Stroke, Vol 15, 728-732, Copyright © 1984 by American Heart Association
S Takebayashi, K Matsuo and M Kaneko
Moyamoya disease was originally defined as a characteristic syndrome of
recurrent headaches, occlusion of the distal internal carotid arteries and
the foggy (moyamoya) clusters of collateral vessels at the base of the
brain as demonstrated by cerebral angiography. The etiology is unknown and
pathobiology is poorly understood. We examined the intracranial arteries in
3 patients to demonstrate characteristic changes and to obtain a better
understanding of the basis mechanisms of the disease. Controls were
obtained from 3 normotensive patients who died as a result of cancer.
Occluded internal carotid arteries were characterized by severe thickening
of the intima with a dense luminal array of smooth muscle cells, a deeper
less cellular zone, pronounced tortuosity of the internal elastica and
thinning of the media. Collateral vessels were arterial in structure and
were affected by similar proliferative changes in the intima, thinning of
the media, and contorted internal elastica. Stainable lipids were not part
of the typical components. Severe contortion of the internal elastica,
medial damage and intimal proliferation may result from recurrent and
sustained spasticity of the cerebral arteries. The distal lenticulostriate
arteries showed severe medial damage similar to what is termed as a
moth-eaten change in hypertensive patients dying of massive cerebral
hemorrhage.
ARTICLES
Ultrastructural studies of cerebral arteries and collateral vessels in moyamoya disease
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