Approach

History . The cause of the patient’s headache is usually established from the history. Important features include the frequency, location, laterality, mode of onset, duration, nature of the headache, and the presence of accompanying symptoms along with precipitating and alleviating factors. Visual aura is highly suggestive of a migraine disturbance. Although nonspecific, photophobia, phonophobia, osmophobia, and gastrointestinal symptoms may also help to discriminate migraine from other causes of headache. The patient’s medical and psychiatric history and medication list provide essential information.

Classification . It is useful to classify headaches into those that are primary (or without specific cause) and those with an organic cause. Migraine, tension-type, and trigeminal autonomic cephalgias are the most common forms of primary headache. The International Headache Society (IHS) has established diagnostic criteria for a variety of headache disorders to provide uniformity in classification ( Boxes 19.1–19.4 ).

These guidelines were primarily created for the purpose of collecting epidemiologic and clinical trial data and may not be practical for use in the clinical setting. Diagnostic difficulty occurs because some patients have multiple headache syndromes and because headache characteristics can change. Additionally, the restrictive IHS requirements for a minimum number of headaches with a certain duration may exclude individuals who likely do have that headache type.

“Red-flag” headache characteristics suggesting a more ominous underlying cause are listed in Box 19.5 . These headaches require more aggressive evaluation, including neuroimaging and possibly lumbar puncture.

Epidemiology

Approximately 18% of women and 6% of men endure recurrent headache classified as migraine. Migraine prevalence is highest among 30–50-year-olds, with more white than black people and with an inverse relationship to income. Nearly 90% of patients with migraine report some disability associated with their headache, with half having severe disability. Patients with migraine may lose several days of work per year related to headache. Despite the high frequency of disability associated with migraine, many patients do not seek medical attention and rely on over-the-counter medications, with many probably inadequately treated. Patients with episodic migraine may develop a chronic disease state, formerly termed transformed migraine, but now called chronic migraine, characterized by 15 or more headaches per month.

Many neurologic and psychiatric disorders may have an association with migraine. According to one study, epilepsy patients have twice the risk of developing migraine than those without epilepsy and may have a shared genetic susceptibility. There is a higher incidence of stroke in patients with migraine, particularly in those with aura. Silent posterior circulation infarcts have been documented in migraine patients. Nonspecific deep white matter lesions on are commonly seen on magnetic resonance imaging (MRI), and there may be lesion progression, but their exact pathogenesis is unclear. Various affective disorders are related to migraine, including depression, manic depressive illness, and panic disorder. Cosensitization may explain the association of chronic migraine with depression and anxiety.

Genetic factors . A genetic basis for migraine is suggested by the observation that 70–90% of migraine patients have a positive family history. Migraine has been linked to chromosomes 1 and 19, with familial hemiplegic migraine (FHM) type 1 linked to chromosome 19. The defective gene likely encodes for a voltage-dependent P-type calcium channel. Chromosome 19 has also been linked to CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy), an autosomal dominant arteriopathy characterized by leukoencephalopathy, subcortical infarction, and migraines. Other studies of FHM suggest additional loci including FHM type 2 caused by a mutation in the gene ATP1A2 and FHM type 3 linked to a mutation in gene SCNA1A. Accumulating data suggest that migraine may be a channelopathy, a condition that ultimately leads to neuronal hyperexcitability.

Pathophysiology

Pain-sensitive structures of the head and face include the skin and blood vessels of the scalp; dura; venous sinuses; arteries; and sensory fibers of the fifth, ninth, and tenth nerves . Although the mechanism of migraine remains uncertain, evidence suggests an important role for the trigeminal vascular projections and the neurochemical serotonin. The trigeminal nerve has terminals on the pial and dural blood vessels that release a variety of vasoactive neuropeptides, including substance P, neurokinin A, and calcitonin gene-related peptide (CGRP). Release of these neuromediators produces sterile neurogenic inflammation by dilating meningeal blood vessels and increasing vascular permeability. Sumatriptan and dihydroergotamine (DHE) act on presynaptic 5HT ₁ D receptors, blocking the release of these neuroactive peptides and 5HT ₁ B receptors to selectively constrict dilated meningeal vessels. Additionally, 5HT ₁ B/D agonists such as the triptans that cross the blood–brain barrier may also block neurons of the trigeminal nucleus caudalis within the medulla.

