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Orbitotemporal Unilateral
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Nightly or daily for several weeks to months Recurrence after many months or years
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Alcohol in some
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Scintillating lights, visual loss, and scotomas Unilateral paresthesias, weakness, dysphasia, vertigo, rarely confusion Lacrimation Stuffed nostril Rhinorrhea Injected conjunctivum Ptosis
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Ergotamine before anticipated attack, O2, sumatriptan Methysergide, corticosteroids, verapamil, valproate, and lithium in recalcitrant cases
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Tension headaches Mainly adults, both sexes, more in women
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Generalized
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Pressure (nonthrobbing), tightness, aching Intense, steady deep pain, may be worse in neck Variable intensity May awaken patient Steady pain Lasts minutes to hours; worse in early AM, increasing severity Persists for weeks to months None Once in a lifetime: weeks to months None Sometimes position Single episode None Neck stiff on forward bending Kernig and Brudzinski signs
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Meningeal irritation (meningitis, subarachnoid hemorrhage) Brain tumor Any age, both sexes
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Generalized, or bioccipital or bifrontal
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Any age, both sexes
Continuous, variable intensity, for days, weeks, or months Rapid evolution minutes to hours
One or more periods of months to years
Fatigue and nervous strain Depression, worry, anxiety
Antianxiety and antidepressant drugs
For meningitis or bleeding (see text)
Unilateral or generalized
Corticosteroids Mannitol Treatment of tumor
Temporal arteritis Over 50 years, either sex
Unilateral or bilateral, usually temporal
Intermittent, then Throbbing, then continuous persistent aching and burning, arteries thickened and tender
Papilledema Vomiting Impaired mentation Seizures Focal signs Loss of vision Polymyalgia rheumatica Fever, weight loss, increased sedimentation rate
Corticosteroids
PART 2
CARDINAL MANIFESTATIONS OF NEUROLOGIC DISEASE
polygenic Certain rare forms of migraine, such as familial hemiplegic migraine, appear to be monogenic disorders, but the role of these genes, one of which codes for a calcium channel (see below), in classic and common migraine is speculative Migraine, with or without aura, is a remarkably common condition; its prevalence among Caucasians is in the range of 4 to 6 percent among men and 13 to 18 percent among women (see Stewart et al) The reported numbers are lower among Asians Migraine may have its onset in childhood but usually begins in adolescence; in more than 80 percent of patients, the onset is before 30 years of age, and the physician should be cautious in attributing headaches that appear for the rst time after this age to migraine In women, the headaches tend to occur during the premenstrual period; in about 15 percent of such migraineurs, the attacks are exclusively perimenstrual ( true menstrual migraine ), although estrogen and progesterone levels throughout the menstrual cycle are the same in normal and migrainous women Menstrual migraine, discussed further on, is thought to be related to the withdrawal of estradiol rather than progesterone (based on the work of Somerville) It is now acknowledged that the in uence of sex hormones on headache is more complex The attacks cease during pregnancy in 75 to 80 percent of women, and in others they continue at a reduced frequency; less often, attacks of migraine or the associated neurologic symptoms rst appear during pregnancy, usually in the rst trimester Although migraine usually diminishes in severity and frequency with age, it may actually worsen in some postmenopausal women, and estrogen therapy may either increase or, paradoxically, diminish the incidence of headaches The use of birth control pills has been associated with an increased frequency and severity of migraine and in rare instances has resulted in a permanent neurologic de cit Some patients link their attacks to certain dietary items particularly chocolate, cheese, fatty foods, oranges, tomatoes, and onions but these connections in most cases seem to us to be overrated Some of these foods are rich in tyramine, which has been incriminated as a provocative factor in migraine Alcohol, particularly red wine or port, regularly provokes an attack in some persons; in others, headaches are consistently induced by exposure to glare or other strong sensory stimuli, sudden jarring of the head ( footballer s migraine ), or by rapid changes in barometric pressure Perhaps the most common ostensible trigger is excess caffeine intake or withdrawal of caffeine Migraine