Migraine with Aura Classic Migraine

Diagnostic Criteria

At least two attacks fillfilling the following: At least three of the following four characteristics:

1. One or more fully reversible aura symptoms

2. At least one aura symptom over more than 4 minutes or 2 or more symptoms occurnng in succession

3. No single aura symptom lasts more than 60 minutes

4. Headache follows aura with a free interval of less than 60 minutes (it may also begin before or simultaneously with the aura) Migraine Without Aura

Diagnostic Criteria

At least five attacks fulfilling the following:

Headache lasting 4 to 72 hours (untreated or unsuccessfully treated) Headache has at least two of the following characteristics:

1. Unilateral location

2. Pulsating quality

3. Moderate or severe intensity (inhibits or prohibits daily activities)

4. Aggravation by walking stairs or similar routine physical activity During headache at least one of the following:

1. Nausea and/or vomiting

2. Photophobia and phonophobia For Both Disorders

History, physical and neurological examinations, and appropriate investigations must adequately exclude secondary disorders. If a secondary condition co-exists, the migraine is considered primary only if the original migraine onset did not occur in close temporal relation with the other disorder.

phase, no phase is obligatory for its diagnosis. Migraine with aura may occur with or without the headache, but migraine without aura requires the headache for its diagnosis (Ta.ble,..5.3.-2) . Premonitory phenomena occur hours to days before the onset of headache in about 60 percent of migraineurs (Table. S^-S) . They include psychological, neurological, constitutional, and autonomic features. The migraine aura is a complex of focal neurological symptoms (positive or negative phenomena) that precedes or accompanies an attack. Most aura symptoms develop over 5 to 20 minutes and usually last less than 60 minutes. The aura can be characterized by visual, sensory, or motor phenomena and may also involve language or brain stem

TABLE 53-3 -- MIGRAINE PRODROME SYMPTOMS

Psychological

Constitutional and Autonomic

Depression

Stiff neck

Hyperactivity

Food cravings

Euphoria

Cold feelings

Talkativeness

Anorexia

Irritability

Sluggishness

Drowsiness

Diarrhea or constipation

Restlessness

Thirst

Urination

Neurological

Fluid retention

Photophobia

Difficulty concentrating

Phonophobia

Dysphasia

Hyperosmia

Yawning

disturbances. If the aura is prolonged, it may meet the criteria of what was formerly termed a complicated migraine. Headache, when present, usually occurs within 60 minutes of the end of the aura, and most patients do not feel well during the period from the end of the aura to the beginning of the headache. Many patients experience a variety of cognitive or emotional symptoms during this time. y

The most frequently occurring aura is visual in nature. Elementary visual disturbances, including scotomata, photopsia, phosphenes (simple flashes), specks, geometrical forms, shimmering, or undulations are the most common and may occur singly or number in the hundreds. Interestingly, these elementary visual disturbances are more likely to occur during than before the headache. An occipital lobe origin is presumed based on their presence in both eyes and often in a hemifield distribution. More complicated hallucinations include teichopsia or fortification spectrum, which is the most characteristic visual aura and nearly diagnostic of migraine. An arc of scintillating lights, usually but not always beginning near the point of fixation, may form into a herringbone-like pattern that expands to encompass an increasing portion of a visual hemifield. It migrates across the visual field with a scintillating edge of often zigzag, flashing, or occasionally colored phenomena. y , [18] Visual distortions and hallucinations, speculated to repre- sent some of Lewis Carroll's descriptions in Alice's Adventures in Wonderland, occur more commonly in children. They are characterized by a complex disorder of visual perception that may include metamorphopsia, micropsia, macropsia, zoom vision (opening up or closing down in the size of objects), or mosaic vision (fracture of image into facets). y

Paresthesias are the second most common aura and typically start in the hand, migrate up the arm, and then extend to involve the face, lips, and tongue. Paresthesias can become bilateral and may be followed by numbness and loss of positional sense. y , y They typically progress over a period of more than 5 minutes and are often associated with a visual aura. More complex symptoms include difficulties in the perception and use of the body; speech and language disturbances; states of double or multiple consciousness associated with dej vu or jamais vu; and elaborate dreamy, nightmarish, trancelike, or delirious states. Motor symptoms, when they occur, are usually associated with sensory symptoms, but true weakness is rare and usually unilateral.