Evidence for the involvement of brainstem structures is supported by positron emission tomography studies that show increased blood flow in the reticular activating formation during the migraine episode. The median raphe nucleus, the periaqueductal gray matter, and the locus ceruleus are regions thought to be activated during a migraine. Activated parasympathetic projections from the superior salivatory nucleus may produce vasodilation of meningeal vessels.

Mechanism of migraine aura . While classic explanations for migraine attributed the aura to vasoconstriction and the headache to vasodilation, contemporary theories suggest otherwise. For instance, blood flow studies have demonstrated reduced flow preceding the onset of the aura but continuing into the headache phase, which would be during a period of vasoconstriction. The aura of migraine is thought to be related to the concept of a spreading cortical depression, which was promulgated by Leão, who demonstrated that electrical depression moved over the cortex at a rate of 2–3 mm per minute. Leão’s observation was supported by Lashley, who calculated his own aura progressing over the visual cortex at the same rate of 2–3 mm per minute. Although blood flow reduction spreads over the cortex at this rate, current thinking attributes this to a secondary event triggered by a primary neurogenic process. Perfusion-weighted imaging, positron emission tomography, magnetoencephalography, and functional MRI support the notion of cortical spreading of oligemia and neuronal depression.

Lance proposed that the neuroadrenergic locus ceruleus, which diffusely projects to the cerebral cortex, may be one neural network involved in the generation of migraine aura. Activation of the locus ceruleus has been implicated in the development of photophobia and phonophobia. Ultimately, the locus ceruleus and the spinal nucleus of the trigeminal nerve are influenced by the cortex and other higher structures. Functional MRI studies suggest that the red nucleus and substantia nigra may also be involved in this neural network. Cortical spreading depression (CSD) may release a variety of substances in the cortex via neuronal channel opening and parenchymal inflammatory cascades, such that suppressing these events have abolished CSD-induced trigeminal vascular activation, mast cell granulation, and headache. How aura and headache are related mechanistically remains uncertain.

Integrated theory . Presently, the precise pathophysiology of migraine remains uncertain, but a cascade of events seems likely. In summary, migraine appears to be initiated by hyperexcitability of cortical and brainstem neurons ( Fig. 19.1 ). The overactivation of these neurons may result from an underlying channelopathy seen in genetically predisposed individuals. Presumably, these hyperexcitable neurons are responsible for activating portions of the cortex (aura) and meningeal vessels (pain). The firing of these hyperexcitable neurons may be influenced by internal and external factors such as hormonal balance, food, stress, and sleep deprivation. The different symptoms observed in migraine probably reflect differential activation of cortical, brainstem, and vascular structures.

Trigeminal vascular theory of migraine headaches. Cortical hyperexcitable neurons, perhaps with an additional influence from brainstem structures, leads to activation of the trigeminovascular system. Signals reach the trigeminal nucleus caudalis in the brainstem, which in turn signals the thalamus, which innervates cortical regions, leading to the conscious sensation of headache and pain. Note activation of the trigeminal nerve afferents lead to neuropeptide release and vasodilation of dural blood vessels, which in turn leads to release of more neuropeptides, perpetuating the cycle. CGRP = calcitonin gene-related peptide, NO = nitric oxide.

In some patients, the migraine process persists, and central sensitization occurs. Continued peripheral sensory input can lead to sensitization of central (second order) trigeminovascular neurons and third-order neurons in the thalamus. The centrally based pain neurons may continue to propagate pain impulses even in the absence of further input from the peripheral trigeminal neurons. Clinically, the patient may develop cutaneous allodynia, during which normally nonpainful tactile stimuli become painful. Such patients may experience localized pain with routine activities such as wearing eyeglasses, combing hair, or taking a shower. In this stage, the patient’s pain may be refractory to triptan therapy. Chronic migraine may result from repeated episodes of cutaneous allodynia.

Symptoms and Signs

A variety of factors may provoke migraine, including menstruation, change in sleep patterns, hunger, stress, certain foods, odors, and even the type of lighting. Hours to days before the onset of migraine, the patient may experience prodromal symptoms manifesting as mood swings, food cravings, anorexia, fluid retention, or urinary frequency.

