470

24.

Salanova V, Andermann F, Rasmussen T, et al: Parietal lobe epilepsy.
Clinical manifestations and outcome in 82 patients treated surgically
between 1929 and 1988. Brain 1995;118:607-627.

25.

Cascino GD: Advances in neuroimaging: Surgical localization. Epilepsia 2001;42:3-12.

26.

Chadwick D: Case for early treatment is not established. BMJ 1995;310:
177-178.

27.

Reynolds EH: Do anticonvulsants alter the natural course of epilepsy?
BMJ 1995;310:176-177.

28.

Shinnar S, Berg AT: Does antiepileptic drug therapy prevent the development of "chronic" epilepsy? Epilepsia 1996;37:701-708.

29.

Sillanpaa M, Jalava M, Kaleva O, Shinnar S: Long-term prognosis of
seizures with onset in childhood. N Engl J Med 1998;338:1715-1722.
Duchowny M: Recent advances in candidate selection for pediatric
epilepsy surgery. Semin Pediatr Neurol 2000;7:178-186.

30.

chemistry, hematologic tests (including proteins C and S, antithrombin III,
lupus anticoagulant, anticardiolipin antibodies, syphilis testing [RPR], factor V Leiden), toxicology, and cerebrospinal fluid analysis were normal;
genetic testing for mitochondrial encephalomyopathy, lactic acidosis, and
strokelike episodes (MELAS) syndrome was negative. Electroencephalography (EEG) showed right hemispheric slowing, which normalized after clinical resolution. Magnetic resonance imaging (MRI) of the brain during the
coma showed right hemispheric edema on T.,-weighted sequences; on timeof-flight magnetic resonance angiography, the diameter of the right middle
cerebral artery was severely reduced as compared with the other side (Figure 1). A repeat MRI/magnetic resonance angiography study 2 weeks after
the ep-isudi-- ~huBVèd lharkedly dinlinished edenla and iivii~ia.i u1cu11c~cu of
the right middle cerebral artery (see Figure 1). There was no evidence of
or cerebellar atrophy or of white-matter
cerebral cortical or
white-matter lesions.~*
lesions. 9
Discussion

,

...

In the case presented, the migraine attack resulted in transient left

Magnetic Resonance Angiogram Evidence of
Vasospasm in Familial Hemiplegic Migraine
ABSTRACT
We report a case of familial hemiplegic migraine in an 11-year-old
girl who presented with coma and left hemiparesis. Magnetic resonance imaging showed reversible vasospasm and evidence of
oligemia, bringing new information regarding neurovascular
changes in familial hemiplegic migraine. (J Child Neurol 2002;17:
470-472).

hemiparesis and coma, with MRI evidence of right hemispheric
edema. Magnetic resonance angiography showed evidence of
reversible narrowing of the right middle cerebral artery distribution, suggesting vasospasm in that territory. The EEG provided additional electrophysiologic evidence of transient regional cortical
involvement. These findings are compatible with transient ischemia.
To our knowledge, this is the first reported magnetic resonance
angiogram evidence of vascular spasm in familial hemiplegic
migraine.
However, an alternate explanation could be oligemia, which
is thought to occur during-and spread similarly to-the cortical
spreading depression, the physiologic correlate of the aura.’° Interestingly, the edema on MRI seemed to spread even beyond the
boundaries of the middle cerebral artery.

Familial hemiplegic migraine is characterized by recurrent episodes

of focal neurologic deficits accompanied by migraine headache and
is genetically linked to chromosome 19 in about 50% of cases.’-3 It
can be associated with ataxia.’ The

pathophysiology of this disease

remains largely unknown. Conventional angiography in patients
with familial hemiplegic migraine and migraine with or without aura
has shown reversible stenosis consistent with vascular spasm. 5,6

However, the procedure itself may induce vasospasm, putting in
question the significance of this finding. Meaney et all reported hemispheric edema and transient carotid artery vasodilatation by magnetic resonance angiogram in a patient with familial hemiplegic
migraine during an attack. Reversible reduction of water diffusion
suggestive of hemispheric cytotoxic edema has also been reported
during prolonged attacks of hemiplegic migraine.’ We present an
11-year-old girl with familial hemiplegic migraine and magnetic resonance angiogram evidence of cerebral arterial vasospasm during
a migraine episode.