The typical migraine headache is unilateral, throbbing, moderate to marked in severity, and aggravated by physical activity. Not all of these features are required. The pain may be bilateral at the onset or start on one side and become generalized. y , y The onset is usually gradual. The pain peaks and then subsides, and usually lasts between 4 and 72 hours in adults and 2 and 48 hours in children. The frequency of attacks is extremely variable, from a few in a lifetime to several a week, and the average migraineur experiences from one to three headaches a month. The head pain varies greatly in intensity, although most migraineurs report pain ratings of five or greater on a 0 to 10 pain scale. Pain is throbbing in 85 percent of cases, y , y although throbbing pain is not diagnostic of migraine because it is often described in other headache types.

The pain of migraine is invariably accompanied by other features. Anorexia is common, and nausea occurs in almost 90 percent of patients, while vomiting occurs in about one third of patients.y Many patients experience sensory hyperexcitability manifested by photophobia, phonophobia, and osmophobia, and seek a dark, quiet room. Blurred vision, nasal stuffiness, diarrhea, polyuria, pallor, or sweating may be noted during the headache phase. There may be localized edema of the scalp or face, scalp tenderness, prominence of a vein or artery in the temple, or stiffness and tenderness of the neck. Impairment of concentration and mood are common. Lightheadedness, rather than true vertigo, and a feeling of faintness may occur. The extremi- ties tend to be cold and moist. Following the headache, during the postdrome phase, the patient may feel tired, "washed out," irritable, and listless, and may have impaired concentration, scalp tenderness, or mood changes. Some people feel unusually refreshed or euphoric after an attack, whereas others note depression and malaise.

A variety of migraine clinical subtypes have been described. Basilar migraine, previously called "basilar artery migraine," was originally believed to be a disorder of adolescent girls, and although there is a clear female predominance, it is now known to affect all age groups. Severe headache is preceded by a visual aura, then brain stem signs of ataxia, vertigo, tinnitus, diplopia, nausea and vomiting, nystagmus, dysarthria, bilateral paresthesia, and a change in level of consciousness and cognition. Confusional migraine, which occurs more commonly in boys than in girls, is characterized by headache accompanied by inattention, distractibility, and difficulty maintaining speech and other motor activities. Spells of basilar and confusional migraine can present a confusing picture and should be considered in patients with paroxysmal brain stem disturbances.^ , y

Hemiplegic migraine occurs in two forms, sporadic and familial, both of which typically begin in childhood and cease with adulthood. y The age of onset of hemiplegic migraine may be earlier than that of common migraine. The attacks are frequently precipitated by minor head injury. Accompanying changes in consciousness ranging from confusion to coma are a feature of hemiplegic migraine, especially in childhood, and occur in 23 percent of patients. The hemiplegia may be part of the aura and last less than 1 hour, or it may continue through the headache phase and last for days or weeks. Familial hemiplegic migraine has an autosomal dominant mode of inheritance with variable penetrance. It has been linked to chromosome 19 in many but not all families. Attacks of migraine lasting longer than 72 hours define status migrainosus. It is often associated with pernicious nausea, vomiting, dehydration, and despair. y

Periodic focal neurological dysfunction, which may be part of the migraine aura, can occur in isolation without the headache. y , y These phenomena can be part of migraine, but they are accepted as migraine only after a full investigation and prolonged follow-up. Occasional headaches occurring in association with the same symptoms as an aura help confirm the migraine diagnosis for the episodes without headache, because patients who have migraine with aura often experience aura without headache.