Headache . The headache of migraine is usually unilateral, throbbing, and of moderate severity (see Box 19.1 ). It may start any time but has a tendency to start in the morning. The onset is usually gradual, and this feature helps to distinguish migraine from more ominous causes such as a subarachnoid hemorrhage. Headaches usually last several hours and may be exacerbated by physical activity. The headache may pass through stages ranging from mild to severe. Various symptoms may accompany the headache, including photophobia, phonophobia, osmophobia, nausea, vomiting, and autonomic symptoms (see Box 19.1 ). Patients may also become irritable, anxious, or depressed during the headache phase.

Aura . The aura of migraine usually begins before the headache, but occasionally it will occur simultaneously or follow it (see Box 19.2 ). Approximately one-third of migraine patients experience an aura. Classically, the aura is visual, but sensory dysfunction may occur. Visual symptoms crescendo over several minutes and resolve by 30 minutes but occasionally will extend to 60 minutes. Some patients will have recurrent auras. Many patients cannot determine whether the phenomenon occurred monocularly or binocularly. Often patients believe a hemifield disturbance occurred in one eye. Although fortification scotomas are the characteristic visual disturbance, patients may also describe bright flashing lights or distorted vision. Some patients describe their vision as looking through clouded or prismatic glass. Classically, an arc of flashing zigzag light starts in the paracentral area of one hemifield and expands peripherally. As the wave of visual disturbance travels across the visual field, it may rotate or undulate. Other patients describe tunnel vision or a bilateral hemifield disturbance. Unusual visual illusions may occur, such as palinopsia (perseveration of visual images) or the Alice in Wonderland syndrome. In the latter, the patient may appreciate objects as overly large, small, distorted, close, or far.

Rarely, patients have persistent positive visual phenomena. The neuroimaging does not show any evidence of infarction, and the aura symptoms persist beyond 1 week. Various types of positive visual aura are discussed in greater detail in Chapter 12 , and the negative visual auras (vision loss) in Chapter 10 .

Sensory disturbance may include paresthesias, dysesthesias, numbness, or hyperesthesia, and these sensory symptoms may follow the headache. Classically, the sensory symptoms begin in the fingertips and march up the arm into the perioral area. Sensory auras are most convincingly migraine when preceded by a classic visual aura. Language and motor auras are uncommon, but their initial occurrence should prompt investigation for transient cerebral ischemia.

Interictal Heightened Visual Sensitivity

Between episodes of headache, migraineurs may have a heightened sensitivity to bright lights, glare, and other visual stimuli. Patients may complain of sensitivity to flickering lights (strobe and fluorescent), patterned stimuli, moving objects, and supermarket aisles (visual vertigo). These visual stimuli may cause headaches, perhaps in part by lowering trigeminal and cervical pain thresholds. Migraineurs may have impaired inhibitory subcortical function that normally suppresses glare-induced pain. Alternative explanations include primary visual cortex and V5 hypersensitivity or reduced inhibition by extrastriate structures. Detailed psychophysical and electrophysiologic studies have also demonstrated that interictal visual function may be subtly altered in migraineurs.

Migraine Subtypes

Migrainous Infarction

The term migrainous infarction refers to migraine aura symptoms associated with a permanent neurologic deficit (visual, motor, or sensory) during the course of a typical migraine with aura attack. MRI may demonstrate the cerebral ischemic changes that typically occur in younger women and in the occipital–parietal regions ( Fig. 19.2 ).

T2-weighted axial image in a patient with migrainous infarction, demonstrating high signal abnormality ( arrow ) in the right occipital lobe.

While there is no effective therapy for evolving migrainous infarction, we treat with high-dose intravenous methylprednisolone and hydration. Triptan medications should be avoided because of their vasoconstrictive properties. Sublingual calcium channel blockers may reduce vasospasm, but this beneficial effect must be weighed against possible hypotension.

Diagnostic Testing in a Patient With Suspected Migraine

Visual fields should be assessed in all new patients with suspected migraine visual aura to exclude field defects. A homonymous defect, for instance, would suggest alternative diagnoses such as release hallucinations or seizures due to a neoplasm.

In patients with a long history of typical migraine and a normal examination, it may be reasonable to defer neuroimaging. However, we have a low threshold for performing MRI in most patients, because there are many causes of headache, and several organic disorders may mimic or trigger migraine, particularly in patients with new onset of symptoms in middle age, atypical features, or marked increase in frequency of headache (see Box 19.5 ). Routine blood work is often unhelpful in young patients, but an erythrocyte sedimentation rate and C-reactive protein is critical in elderly patients with transient loss of vision or headache to help exclude temporal arteritis. Lumbar puncture, obtained after neuroimaging, is an important test when a subarachnoid hemorrhage, meningitis, or pseudotumor cerebri syndrome is suspected.