The coexistence of edema and large artery stenosis likely
represents hypoperfusion. Because the magnetic resonance angiog-

raphy study was done several hours after the onset of the aura symptoms, one cannot postulate whether the narrowed arterial diameter
is a primary or secondary phenomenon to the cortical dysfunction. 11l
The apparent discrepancy between the large artery vasodilation
reported by Meaney et al and our observation of vasoconstriction
could be explained by a potential time difference from onset of aura
to imaging and could also relate to the few observed cases of ini-

tial hyperperfusion. 12, 13 However, although it is tempting to try to
establish a relation between the diameter of the artery and the

underlying blood flow, the link is indirect at best.’4
Further studies need to confirm the significance of the current fmdings. In addition, similar observations should be attempted
in migraine without aura, where oligemia may also occur. 15
Calin I. Prodan, MD
Neil R. Holland, MBBS
Marc E. Lenaerts, MD

Case Report

Julie T. Parke, MD
Department of Neurology
University of Oklahoma

An 11-year-old girl was admitted with loss of consciousness following a right-

sided headache. She had left extensor plantar response and hyporeflexia
and remained comatose for 5 hours. On awakening, she was still complaining of severe throbbing headache and had a left hemiparesis and right
gaze preference for another 12 hours. No cerebellar signs were noted.
Within 24 hours, her neurologic examination had completely normalized.
Her past medical history was remarkable for migraine headaches since age
6 years, and she had one similar episode of headache with a 6-hour-long,
fully reversible, left hemiparesis. There was a family history of hemiplegic
migraine in five paternal relatives, without dementia or ataxia. Serum bio-

Oklahoma City, Oklahoma
Received March 18, 2002. Accepted for publication April 4, 2002.
Address correspondence to Dr Marc E. Lenaerts, 711 S.L. Young Blvd, PPOB,
Suite 215, Oklahoma City, OK 73104. Tel: 405-271-4113; fax: 405-271-5723;
e-mail: marc-lenaerts@ouhsc.edu.

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471

T 2-weighted magnetic resonance images (MRIS) during
(A) and after (B) the migraine episode. Magnetic resonance angiogram
images during (C) and after (D) the migraine episode. Note the right
middle cerebral artery narrowing during the episode and marked resFigure 1.

olution afterward, as well as the transient right hemispheric edema
during the migraine episode.

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472

References
1.

Clarke JM: On recurrent motor paralysis in migraine. BMJ 1910;1:
1534-1538.

2.

Joutel A, Bousser MG, Biousse V, et al: A gene for familial hemiplegic
migraine maps to chromosome 19. Nat Genet 1993;5:40-545.

3.

Joutel A, Tournier-Lasserve E, Bousser MG: Hemiplegic migraine.
Presse Med 1995;24:411-414.

4.

Ophoff RA, Terwindt GM, Vergouwe MN, et al: Familial hemiplegic
2+
migraine and episodic ataxia type-2 are caused by mutations in the Ca
channel gene CACNLIA4. Cell 1996;1:543-552.
Jensen IW: Unusual angiographic appearance during attack of hemiplegic migraine.
e 1986;26:295 296.
Headach
Solomon S, Lipton RB, Harris PY: Arterial stenosis in migraine: Spasm
or arteriopathy? Headache 1990;30:52-61.
Meaney JF, Williams CE, Humphrey PR: Case report: Transient unilateral cerebral oedema in hemiplegic migraine: MR imaging and
angiography. 1996;51:72-76
Clin Radiol
.
Chabriat H, Vahedi K, Clark CA, et al: Decreased hemispheric water
mobility in hemiplegic migraine related to mutation of CACNA1A
gene. Neurology 2000;54:510-512.

5.

6.
7.

8.

9.

Igarashi H, Sakai F, Kan S, et al: Magnetic resonance imaging of the
brain in patients with migraine. Cephalalgia 1991;11:69-74.

10.

Lauritzen M, Skyhoj Olsen T, Lassen NA, Paulson OB: The changes of
regional cerebral blood flow during the course of classical migraine
attacks. Ann Neurol 1983;13:633-641.

11.

Lauritzen M: Cerebral blood flow in migraine and cortical spreading
depression. Acta Neurol Scand 1987;76:1-40.

12.

Olesen O, Larsen B, Lauritzen M: Focal hyperemia followed by spreading oligemia and impaired activation of rCBF in classic migraine.

13-

Olesen J, Friberg L, Olesen TS, et al: Timing and topography of cerebral blood flow, aura and headache during migraine attacks. Ann
Neurol 1990;28:791-798.

14.

Olesen J, Diener H-C: Hemodynamics and neuroimaging of migraine,
in Olesen J, Welch KMA, Tfelt-Hansen P (eds): The Headaches. Philadelphia, Lippincott, Williams & Wilkins, 2000, 290-291.

15.

Woods RP, Iacobini M, Mazziotta JC: Bilateral spreading cerebral
hypoperfusion during spontaneous migraine headache. N Engl J Med

Cephalalgia 1981;9:344-352.

1994;331:1689-1692.

Downloaded from jcn.sagepub.com at The University of Hong Kong Libraries on May 9, 2015