Late-life migrainous accompaniments are transient neurological

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_TABLE 53-4 -- MAIN CRITERIA FOR THE DIAGNOSIS OF LATE-LIFE MIGRAINOUS ACCOMPANIMENTS

Scintillations (or other visual display), parenthesias, aphasia, dysarthria, and paralysis Build-up of scintillations March of paresthesias

Progression from one accompaniment to another, often with a delay

Two or more similar attacks

Headache in 50 percent of attacks

Episodes last 15 to 25 minutes

Characteristic mid-life "flurry" of attacks

Generally benign course

Normal angiography

Rule out cerebral thrombosis, embolism and dissection, epilepsy, thrombocythemia, polycythemia, and thrombotic thrombocytopenia phenomena that frequently are not associated with headache. [22] Attacks of episodic focal neurological dysfunction, which last from 1 minute to 72 hours, can occur with variable recurrence. Fisher considered scintillating scotoma to be diagnostic of migraine, even when it occurred in isolation, whereas other episodic neurological symptoms (paresthesias, aphasia, and sensory and motor symptoms) needed more careful evaluation [TabeSS-l) . Transient migrainous accompaniments may occur for the first time after the age of 45 and can easily be confused with transient ischemic attacks of cerebrovascular origin.

Ophthalmoplegic migraine^} is associated with acute attacks of third nerve palsy associated with a dilated pupil and migrainous unilateral eye pain. The fourth and sixth cranial nerves may be involved, but this situation is rare. The duration of ophthalmoplegia varies from hours to months. The differential diagnosis includes intracranial aneurysms and chronic sinusitis with a mucocele. However, many cases of ophthalmoplegic migraine fit the criteria for the Tolosa-Hunt syndrome of painful ophthalmoplegia^ : (1) steady, gnawing, boring, eye pain; (2) involvement of nerves of the cavernous sinus; (3) symptoms lasting days or weeks; (4) spontaneous remission, with recurrent attacks occurring after months or years; (5) computed tomography (CT) or magnetic resonance imaging (MRI) limiting disorder to the cavernous sinus; and (6) steroid responsiveness.

Various pain patterns have been described in migraine. Patients who have daily headaches that persist for months often have transformed migraine, with a past history of episodic migraine typically beginning in their teens or twenties. y Most of these patients are women, 90 percent of whom have a history of migraine without aura. The headaches grow more frequent, and the associated symptoms of photophobia, phonophobia, and nausea become less severe and less frequent than during typical migraine. Patients often develop a pattern of daily, or nearly daily, headaches that phenomenologically resemble chronic TTH, with mild to moderate pain but with photophobia, phonophobia, or gastrointestinal features. Other features of migraine, including unilaterality and aggravation by menstruation and other trigger factors, may persist. Attacks of full-blown migraine superimposed on a background of less severe headaches often occur. In the past, this was called mixed tension-vascular headache. Most patients with transformed migraine overuse symptomatic medication. Stopping the overused medication frequently results in distinct headache improvement. Eighty percent of patients with transformed migraine have depression. The depression often lifts when the pattern of medication overuse and daily headache is interrupted.

Because of the limitations of patient recall, a past history of migraine and an escalation of symptoms over 3 months may be impossible to obtain. Three alternative diagnostic links to migraine have been proposed: (1) a prior history of IHS migraine; (2) a clear period of escalating headache frequency with decreasing severity of migrainous features; or (3) current superimposed attacks of headaches that meet all of the IHS criteria for migraine except duration.

There are a number of disorders that may present with migraine symptoms. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited arterial disease of the brain that has been mapped to chromosome 19. y The complete CADASIL syndrome consists of recurrent episodes of focal brain deficits (recurrent strokes) starting in midadult life, often leading to dementia, residual motor disability, and pseudobulbar palsy. Even before clinical symptoms or signs have developed, at-risk individuals may demonstrate an abnormal MRI, with extensive areas of increased T2 signals in the white matter. Many cases of familial hemiplegic migraine (all with associated cerebellar features) map to chromosome 19, close to the gene locus for CADASIL. The main clinical presentation of CADASIL is recurrent subcortical events, either transient or (more often) permanent. However, the vascular presentation is not constant, and other symptoms, such as dementia, migraine with aura, and depression, can occur.

Mitochondrial encephalopathy with lactic acidosis and strokelike spells (MELAS) syndrome may present with otherwise typical migraine or migraine with hemiparesis. Ornithine transcarbamylase deficiency, a urea cycle enzyme abnormality, may resemble confusional migraine in children and young adults, with migraine-like headaches, cyclic vomiting, confusion, hallucinations, visual disturbances, and ataxia.