Treatment

Migraine treatment depends on the characteristics of the pain. Abortive treatment alone is recommended when the attacks occur less than twice a week and are short lived ( Table 19.1 ). Prophylactic therapy is added when the headaches are more frequent or disabling to the patient’s occupation or social activities. Patients with frequent migraine are treated with a combination of preventive and abortive therapies.

Acephalgic Migraine (Typical Aura Without Headache)

Patients with migraine may have transient neurologic events without headache. When such events occur in patients older than 40 years, concern of a possible transient ischemic attack (TIA) is raised. In any age group, seizures may be considered. Fortunately, there are features that help differentiate these disorders. The migraine aura has a characteristic buildup of scintillations. The episodes may occur in a flurry and tend to last longer than a typical TIA. Patients often have a remote history of migraine headaches. Some even have mild accompanying discomfort but do not think it is important to report it. The intensity of their headache is often not as severe as it was previously.

In a younger patient with visual aura without headache, but with a previous history of migraine, further testing may be unnecessary. However, in patients older than 40 years, the new onset of acephalgic migraine is a diagnosis of exclusion. Workup would include neuroimaging to exclude a lesion of the occipital cortex and to check for ischemic changes. Electroencephalography can be performed to rule out an irritative lesion. When the aura is characterized by visual loss, cardiac emboli, carotid insufficiency, and hypercoagulable states should also be considered.

Treatment of acephalgic migraine . If tolerated, 325 mg of aspirin per day may be given, particularly in elderly individuals in whom the diagnosis may be uncertain. It may lessen acephalgic migraine episodes and help prevent stroke in case the events were in fact TIAs. In patients in whom aspirin is ineffective or intolerable, calcium channel blockers (verapamil, 120–360 mg (SR) per day), can be considered for those with recurrent disabling episodes. Often, patients are reassured knowing that their visual episodes are migrainous and benign and decline pharmacologic treatment.

Chronic Daily Headache

Chronic daily headache is a category of headaches including chronic migraine (described already), chronic tension-type headaches, hemicrania continua, new daily persistent headache, and posttraumatic headaches. Affected patients have headaches for 15 or more days per month, and the majority have an element of medication-overuse headache.

Tension-Type Headaches

Tension headaches are probably a continuum of migraine headaches. The pain is mild to moderate in intensity and is often described as pressure or tightness. There is no aura, nausea, or vomiting, and the headaches are usually not aggravated by exercise (see Box 19.3 ). Photophobia and phonophobia are rarely present, and the headache location is often bilateral, over the eyes, temples, occipital region, or on top of the head. Patients often have tender spots over the shoulder girdle muscles. Most tension headaches are episodic; however, some patients have chronic tension-type headaches without pain-free intervals.

Many patients with chronic daily tension headaches have a history of migraine, and migraines may periodically occur. Many of these patients have concurrent depression. In patients with tension headache, one should be aware of the possibility of secondary headache disorders or rebound headaches from analgesic overuse (see Medication-Overuse Headache ).

Treatment . Such patients with tension headaches pose a therapeutic challenge. The abortive and prophylactic medications used in migraineurs may be tried. Nortriptyline and amitriptyline may be the first-line agents for these patients, and topiramate may be effective. Patients with tension headaches should be assessed for temporomandibular joint dysfunction or sinus disease before starting preventive therapy. Some patients with tension headache do not respond to medication and may benefit from other therapies, including stretching exercises and acupuncture. Botulinum toxin injected into the frontal and occipital muscles may be used in refractory tension headache, but studies have not demonstrated fewer headaches in this subset of patients.

Medication-Overuse Headache

Medication-overuse headaches share similar clinical characteristics with tension headaches. Although rebound headache is classically associated with overuse of narcotics (taken 10 days a month or more) or over-the-counter analgesics (taken 15 days a month or more), this phenomenon also may be observed with triptan and ergot overuse.

