Saudi Journal of Anaesthesia

: 2014  |  Volume : 8  |  Issue : 5  |  Page : 123--124

Hemiplegic migraine: An atypical presentation of Moyamoya disease

Chinmoy Roy1, Nilay Chatterjee2,  
1 Department of Anesthesia and Pain Medicine, Institute of Neurosciences, Kolkata, West Bengal, India
2 Department of Neuroanesthesiology and Pain Medicine, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India

Correspondence Address:
Dr. Nilay Chatterjee
NFQ B-10, Sree Chitra Tirunal Institute for Medical Sciences and Technology Faculty Quarters, Kumarapuram, Poonthi Road, Medical College PO, Trivandrum - 695 011, Kerala

How to cite this article:
Roy C, Chatterjee N. Hemiplegic migraine: An atypical presentation of Moyamoya disease.Saudi J Anaesth 2014;8:123-124

How to cite this URL:
Roy C, Chatterjee N. Hemiplegic migraine: An atypical presentation of Moyamoya disease. Saudi J Anaesth [serial online] 2014 [cited 2022 Oct 7 ];8:123-124
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Migraine is a common disorder causing attacks of disabling headaches that can be accompanied by an aura in a third of patients. Few patients having motor weakness during the aura qualify for hemiplegic migraine. [1] This can occur as a sporadic or familial disorder; the latter type is characterized by affection of one first or second degree relative, and then known as familial hemiplegic migraine. [2] Moyamoya disease is a progressive cerebrovascular occlusive disease identified by stenosis of distal internal carotid arteries (ICA) and their branches. Dilation of small cerebral vessels in an attempt to compensate for the decreased blood flow in the anterior circulation produces the characteristic "puff of smoke" appearance on angiography. [3] Headache is one of the common presentations of moyamoya disease in children. [4] Pathophysiological mechanisms for headache remain unclear, but presumed to be closely related to the intracranial oligemic state, as reflected by the disappearance of headache after the improvement of cerebral bold flow augmentation surgery, that is, vascular anastomosis. [5] Familial hemiplegic migraine could be a rare presentation of moyamoya disease. We describe a case of moyamoya disease presented as familial hemiplegic migraine.

An 11-year-old girl presented to the pain clinic with periodic throbbing pain over right forehead associated with complete hemiparesis of left half of the body since last 2 years. Frequency of headache was 1-2 times/month with intermittent symptom free periods up to 3 months. Headache was not related to any particular time of the day and any precipitating, aggravating or relieving factors were absent. Initially, her complaints began with nausea, circumoral paresthesia and paresthesia of the left half of the body lasting for 20-30 min. Initial aura symptoms were followed by pain and hemiparesis for 4-10 h with spontaneous and complete recovery thereafter. She was treated for migraine without any effect. There were no past histories of head injury, seizures, involuntary movements, developmental delay, central nervous system infections, or other significant medical illness. On examination, she was afebrile, with normal vitals, pupils were equal (7 mm) and reactive to light, cranial nerve examination was normal and disc margins were sharp on fundoscopy. Tone, power, and reflexes were within normal ranges; bilaterally in both the extremities. Patient's elder sister (15-year-old) had similar symptoms but never underwent treatment. Magnetic resonance imaging brain showed bilateral periventricular deep white matter ischemia with mild cortical atrophy. Cerebral angiography revealed the occlusion of supra-clinoid segments of ICA bilaterally with evidences of moyamoya vessels [Figure 1]. Pial to pial collateral circulation from bilateral posterior cerebral artery to anterior cerebral artery and middle cerebral artery noted through pericallosal collateral vessels [Figure 1]. Considering moyamoya disease is the primary pathology responsible for the headache patient was referred for neurosurgical management.{Figure 1}

This particular case report and the review of the existing literatures suggest that pediatric patients presenting with migraineous or cluster headache refractory to prophylactic and abortive medical therapies should be evaluated further with cerebral angiography. If moyamoya disease is responsible, an early revascularization procedure will alter the prognosis and life expectancy in such patients.


1Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders: 2 nd edition. Cephalalgia 2004;24 Suppl 1:9-160.
2Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: Pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol 2011;10:457-70.
3Scott RM, Smith ER. Moyamoya disease and moyamoya syndrome. N Engl J Med 2009;360:1226-37.
4Park-Matsumoto YC, Tazawa T, Shimizu J. Migraine with aura-like headache associated with moyamoya disease. Acta Neurol Scand 1999;100:119-21.
5Matsushima Y, Aoyagi M, Niimi Y, Masaoka H, Ohno K. Symptoms and their pattern of progression in childhood moyamoya disease. Brain Dev 1990;12:784-9.