Differential Diagnosis. Migraine-like headaches with or without aura may occur as a result of a wide variety of structural abnormalities of the brain, including tumors, infections, and vascular malformations. Such headaches may be thought of as secondary migraine. Cerebrovascular disorders such as infarction, transient ischemic attack, venous thrombosis, vasculitis, and carotid or vertebral dissection should be included in the differentiation of migraine. Idiopathic intracranial hypertension (IIH), low pressure headache, and intracranial neoplasms may also mimic migraine, as may metabolic abnormalities, hypoxia, hypoglycemia, dialysis, pheochromocytoma, and various chemicals and medications. Raeder's syndrome, with carotid artery abnormalities along the base of the skull, may also resemble migraine. Sinusitis or glaucoma may occasionally resemble migraine, and sometimes epilepsy produces intermittent headaches. Although rare, the aura of migraine may lead directly to a partial or complex partial seizure, termed migralepsy. '2a' Other primary headache disorders such as TTH, cluster, and hypnic headache should be considered (see Table. .53-1) .y

Evaluation. Patients who have normal neurological examinations

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and benign recurrent headaches that fit the IHS criteria of migraine do not require brain imaging. y Patients who have abnormal neurological examinations, atypical histories, or a sudden unexplained change in the frequency or major characteristics of their headaches should be imaged. All patients with chronic daily headaches should be imaged with CT or MRI, even if they fulfill the criteria for typical migraine. Unless a metabolic abnormality is suspected, blood tests for diagnostic purposes are rarely indicated. However, routine blood tests and an electrocardiogram should be obtained before initiating therapy for migraine. Despite ten million physician visits each year, migraine is still underdiagnosed and undertreated. y

Management. Most migraineurs describe headache- provoking (trigger) factors (Ta.b]e,.5.3-5). , with alcohol, stress, menstruation, and diet being the most common. Patients should attempt to avoid these triggers and keep regular exercise, meal, and sleep patterns. y In the pharmacological treatment of migraine, there are two strategies: (1) abortive medications (Table,,53-6). , which are used to terminate attacks, and (2) prophylactic medications (Table,,53-7). , which are used to prevent future ones. Some rapid- acting drugs, such as metoclopramide, can prevent a headache attack when they are administered during the prodrome. Drugs used for abortive treatment include analgesics (aspirin, acetaminophen), nonsteroidal anti-inflammatory drugs (NSAIDs), serotonin agonists, ergot alkaloids, neuroleptics, steroids, and narcotics. The sooner treatment is begun, the more effective it will be. Caffeine, butalbital, isometheptene, and dichloralphenazone are adjunctive medications included in combination preparations to improve efficacy. y

Analgesics and NSAIDs are first-line therapy in mild to moderate headache attacks. If patients are prone to stomach ulcers, acetaminophen should be used. If these drugs fail, combination analgesics can be used. More severe attacks should be treated with ergotamine, DHE, or sumatriptan. Ergotamine tartrate use should be limited to twice weekly, because of the risk of rebound headaches caused by more frequent dosing. Sumatriptan is effective but expensive, and up to 40 percent of patients have recurrent headaches with this drug. Some clinicians believe that patient-administered sumatriptan and DHE have the best efficacy-to-adverse-effect ratio of all the acute-treatment medications and are the most cost effective because their use results in fewer emergency visits. Because nausea and vomiting are symptoms that are commonly associated with

TABLE 53-5 -- TRIGGER FACTORS OF MIGRAINE

Psychological

Physical

Stress

Exercise

Tension

Fatigue

Anxiety

Sexual activity

Letdown

High altitude

Neurological and Medical

Dietary

Bright lights or glare

Missed or delayed meals

Odors

Certain foods

Changes in sleep patterns

Alcohol

Hormonal changes (menstruation)

Changes in weather or temperature

TABLE 53-6 -- ABORTIVE PHARMACOTHERAPY FOR VARIOUS HEADACHE TYPES

Agent

Starting Dose

Maximum Daily Dose

Use

Ergot Alkaloids

Dihydroergotamine

0.25-1 mg IM/IV

2 mg IV

M,C

I mg SC

3 mg SC/IM

Ergotamine

2-3 mg IN

6 mg IN

M,C

1-2 mg PO/SL/PR

6 mg PO/SL/PR

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