Treatment . It is important to taper the overused medication, exercising caution with quick discontinuation of certain responsible medications (barbiturates, benzodiazepines, opioids, or caffeine-containing compounds) because of the potential for life-threatening complications. If not already a contributing factor, NSAIDs or a short course of corticosteroids may help the patient’s symptoms. During the taper, adding botulinum toxin may be effective in patients with acute medication-overuse headache and baseline chronic migraine. If outpatient detoxification fails, inpatient management may be necessary. Intravenous DHE, 1 mg, combined with 10 mg of intravenous metoclopramide every 8 hours for 3–5 days is one effective regimen to treat analgesic overuse.

Cluster Headache

Cluster headache is a severe unilateral headache disorder that predominantly occurs in men (see Box 19.4 ). These headaches occur in clusters or on a daily basis for weeks to months. Although most cluster headaches are episodic, rarely they are chronic and without remission. Cluster headaches are often severe, awakening a patient from sleep. They localize over the periorbital region and are often associated with rhinorrhea and tearing. Alcohol is a common trigger. The attacks are much shorter than migraine, lasting 0.5–3 hours. There is often no nausea or vomiting; aura is rare but can occur. Many patients develop signs of a Horner syndrome. Occasionally, only ptosis or miosis is present. After several attacks, a permanent Horner syndrome may ensue. Contrasting the migraine patient who desires sleep, a cluster headache patient cannot stay still. The headache is so severe that some patients literally run around the room screaming, and some patients even contemplate suicide. Interictally, some patients will suffer typical migraine headaches.

The major entity to exclude in a patient with a painful Horner syndrome is a carotid dissection (see Chapter 13 ). Other entities that may mimic cluster headaches include arteriovenous malformation, brainstem compression by tumor, vertebral artery aneurysm, cavernous carotid artery aneurysms, sinusitis, temporal arteritis, Tolosa–Hunt syndrome, orbital myositis, and pituitary tumors. In patients presenting with a painful Horner syndrome, MRI of the neck, including axial T1-weighted images and angiographic sequences, is recommended to exclude carotid dissection.

Treatment . The treatment of acute cluster attack may be difficult. Emergently, the following regimens may be tried:

• 1.
  

  100% oxygen therapy via a face mask at 12–15 L/min for 15–20 minutes

  
  
• 2.
  

  Subcutaneous sumatriptan or intranasal sumatriptan or zolmitriptan

  
  
• 3.
  

  Intranasal or intravenous DHE

  
  
• 4.
  

  Intranasal lidocaine 4%

  
  
• 5.
  

  Corticosteroids (2-week prednisone taper)

Corticosteroids often take hours to begin to work. However, they may be useful in transition to other preventive therapies. Verapamil is considered first-line prophylactic treatment in prolonged episodes or chronic cluster headache and is comparable to lithium. Other options include twice-daily ergotamine therapy. Indomethacin may benefit certain patients, and valproic acid may provide effective prophylaxis. The newer anticonvulsants including gabapentin, pregabalin, topiramate, and lamotrigine may be tried.

When patients are refractory to monotherapy, combination therapy may be necessary. Verapamil and lithium are usually tried together first. Valproate and verapamil is another effective combination.

Rarely, patients may require intravenous DHE every 8 hours to break the cluster. When all medications fail and headaches are strictly unilateral, radiofrequency thermocoagulation of the Gasserian ganglion or trigeminal nerve section may be tried. However, the resultant corneal and trigeminal anesthesia may lead to complications, and the procedures are not always effective. Intractable cluster headaches have also been treated by deep hypothalamic, occipital nerve, or sphenopalatine ganglion stimulation.

Paroxysmal Hemicrania and Hemicrania Continua

Paroxysmal hemicrania refers to a headache syndrome characterized by brief episodes of unilateral pain localizing over the orbital and temporal regions lasting 2–45 minutes. Patients experience multiple attacks a day, and some have 40–50 attacks in a 24-hour period. The pain is associated with autonomic dysfunction such as conjunctival injection, lacrimation, nasal congestion, ptosis, and eyelid edema. While there are many similarities between paroxysmal hemicrania and cluster headache, paroxysmal hemicrania is distinguished by its shorter duration and exquisite response to indomethacin. It has been described as a disorder in women, but other studies did not demonstrate a female preponderance. Although indomethacin is the treatment of choice, other patients may respond to topiramate, verapamil, acetazolamide, or other NSAIDs. Some patients will have superimposed mild and dull hemicranial discomfort between episodes.

