Thursday 24 December 2015

Yes---Enjoy the puff of smoke

Q.All are true regarding Moyamoya disease except
a. progressive symmetric occlusion involving the bifurcations of the internal carotid arteries (ICAs) and the proximal anterior and middle cerebral arteries
b. development of an extensive network of enlarged basal, transcortical, and transdural collateral vessels.
c. presence  of the expected flow void within the cavernous and supraclinoid portions of the ICAs
d. The angiographic appearance of the innumerable tiny collateral vessels, termed “puff of smoke” or “moyamoya” in Japanese.
e. Moyamoya disease has a bimodal age presentation, with the first peak occurring in the first decade of life, associated with cerebral infarction
ANS.—c
Moyamoya disease has a bimodal age presentation, with the first peak occurring in the first decade of life, associated with cerebral infarction as progressive carotid occlusion develops. Adult patients most often present in the fourth decade with intracranial hemorrhage arising from the rupture of the delicate network of collateral vessels
Absence of the expected flow void within the cavernous and supraclinoid portions of the ICAs is a consequence of narrowing and ultimately occlusion of these vessels

There is increased incidence of moyamoya changes in patients with Down syndrome.

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