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Moyamoya disease with neuro-ophthalmic manifestations: a rare case report

Authors Das D, Handique SK, Bhattacharjee H, Buragohain SK, Bharali G, Deka AC, Singh SK

Published 20 May 2010 Volume 2010:2 Pages 63—65


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Dipankar Das1, Sanjeev Kumar Handique2, Harsha Bhattacharjee1, Sanjoy Kumar Buragohain1, Gayatri Bharali1, Akshay Chandra Deka1, Sunil Kumar Singh1

1Sri Sankaradeva Nethralaya, Guwahati, Assam, India; 2Department of Radiology and Imaging, Guwahati Neurological Research Centre and Hospital, Assam, India

Abstract: Moyamoya (meaning a hazy puff of smoke) disease (MMD) is a rare, idiopathic, persistent, occlusive cerebrovascular disease involving bilateral progressive stenosis or occlusion of a terminal portion of the internal carotid artery, or a proximal portion of the anterior cerebral arteries and the middle cerebral arteries. There are irregular perforating vascular networks (moyamoya vessels), seen in the base of the brain, which produce magnetic resonance images of this ‘puff of smoke’ condition. Although MMD is prevalent mostly in Japan, it is also occasionally seen outside Asia. We report an interesting case of bilateral optic nerve involvement of a radiologically diagnosed case of MMD in a young lady seen in the north-east part of India.
Keywords: magnetic resonance angiography, stroke, optic atrophy

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