In the new classification of migraine,1 Subtype 1.6 indicates complications of migraine.  This would include all of the permanent defects discussed in this section.

Focal symptoms and signs of the aura may persist beyond a headache phase.  In the previous classification, this was termed complicated migraine.  It is now defined by the IHS classification with two labels with increased specificity.  If the aura lasts for longer than one hour but less than one week, the term migraine with prolonged aura is applied.  If the signs persist for more than one week or a neuroimaging procedure demonstrates a stroke, a migrainous infarction has occurred.  As pointed out previously, mid or later life the aura may not be followed by headache and has been termed migraine accompagnée or migraine associée.  Migraine with aura (classic) in early reports was sometimes referred to as “ophthalmic migraine” (to be differentiated from ophthalmoplegic migraine, a subtype of migraine with aura).  Migraine with aura is further reviewed under the headings: Cerebral, Ophthalmoplegic, Retinal, Basilar, and Other Varieties

 Cerebral

A variety of cerebral symptoms may occur in migraine with aura, including motor, visual, and other sensory defects.  As pointed out previously, if the aura lasts for more than one hour , but less than one week, the term migraine with prolonged aura is applied.  However, if the signs persist for more than one week, or a neuroimaging procedure shows a stroke the term used is migrainous infarction.  The HIS classification of migraine-related stroke is presented in Table 4.  Welch23 has classified migraine-related stroke into four subtypes. 

1.5.4             Migrainous infarction

        

                 Description

One or more migrainous symptoms associated with an  ischaemic brain lesion in appropriate territory demonstrated by neuroimaging.

                  Diagnostic criteria

A.         The present attack in a patient with 1.2 Migraine with aura is typical of previous attacks except that one or more aura symptoms persists for >60 minutes

 

B.          Neuroimaging demonstrates ischaemic infarction in a relevant area

 

C.          Not attributed to another disorder

 

                 Comments

 Ischaemic stroke in a migraine suffer may be categorized as cerebral infarction or other cause coexisting with migraine, cerebral infarction of other cause presenting with symptoms resembling migraine with aura, or cerebral infarction occurring during the course of a typical migraine with aura attack.  Only the last fulfills criteria for 1.5.4 Migrainous infarction.

Increased risk for stroke in migraine patients has been demonstrated in women under age 45 in several studies. Evidence for an association between migraine and stroke in older women and in men is inconsistent.

 

These are described briefly. 

I.          Coexisting stroke and migraine.  Definition.  A clearly defined clinical stroke syndrome must occur remotely in time from a typical migraine attack.  Stoke in the young is rare; in contrast, migraine is common.  As pointed out by Welch, the two conditions should coexist without migraine being a contributing risk factor for stroke.

II.         Stroke with clinical features of migraine.  Definition.  A structural lesion that is unrelated to migraine pathogenesis presents with clinical features of a migraine attack.  Subtype A is symptomatic and in these patients, established structural CNS lesions or cerebral vessels cause episodic symptoms typical of migraine with neurologic aura, although infrequently.  Such cases could be termed symptomatic migraine.  Cases of cerebral arteriovenous malformation exemplify this concept and may masquerade as migraine with aura.7,73

 Welch’s subtype B is a migraine mimic.  In this category, stroke caused by acute and progressive structural disease is accompanied by headache and a constellation of progressive neurologic signs and symptoms.  These situations are difficult to distinguish from those of migraine, hence the term migraine mimic.  The diagnosis can be most difficult in patients who continue to have migraine late in life, when the incident of cerebrovascular disease increases.23

III.       Migraine-induced stroke.  Definition.  Migraine-induced stroke must meet the following criteria: (1) neurologic deficit must be identical to the migraine symptoms of previous attacks; (2) the stroke must occur during the course of a typical migraine attack; and (3) all other causes of stroke have been excluded, although stroke risk factors may be present.

IV.       Uncertain classification, Welch has indicated that many migraine-related strokes can not be categorized with certainty.  For example, the IHS definition of migraine-induced stroke does not prevent the diagnosis in patients with migraine without aura.  Migraine-induced stroke associated with treatment of the attack is appropriately classified in category.  In addition, there are occasional cases of migraine-like symptoms and persistent neurologic deficit associated with CSF protein and pleocytosis.74,75  Other rare syndromes and migraine-related stroke include migraine associated with mitochondrial encephalopathies76 and “migraine coma.”77 Intracerebral hemorrhage has been reported,78 with most cases, according to Welch, being migraine mimics.  Whether there is a role of antiphospholipid antibodies is still to be determined.79  Permanent homonymous visual field defects are well documented in migraine patients.67,80-82  The defects almost always occur in patients who have previously had migrainous attacks with transient scintillating scotomas.  Computed cranial tomography (CT) or magnetic resonance imaging (MRI) has now documented a number of cerebral infarctions usually in the occipital and parietal regions.  Rothrock and colleagues83 evaluated 22 patients with migraine‑associated stroke finding that 91 percent were women and 23 percent had a prior history of presumed migrainous stroke.  They concluded that extracranial and intracranial vasospasm played a major role in some cases they were able to document angiographically.  One controlled study of migraine with aura reported that 91% of patients who had stroke during an attack had no arterial lesions.  This was as opposed to 9% of migraine with aura patients who suffered stroke remote from a migraine attack and 18% of patients with stroke without a migraine history.84  In a rigorous case controlled study no overall association between migraine and ischemic stroke was found, but among women younger than 45 years, migraine and stroke were significantly associated; the risk was increased four fold and it became even greater in women who smoked.85