Headaches which are similar in quality but continuous are termed hemicrania continua and are also responsive to indomethacin.

SUNCT Syndrome

The acronym for this syndrome stands for “ s hort-lasting, u nilateral, n euralgiform headache with c onjunctival injection, and t earing.” Patients may experience hundreds of attacks a day lasting 5–240 seconds. This rare condition is similar to the paroxysmal hemicranias but is distinguished by less severe pain, more marked autonomic activation, and unresponsiveness to indomethacin. Lamotrigine is the most effective treatment in nearly two-thirds of patients, but gabapentin, topiramate, and zonisamide can also be used prophylactically. Like cluster headaches, SUNCT syndrome may be mimicked by pituitary tumors and posterior fossa lesions.

Ice Pick Headache (Primary Stabbing Headache)

Some patients experience sudden stabbing pain that may be described as like being stabbed with an ice pick. The pain is often located in the temple region, but the occipital area may also be involved. The pain often lasts seconds but may persist for minutes. Many patients have underlying migraine or tension headaches. The pain may occur several times a day and may awaken a sleeping patient. Occasionally, the examiner can palpate the area and elicit the pain. The pain of a corneal erosion may be brief and present upon awakening and should be carefully considered in a patient with stabbing periorbital pain. Patients respond best to indomethacin or other NSAIDs.

Cough Headache

Patients with cough headache may be divided into two broad groups. First, cough-induced headaches may be associated with an intracranial lesion, particularly a posterior fossa lesion. Arnold–Chiari tumors and subdural hematomas are other causes. Given this differential diagnosis, neuroimaging is always indicated with this type of headache.

Patients in the second group with cough-induced headache have no detectable intracranial lesions despite extensive investigation. These patients often respond to a course of indomethacin or other NSAID.

Thunderclap Headache and the Reversible Cerebral Vasoconstriction Syndrome

Thunderclap headache. This term is used to describe a sudden and severe headache. Although controversy exists regarding the significance of such a headache, we believe an intracerebral hemorrhage or an unruptured aneurysm has to be strongly considered. Raps et al. concluded that thunderclap headache may be induced by a variety of mechanisms, including thrombosis or expansion of an aneurysm or intramural hemorrhage. Some patients with thunderclap headache have no identifiable cause. Patients with thunderclap headache should be evaluated with emergent computed tomography (CT) and lumbar puncture. If both are negative, and the suspicion remains high for an unruptured aneurysm, CT or MRA should be performed.

Reversible cerebral vasoconstriction syndrome . One of the most common causes of thunderclap headache with associated diffuse, segmental reversible cerebral vasospasm is referred to as reversible cerebral vasoconstriction syndrome (RCVS). It can spontaneously occur and resolve within 1–3 months. Risk factors of RCVS include being postpartum or having a history of migraine. Vasoactive substances that can induce RCVS include cannabis, cocaine, amphetamines, ecstasy, ergotamine, triptans, nasal decongestants, and SSRIs. Combining binge drinking and the use of vasoactive substances (particularly cannabis) is a significant risk factor. Patients present with recurrent thunderclap headaches, normal or near-normal cerebrospinal fluid, and reversible vasospasm of cerebral arteries noted on conventional cerebral angiography, CT, or MRA. Patients may have the headache alone or in combination with neurologic symptoms including deficits in mental status, motor examination, sensation, speech, ataxia, and seizures. Visual symptoms of blurred vision, vision loss, visual field defect, and retroorbital eye pain have been described. The main complications of RCVS are ischemic or hemorrhagic stroke (7%; see Chapter 8 ) and subarachnoid hemorrhage (22%). The utility of treating RCVS with calcium channel blockers is inconclusive. Even without treatment, patients typically have complete or near resolution of vasospasm within 3 weeks of RCVS onset.

Greater Occipital Neuralgia

Some patients experience episodic pain in the back of head at the exit site of the greater occipital nerve. The pain often ascends ipsilaterally along the nerve’s distribution behind the ear then occasionally forward toward the eye. The discomfort usually lasts seconds but may recur frequently during the day. The patient typically has pain and tenderness at the point where the greater occipital nerve pierces the tendinous insertion of the splenius capitis muscle at the skull base. Many patients have superimposed tension and migraine headaches.

