Journal of Postgraduate Medicine
 Open access journal indexed with Index Medicus & ISI's SCI  
Users online: 1097  
Home | Subscribe | Feedback | Login 
About Latest Articles Back-Issues Article Submission Resources Sections Etcetera Contact
 
  NAVIGATE Here 
 ::   Next article
 ::   Previous article
 ::   Table of Contents

 RESOURCE Links
 ::   Similar in PUBMED
 ::  Search Pubmed for
 ::  Search in Google Scholar for
 ::Related articles
 ::   Citation Manager
 ::   Access Statistics
 ::   Reader Comments
 ::   Email Alert *
 ::   Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed282    
    Printed5    
    Emailed0    
    PDF Downloaded5    
    Comments [Add]    

Recommend this journal


 

 CASE REPORT
Year : 2018  |  Volume : 64  |  Issue : 4  |  Page : 240-242

Moyamoya syndrome in hemoglobin E-beta thalassemia: A rare presentation and association


1 Department of Pediatrics, Nanavati Super Speciality Hospital, Mumbai, Maharashtra, India
2 Department of Radiology, Nanavati Super Speciality Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Dr. P N Doctor
Department of Pediatrics, Nanavati Super Speciality Hospital, Mumbai, Maharashtra
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpgm.JPGM_468_17

Rights and Permissions

Moyamoya disease is an idiopathic, nonatherosclerotic, noninflammatory, chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis, typically the supraclinoid internal carotid arteries, followed by extensive collateralization, which are prone to thrombosis, aneurysm, and hemorrhage. Secondary moyamoya phenomenon or moyamoya syndrome (MMS) occurs in a wide range of clinical scenarios including prothrombotic states such as sickle cell anemia, but the association with other hemoglobinopathies is less frequently observed. We describe a case of a 25-year-old female with hemoglobin E-beta thalassemia who had a rare presentation of MMS in the form of choreoathetoid movements in the left upper and lower extremities. We describe this association, primarily to emphasize thalassemia as an extremely rare but a potential etiology of MMS. Since MMS is a progressive disease, it is important to diagnose and initiate treatment to prevent worsening of the disease and recurrence of stroke.






[FULL TEXT] [PDF]*


        
Print this article     Email this article

Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
Published by Wolters Kluwer - Medknow