma thickness greater as part of a comprehensive TBI management program for than 10 mm, a score less than 9 on the GCS, and the presence patients with persistent symptoms. Return to play may be of pupillary asymmetry or fixation are all indications for considered after 1 or 2 days of physical and cognitive rest, reso- immediate surgical treatment. In patients with chronic hema- lution of symptoms for 7 days both at rest and with exertion, tomas, a hematoma thickness greater than 10 mm, a midline and normalization of cognition. There are no available guide- shift greater than 5 mm, and significant neurologic compro- lines for permanent disqualification from contact sports for mise are all indications for neurosurgical drainage. athletes with mild TBI.

in a “lentiform” fashion (Figure 3). Most epidural hematomas involve a laceration of the middle meningeal artery from a tem- poral bone fracture. Lateral extension is limited by dural attach- e In patients with epidural hematoma, stupor or coma ments at the skull sutures, and expansion occurs inward toward with ipsilateral oculomotor nerve palsy and contralat- the brain parenchyma. Many patients with an epidural hema- eral hemiparesis may signal transtentorial herniation. toma have a “lucid interval” followed by rapid neurologic com- e Chronic subdural hematoma may be associated with promise. Headache, vomiting, and declining mental status may nonspecific symptoms, including headache, altered occur early. Stupor or coma with ipsilateral oculomotor nerve mental status, or somnolence, in addition to focal neu- (cranial nerve III) palsy and contralateral hemiparesis may sig- rologic findings. nal transtentorial (uncal) herniation. Urgent surgical evacua- tion is recommended for those with a GCS score less than 9, anisocoria, or a hematoma greater than 30 mL in volume. A subdural hematoma (Figure 4) is a collection of blood Head Injury in Special Populations between the brain and dura mater. Rupture of bridging veins Athletes within this space is typically responsible. This may occur Any athlete suspected of sustaining a mild TBI should be spontaneously, as a complication of anticoagulation, or after immediately removed from play and assessed by a health care trauma. Because of cerebral atrophy and subsequent tension provider trained in the evaluation and management of concus- on the subdural bridging veins, older persons and those with sion. Guidelines recommend initial screening with a symptom alcoholism are particularly susceptible. Presentations may be checklist and neurologic examination involving specific cogni- acute, subacute, or chronic. Whereas acute subdural hema- tive evaluation and balance testing. Specific instruments, such toma typically presents with coma or neurologic compromise, as the Sport Concussion Assessment Tool 5th edition (SCATS) subacute and chronic hematoma (which develops days to (http://dx.doi.org/10.1136/bjsports-2017-097506SCATS), are weeks after injury) may be associated with nonspecific symp- useful in the acute detection of sports-related concussion. toms-including headache, altered mental status, or somno- Neuropsychological testing provides an objective and more lence—in addition to focal neurologic findings. In patients with sensitive measure of cognitive function; it should be employed acute subdural hematomas, a hematoma thickness greater as part of a comprehensive TBI management program for than 10 mm, a score less than 9 on the GCS, and the presence patients with persistent symptoms. Return to play may be of pupillary asymmetry or fixation are all indications for considered after 1 or 2 days of physical and cognitive rest, reso- immediate surgical treatment. In patients with chronic hema- lution of symptoms for 7 days both at rest and with exertion, tomas, a hematoma thickness greater than 10 mm, a midline and normalization of cognition. There are no available guide- shift greater than 5 mm, and significant neurologic compro- lines for permanent disqualification from contact sports for mise are all indications for neurosurgical drainage. athletes with mild TBI. 13