Moyamoya syndrome related to neurofibromatosis of type 1: a case report

The terms of moyamoya disease or syndrome (in association with another underlying disorder) are used to characterize a rare cerebrovascular condition characterized by progressive stenosis and occlusion of the distal part of internal carotid arteries (ICA) and of its proximal branches associated with...

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Bibliographic Details
Published in:Acta neurologica Belgica 2013-12, Vol.113 (4), p.539-541
Main Authors: Delvoye, François, Hervé, Dominique, Chabriat, Hugues, Mawet, Jérôme
Format: Article
Language:English
Subjects:
Angiography, Digital Subtraction
Biomedical and Life Sciences
Biomedicine
Cerebral Angiography
Humans
Infratentorial Neoplasms - complications
Infratentorial Neoplasms - epidemiology
Letter to the Editor
Male
Medicine/Public Health
Middle Aged
Moyamoya Disease - diagnostic imaging
Moyamoya Disease - etiology
Neurofibromatosis 1 - complications
Neurology
Neuroradiology
Neurosciences
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Description
Summary:The terms of moyamoya disease or syndrome (in association with another underlying disorder) are used to characterize a rare cerebrovascular condition characterized by progressive stenosis and occlusion of the distal part of internal carotid arteries (ICA) and of its proximal branches associated with more or less extensive intracranial neo-vascular networks of very thin blood vessels (moyamoya vessels). Moyamoya disease is ~10 times more frequent in Asian than in European populations. The main clinical manifestations include focal transient or permanent neurological deficits, related to ischemic or hemorrhagic lesions. Transient ischemic attacks (TIAs) and ischemic stroke can occur due to hemodynamic disturbances. Intracerebral hemorrhages are mostly observed in adults and are presumably related to the rupture of fragile neovessels developing in response to chronic hypoperfusion.
ISSN:0300-9009
2240-2993
DOI:10.1007/s13760-013-0250-2