Treatment with local injection of lidocaine has a high success rate. Occasionally repeated injections are necessary to break the cycle. If patients do not respond to local lidocaine injection, NSAIDs, carbamazepine, gabapentin, a soft cervical collar at night, or a short course of corticosteroids may be tried. Refractory cases may be treated with occipital nerve stimulation and very rarely surgical decompression or radiofrequency ablation.

Hypnic Headache

Elderly patients may experience headaches shortly after falling asleep. The headache is throbbing and global in location, is short lived, and usually resolves within an hour. This disorder is also recognized in young patients and may be unilateral. Caffeine at bedtime is usually the first-line treatment of these headaches. Lithium may also prevent the headaches, but its side-effects in the elderly population may limit its use.

Carotid Dissection

Patients with acute carotid dissection may develop throbbing pain over the periorbital area. Many patients will have accompanying neurologic signs such as a Horner syndrome or contralateral weakness or numbness. A patient with an isolated painful Horner syndrome should be considered to have a carotid dissection until proven otherwise. Other clinical manifestations, radiographic features, and management of this condition are discussed in detail in Chapter 13 .

Temporal Arteritis

The headache of temporal arteritis is often described as an ache concentrated over the temporal arteries. The pain rarely can be severe and the area exquisitely sensitive to touch. Although the pain is often concentrated over the temporal region, it may occur over any portion of the head. The pain typically abates within days of starting corticosteroids. This condition, particularly with regard to visual loss, pathophysiology, and management, is reviewed in Chapter 5 .

Posttraumatic Headache

Mild to moderate headaches are common after head injury. Patients may or may not have experienced a loss of consciousness. Typically, the headache is one of many symptoms that accompany the posttraumatic syndrome. Vertigo, irritability, concentration difficulty, and short-term memory loss are other frequent complaints. The posttraumatic headache may simulate tension headaches or migraine headaches and may improve spontaneously or persist indefinitely. The IHS criteria require the onset of the headache to be within 7 days after the injury or upon return of consciousness. Migraine, common daily headache, and cluster may follow mild head trauma. Posttraumatic headaches may last for several years, regardless of financial compensation issues. If tolerated, antidepressant compounds may reduce some of the symptoms. Other prophylactic headache medications such as propranolol, verapamil, topiramate, and valproic acid may also be tried with variable success.

Glossopharyngeal Neuralgia

Patients with glossopharyngeal neuralgia experience pain in the pharynx and base of the tongue. The pain may be triggered by swallowing, coughing, or talking. and the pain is usually sharp, stabbing, or lancinating. The condition is usually idiopathic, but mass lesions should be carefully considered. As in patients with trigeminal neuralgia, most cases result from compression by a dolichoectatic artery or venous structure. Treatment of glossopharyngeal neuralgia includes anticonvulsants, antidepressants, and baclofen therapy. If medical therapy fails, the glossopharyngeal nerve may be thermocoagulated. Other patients may benefit from microvascular decompression.

Intracranial Hypotension

Headache is a prominent symptom of patients suffering from intracranial hypotension, also termed CSF hypovolemia syndrome . Patients may have associated nausea, vomiting, tinnitus, and photophobia. The hallmark feature of this disorder is pain aggravated by an upright posture, but the rare patient may paradoxically have a headache when lying down. Some patients will develop unilateral or bilateral sixth nerve palsies resulting from the traction on these nerves as the brain sinks downward from the intracranial hypotension. Rarely, patients may complain of transient visual obscurations or blurred vision, and binasal field defects have been documented. Rare complications include nystagmus from eighth nerve dysfunction and enophthalmos. The downward displacement of the brain is seen on sagittal MRI, and diffuse meningeal enhancement is often present ( Fig. 19.3A,B ). If severe, bilateral subdural hematomas or organized hygromas ( Fig. 19.3C ) may develop.

Only gold members can continue reading. Log In or Register to continue

Share this:

• Click to share on Twitter (Opens in new window)
• Click to share on Facebook (Opens in new window)

Related

Related posts:

1. The Neuro-Ophthalmic Examination
2. Pupillary Disorders
3. Transient Visual Loss or Blurring
4. Eye Movement Disorders: Third, Fourth, and Sixth Nerve Palsies and Other Causes of Diplopia and Ocular Misalignment

Stay updated, free articles. Join our Telegram channel

Join