Annals of Indian Academy of Neurology
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ORIGINAL ARTICLE
Year : 2014  |  Volume : 17  |  Issue : 3  |  Page : 317-320

Varied presentations of moyamoya disease in a tertiary care hospital of north-east India


1 Department of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India
2 Assistant Professor of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India
3 Associate Professor of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India
4 Professor of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India
5 Professor and Head of the Department of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India

Correspondence Address:
Ashok K Kayal
Professor and Head of the Department of Neurology, Gauhati Medical College and Hospital, Bhangagarh, Guwahati - 781 032, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.138518

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Introduction: Moyamoya disease is a chronic progressive cerebrovascular disorder, characterized by stenosis or occlusion of bilateral internal carotid arteries (ICAs), anterior cerebral arteries (ACAs) and middle cerebral arteries (MCAs), accompanied by a collateral network of vessels formed at the base of the brain. Ischemia and intracranial hemorrhage are the common typical manifestations. However moyamoya disease has been associated with atypical presentations like headache, seizures and involuntary movements. Although frequently reported from Asian countries like Japan, China and Korea, only few studies reported on clinical manifestations of moyamoya disease from India. Objectives: To study the varied presentations of moyamoya disease in a tertiary care hospital of north-east India. Material and Methods: Relevant investigations were done to rule out other causes of moyamoya syndrome. Results: We report 6 cases of moyamoya disease with varied presentations from a tertiary care referral government hospital. Case 1, 2 and 6 presented with alternating hemiparesis. Case 3 had amaurosis fugax. Case 4 had history suggestive of ischemic stroke and presented with hemichorea. Case 4 had focal seizure as the only manifestation. Cases 4 and 5 notably had stenosis of posterior cerebral artery (PCA) in addition to stenosis of bilateral ICAs, ACAs and MCAs. Conclusion: Owing to its low incidence in India, moyamoya disease is easily overlooked as a possible diagnosis. However, because of its progressive nature, it is imperative to diagnose this disease early and offer surgical treatment to the patients.


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