with aura frequently has its onset soon after awakening, but it may occur at any time of day During the preceding day or so, there may have been mild changes in mood (sometimes a surge of energy or a feeling of well-being), hunger or anorexia, drowsiness, and frequent yawning Then, abruptly, there is a disturbance of vision consisting usually of unformed ashes of white, or silver or, rarely, of multicolored lights (photopsia) This may be followed by an enlarging blind spot with a shimmering edge (scintillating scotoma), or formations of dazzling zigzag lines (arranged like the battlements of a castle, hence the term forti cation spectra or teichopsia) Other patients complain instead of blurred or shimmering or cloudy vision, as though they were looking through thick or smoked glass or the wavy distortions produced by heat rising from asphalt These luminous hallucinations move slowly across the visual eld for several minutes and may leave an island of visual loss in their wake (scotomatous defects); the latter are usually bilateral and often homonymous (involving corresponding parts of the eld of vision of each eye), pointing to their origin in
the visual cortex Patients almost invariably attribute these visual symptoms to one eye rather than to parts of both elds Ophthalmologic abnormalities of retinal and optic nerve vessels have been described in some cases but are not typical (see further on) Other focal neurologic symptoms, much less common than visual ones, include numbness and tingling of the lips, face, and hand (on one or both sides); slight confusion of thinking; weakness of an arm or leg; mild aphasia or dysarthria, dizziness, and uncertainty of gait; or drowsiness Only one or a few of these neurologic phenomena are present in any given patient, and they tend to occur in more or less the same combination in each attack If weakness or paresthetic numbness spreads from one part of the body to another or if one neurologic symptom follows another, this occurs relatively slowly over a period of minutes (not over seconds, as in a seizure, or simultaneously in all affected parts as in a transient ischemic attack) These neurologic symptoms last for 1 to 15 min, sometimes longer; as they begin to recede, they are followed by a unilateral dull pain that progresses to a throbbing headache (usually but not always on the side of the cerebral disturbance), which slowly increases in intensity At its peak, within minutes to an hour, the patient is forced to lie down and to shun light and noise Light is irritating and may be painful to the globes, or it is perceived as overly bright (dazzle), and strong odors are disagreeable Nausea and, less often, vomiting may occur The headache lasts for hours and sometimes for a day or even longer and is always the most unpleasant feature of the illness The temporal scalp vessels may be tender and the headache is worsened by strain or jarring of the body or rapid movement of the head Pressure on the scalp vessels or carotid artery may momentarily reduce the pain Between attacks, the migrainous patient is normal For a time, when psychosomatic medicine was much in vogue, there was insistence on a migrainous personality, characterized by tenseness, rigidity of attitudes and thinking, meticulousness, and perfectionism Further analyses, however, have not established a particular personality type in the migraineur Moreover, the fact that the headaches may begin in early childhood, when the personality is relatively amorphous, would argue against this idea There is no clear relationship, despite many statements to the contrary, between migraine and neurosis Some patients have noted that their attacks of migraine tend to occur during the let-down period, after many days of hard work or nervous tension, but the temporal relations between headache and the day s or week s activities have not proved to be consistent A relationship to epilepsy is also tenuous; however, the incidence of seizures is slightly higher in migrainous patients and their relatives than in the general population Lance and Anthony nd no mechanism common to migraine and epilepsy There does seem to be in migraineurs an overrepresentation of motion sickness and of fainting Migraine Variants Much variation occurs The headache may be exceptionally severe and abrupt in onset ( crash migraine or thunderclap headache ), raising the specter of subarachnoid hemorrhage Careful questioning sometimes reveals that the headache did not attain its peak of severity rapidly but evolved over several minutes or half hour, but often the distinction from subarachnoid hemorrhage can be made only by examination of the CSF and imaging of the brain (see further on, under Special Varieties of Headache ) The headache may at times precede or accompany rather than follow the neurologic abnormalities of migraine with aura Though typically hemicranial (the French word migraine is said to be de-
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