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Indian Pediatr 2013;50: 1063 |
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Childhood Moyamoya Disease: A Clinical and
Angiographic Study from Eastern India
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Anuj Gupta, Deep Das, Biman Kanti Roy and Goutam
Ganguly
Department of Neurology Bangur Institute of
Neurosciences, Kolkata 700 025, India.
Email: [email protected]
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Moyamoya disease (MMD) is a rare cerebrovascular disease of childhood
with majority of cases from Japan. This is a case series of 14 children
diagnosed on the basis of characteristic angiographic findings. Various
clinical features and chief angiographic findings were analyzed.
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Moya moya disease is a rare cerebrovascular disease of
unknown etiology. We herein present a case series of 14
children (7 males and 7 females, mean age: 6.89; age range:
2-14 years) identified among 241 diagnostic cerebral
angiographies performed over a period of two years. Majority
(n=11) presented with ischemic stroke; and only one
with hemorrhagic stroke. Ten patients had recurrent TIA;
weakness of 1 or more limbs was seen in 7; headache in 6 and
seizures in 4 patients. Cerebral ischemic symptoms including
cognitive defect, speech and sensory disturbances were
present in 2, 3, and 3 children, respectively. The only
child who presented with hemorrhagic stroke had thalessemia
major, and the history of antecedent head trauma was present
in one child. None had positive family history of the
disorder. Ancillary laboratory tests including blood
glucose, serum electrolytes, complete hemogram with
peripheral blood smear for sickle cells, and serum lactate
levels were normal in all study subjects. The patient with
thalessemia major showed a hypercoagulable state.
Cerebral angiographies showed obstruction
or stenosis of the supraclinoid portion of the ICA and the
proximal portions of anterior and middle cerebral artery
with a typical fine network of vessels at the base of brain
with hazy, puff of-smoke appearance, and development of
transdural and leptomeningeal anastomoses in all children.
Bilateral abnormalities were present in majority (11/14).
Two angiographies showed stenosis in posterior circulation
also.
Hemorrhagic stroke in pediatric Moxamoya
disease is reportedly uncommon. In the present case, it is
believed to be as a result of hypertension which occurs due
to vasopressive substances provided by multiple transfusions
[1]. Apart from stroke, headache was another consistent
feature in our series which is presumed to be closely
related to cerebral hypoperfusion [2].
Cognitive decline as initial
manifestation signifies the need to consider Moyamoya
disease in children presenting with such symptoms in absence
of typical findings. The unilateral disease can progress to
bilateral disease [3] thus requiring long term follow-up
later. Involvement of posterior circulation indicating rapid
progression of disease, is in accordance with other recent
studies from India [4,5].
References
1. Wasi P, Na Nakorn S, Pootrakul P,
Sonakul D, Piankijagum A, Pacharee P. A syndrome of
hypertension, convulsion, and cerebral haemorrhage in
thalassaemic patients after multiple blood-transfusions.
Lancet. 1978;2: 602-604.
2. Matsushima Y, Aoyagi M, Niimi Y,
Masaoka H, Ohno K. Symptoms and their pattern of progression
in childhood moyamoya disease. Brain Dev. 1990;12:784-789.
3. Kelly ME, Bell-Stephens TE, Marks MP,
Do HM, Steinberg GK. Progression of unilateral moyamoya
disease: A clinical series. Cerebrovasc Dis. 2006;22:109-15.
4. Singhi P, Choudhary A, Khandelwal N.
Pediatric moyamoya disease: clinical profile, literature
review and sixteen year experience from a tertiary care
teaching insti-tute. Indian J Pediatr. 2013 Mar 24. [Epub
ahead of print]
5. Chinchure SD, Pendharkar HS, Gupta AK, Bodhey N,
Harsha KJ. Adult onset moyamoya disease: Institutional
experience. Neurol India. 2011;59:733–8.
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