According to Hollenhorst,86 approximately 4% of patients who have a typical sequence of visual aura followed by hemicranial headache experience transient hemianopsia lasting up to 15 minutes.  Much rarer are patients with permanent hemianopia. Bilateral upper quadrantic defects have been reported.87,88   

Other sensory disturbances such as paresthesias particularly involve hands, fingers, and lips.87,88  Various aspects of cerebral migraine with aura are illustrated by the following case history:

A 26‑year‑old woman was seen with a complaint of difficulty with vision.  She had a history of migraine since age 12, characterized by an aura of "black spots" slowly spreading over the field of vision for 20 minutes, occasionally accompanied by numbness in the right hand and arm. Thereafter throbbing headache would occur that was left‑sided 90% of the time.  Her father had a history of classic migraine as a young man.   Ten days prior to first being seen, she had a typical attack of migraine, but with persistent difficulty in vision following the episode. Examination was entirely normal except for a congruous right homonymous visual field defect (Fig 9). Ten days later she again developed her visual aura, but with a moderate right hemiparesis as well, during her headache.  Brain scan and cerebral arteriography were normal.  During the next week all neurologic abnormality including her visual field defect cleared completely. 

Frequently a disturbance of language occurs with migraine, as pointed out by Sir George Airy in 1865, who described his own inability to speak during an attack.

A typical history related to the author by his brother is as follows:

While reading, I become aware that I am unable to understand what I have just read.  After rereading a paragraph two or three times, I begin to realize that I cannot understand the sense of words. The letters can be identified but the words are unintelligible...At this point a numb feeling occurs in my right hand and I finally realize that I am at the start of another migraine attack. 

A wide variety of language difficulties, sensory defects, and motor abnormalities have been described (most often transient but rarely permanent) from presumed cerebral infarction. Caplan et Al90 reported 12 patients with transient global amnesia and prior migraine.  In 3 patients, the classic migrainous phenomenon accompanied the amnestic attack. 

Variable EEG findings occur.89,91,92   There is general lack of agreement as to the incidence and significance of abnormalities in the EEGs of patients with migraine.  Some authors report a normal EEG,93 but various abnormal patterns have been recorded.91,92 A detailed review by Hockaday and Whitty94 indicated an incidence of EEG abnormality in 61% of 560 migraine patients.  The highest frequency of abnormality occurred in patients with transient lateralized motor or sensory auras. 

Friedman95 first reported the results of angiography during an attack of migraine; there were no abnormalities.  While the majority of patients in reported cases show normal arteriograms, others have demonstrated some abnormality during an attack (see Cluster Headache).  However, angiography is considered by some to have increased risk in patients with migraine67,96 and has not yet provided useful information on the pathophysiology of complicated migraine.97-99

Hemiplegic migraine occurs both sporadically and as a familial syndrome.  This entity is defined as "vascular headache" featured by sensory and motor phenomena which persist during and (for a brief time) after the headache.100  A narrower view would be to use the term hemiplegic migraine when only motor involvement (i.e., weakness or paralysis) occurs.

The first mention of transient hemiparesis during an attack of migraine was by Liveing,5 and multiple reviews and case reports have appeared since.101,102  Heyck103 reviewed the neurologic complications of 980 of 3,890 patients with migraine.  The majority of these patients complained of unilateral tingling or numbness which invariably involved the hand and sometimes spread to the arm, face, tongue, and, rarely, to the leg.  The symptoms seldom lasted more than 30 minutes and could occur before or at the peak of the headache. Twelve of these patients had unilateral motor disturbances ranging from minimal loss of function to complete paralysis. There have been few permanent sequelae attributed to hemiplegic migraine; progressive dementia was noted by Symonds, and permanent hemiplegia has  been reported.89,97,103

Reports of hemiplegic migraine in the literature seem to indicate that most cases are familial.  However, Heyck97 pointed out the tendency to report familial cases; if unselected patients with the syndrome are reviewed, most do not occur in families with hemiplegic migraine, but rather in "families with ordinary migraine as often as common or classic migraine." Familial hemiplegic migraine is well documented,101,104,105 at times in kindred with associated neuro‑ophthalmologic findings such as retinal degeneration and nystagmus.106  One interesting report is that of Dooling and Sweeney107, who describe a blind woman whose attacks were precipitated by breast feeding her infant. This led to the speculation that oxytocin (chemically similar to ergotamine) could exercise a complex effect on cerebral vessels predisposed to vasospasm. 

 

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