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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
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.
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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.
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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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