title: Varied presentations of moyamoya disease in a tertiary care hospital of north-east India.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  To study the varied presentations of moyamoya disease in a tertiary care hospital of north-east India.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India.
authors.forename: Papori
authors.initials: P
authors.lastname: Borah
authors.affiliation: Department of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India.
authors.forename: Vikas
authors.initials: V
authors.lastname: Sharma
authors.affiliation: Assistant Professor of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India.
authors.forename: Lakshya Jyoti
authors.initials: LJ
authors.lastname: Basumatary
authors.affiliation: Associate Professor of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India.
authors.forename: Marami
authors.initials: M
authors.lastname: Das
authors.affiliation: Professor of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India.
authors.forename: Munindra
authors.initials: M
authors.lastname: Goswami
authors.affiliation: Professor and Head of the Department of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India.
authors.forename: Ashok K
authors.initials: AK
authors.lastname: Kayal
keywords: Cerebral infarction; moyamoya; transient ischemic attack