HEADACHE AND OTHER CRANIOFACIAL PAINS
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rived from megrim, which in turn was derived from the Latin hemicrania and its corrupted forms hemigranea and migranea), the pain may be frontal, temporal, or quite often, generalized Milder forms of migraine, especially if partially controlled by medication, may not force the patient to withdraw from accustomed activities Any one of the three principal components neurologic abnormality, headache, and nausea and vomiting may be absent With advancing age, there is a tendency for the headache and nausea to become less severe, nally leaving only the neurologic abnormality ( aura without migraine ), which itself recurs with decreasing frequency This is also subject to variation Visual disturbances, by far the most common, differ in detail from patient to patient; numbness and tingling of the lips and the ngers of one hand are probably next in frequency, followed by transient aphasia or a thickness of speech and hemiparesis as mentioned earlier Rarely, there is sudden but transient blindness or a hemianopia at the onset, accompanied by only a mild headache All of these neurologic syndromes must be distinguished from transient ischemic attacks (TIA) and small strokes, which they resemble if the typical headache fails to follow the neurologic symptoms (see further on, under Stroke and TIA with Migraine ) Basilar Migraine A less common form of the migraine syndrome, with prominent brainstem symptoms, was described by Bickerstaff The patients, usually young women with a family history of migraine, rst develop visual phenomena like those of typical migraine except that they occupy the whole of both visual elds (temporary cortical blindness may occur) There may be associated vertigo, staggering, incoordination of the limbs, dysarthria, and tingling in both hands and feet and sometimes around both sides of the mouth These symptoms last 10 to 30 min and are followed by headache, which is usually occipital Some patients, at the stage when the headache is likely to begin, may faint, and others become confused or stuporous, a state that may persist for several hours or longer Exceptionally there is an alarming period of coma or quadriplegia The symptoms closely resemble those due to ischemia in the territory of the basilar-posterior cerebral arteries hence the name basilar artery or vertebrobasilar migraine Subsequent studies have indicated that basilar migraine, though more common in children and adolescents, affects men and women more or less equally over a wide age range, and that the condition is not always benign and transient (see further on, under Complicated Migraine ) Ophthalmoplegic and Retinal Migraine The former are recurrent unilateral headaches associated with weakness of extraocular muscles A transient third-nerve palsy with ptosis, with or without involvement of the pupil, is the usual picture; rarely, the sixth nerve is affected This disorder is more common in children The paresis often outlasts the headache by days or weeks; after many attacks, a slight mydriasis and, rarely, ophthalmoparesis may remain as permanent defects Retinal and anterior optic nerve ischemia has also been documented In some cases, the retinal arterioles are attenuated and sometimes there are retinal hemorrhages; in other cases, narrowing of retinal arteries or venules has been observed during an attack, as described in the cases reported by Berger and colleagues In still other cases, monocular blindness is associated with disc edema and peripapillary hemorrhages, and vision recovers only partially after several months (Hupp et al) Such events are referred to as retinal migraine, or, more accurately, ocular migraine, since either the retinal or the ciliary circulation may be involved It is well to remember that in adults the syndrome of headache, unilateral
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ophthalmoparesis, and loss of vision may have more serious causes, including temporal (cranial) arteritis Migraine following Head Injury A particularly troublesome migraine variant occurs in a child or adolescent who, after a trivial head injury, may lose sight, suffer severe headache, or be plunged into a state of confusion, with belligerent and irrational behavior that lasts for hours or several days before clearing In yet another variant there is an abrupt onset of either one-sided paralysis or aphasia after virtually every minor head injury (we have seen this condition several times in college athletes) but without visual symptoms and little or no headache Although a family history of migraine is frequent in such cases, there has been no hemiplegia in other family members Migraine in Young Children This may present particular dif culties in diagnosis, since a young child s capacity for accurate description is limited Instead of complaining of headache, the child appears limp and pale and complains of abdominal pain; vomiting is more frequent than in the adult and there may be slight fever Recurrent attacks are referred to by pediatricians as the periodic syndrome Another variant in the child is episodic vertigo and staggering (paroxysmal disequilibrium) followed by headache (see Watson and Steele) Also, there are the puzzling patients with bouts of fever or transient disturbances in mood (psychic equivalents) and abdominal pain (abdominal migraine), dubious entities at best We have also seen several infants and young children who have had attacks of hemiplegia (without headache), rst on one side then the other, every few weeks Recovery was complete, and arteriography in one child, after more than 70 attacks, was normal The relationship of this condition to neurologic migraine remains uncertain The only advantage of considering such attacks as migrainous is that it may protect some patients from unnecessary diagnostic procedures and surgical intervention; but, by the same token, it may delay appropriate investigation and treatment Hemiplegic Migraine In a related disorder, known as hemiplegic migraine, an infant, child, or adult has episodes of unilateral paralysis that may long outlast the headache Several families have been described in which this condition was inherited as an autosomal dominant trait ( familial hemiplegic migraine) Linkageanalysis studies of this disorder have localized the responsible gene to chromosome 19 in one-third of families (Joutel et al); in other families, the gene has localized to chromosome 1; in yet others, no linkage has been found The gene on chromosome 19 codes for a voltage-gated calcium channel protein, which raises the provocative possibility that other forms of migraine are also due to an ion channel disorder Instances of hemiplegic migraine may account for some of the inexplicable strokes in young women and older adults of both sexes The situation is, however, more complex; Ducros and colleagues have found a variety of other neurologic features in these families, including peristent cerebellar ataxia and nystagmus in 20 percent; others had attacks of coma and hemiplegia, from which they recovered Complicating the situation is the undoubted existence of sporadic migraine with transient hemiplegia that has no familial trait Neurologic symptoms lasting more than an hour or so should prompt investigation for alternative causes of headache, but none may be found Stroke and Transient Ischemic Attacks with Migraine (See also Chap 34) Attacks of migraine, instead of beginning in childhood and recurring in stereotyped fashion every few weeks or months with diminishing frequency in middle and late adult years, may have their onset later in life or may suddenly increase in fre-
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