Document Detail


Susac's syndrome: beneficial effects of corticosteroid therapy in a Japanese case.
MedLine Citation:
PMID:  11300147     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Susac's syndrome is a rare disorder characterized by the triad of microangiopathy of the brain and retina with hearing loss. More than 50 affected individuals have been reported worldwide, all Caucasians. We herein identify the first Japanese patient with Susac's syndrome. A 36-year-old man developed recurrent subacute encephalopathy, bi- a lateral sensorineural hearing loss, and retinal arteriolar occlusions, caused by microangiopathy from a year previously. T2-weighted MRI showed multiple high-signal lesions ti predominantly in the periventricular white matter. During the exacerbated phase both high-dose intravenous methyl-prednisolone and oral prednisone therapy produced beneficial effects. He showed definite remission within 2 years from the disease onset.
Authors:
K Tashima; E Uyama; Y Hashimoto; T Yonehara; M Uchino
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Internal medicine (Tokyo, Japan)     Volume:  40     ISSN:  0918-2918     ISO Abbreviation:  Intern. Med.     Publication Date:  2001 Feb 
Date Detail:
Created Date:  2001-04-12     Completed Date:  2001-07-12     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9204241     Medline TA:  Intern Med     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  135-9     Citation Subset:  IM    
Affiliation:
Department of Neurology, Kumamoto University School of Medicine.
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MeSH Terms
Descriptor/Qualifier:
Adult
Anti-Inflammatory Agents / therapeutic use*
Asian Continental Ancestry Group
Asthma / complications
Ataxia / etiology
Brain / pathology
Cognition Disorders / etiology
Confusion / etiology
Diagnosis, Differential
Diltiazem / therapeutic use
Drug Therapy, Combination
Hearing Loss, Bilateral / diagnosis,  drug therapy*
Hearing Loss, Sensorineural / diagnosis,  drug therapy*
Humans
Magnetic Resonance Imaging
Male
Methylprednisolone / therapeutic use*
Multiple Sclerosis / diagnosis
Papilledema / diagnosis,  drug therapy*
Prednisone / therapeutic use*
Reflex, Abnormal
Retinal Artery / pathology
Retinal Artery Occlusion / diagnosis,  drug therapy*
Skin Diseases, Vascular / etiology
Syndrome
Ticlopidine / therapeutic use
Vasculitis, Central Nervous System / diagnosis,  drug therapy*
Chemical
Reg. No./Substance:
0/Anti-Inflammatory Agents; 42399-41-7/Diltiazem; 53-03-2/Prednisone; 55142-85-3/Ticlopidine; 83-43-2/Methylprednisolone

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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