Document Detail


A Japanese case of Fragile-X-associated tremor/ataxia syndrome (FXTAS).
MedLine Citation:
PMID:  20558944     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
A 71-year-old man developed postural tremor and was treated as an essential tremor patient. Nine years after the tremor onset, he developed symptoms resembling Fragile-X-associated tremor/ataxia syndrome (FXTAS), including exacerbated (increased coarseness and amplitude) tremor in the right arm, ataxic gait, and brain MRI showed lesions in the bilateral middle cerebellar peduncles (MCP). Evidence of premutation in the form of 83 CGG repeats of the Fragile-X-mental retardation 1 (FMR1) gene confirmed the diagnosis of FXTAS. FXTAS causes various neurological symptoms including in some cases tremor resembling essential tremor in the early stages. FMR1 gene premutation should be checked when the patient develops intention tremor, cerebral dysfunction and/or a brain MRI shows MCP lesions.
Authors:
Kazuhiro Ishii; Ai Hosaka; Kaori Adachi; Eiji Nanba; Akira Tamaoka
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-06-15
Journal Detail:
Title:  Internal medicine (Tokyo, Japan)     Volume:  49     ISSN:  1349-7235     ISO Abbreviation:  Intern. Med.     Publication Date:  2010  
Date Detail:
Created Date:  2010-06-18     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9204241     Medline TA:  Intern Med     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  1205-8     Citation Subset:  IM    
Affiliation:
Department of Neurology, Institute of Clinical Medicine, Majors of Medical Sciences, Graduate School of Comprehensive Human Sciences University of Tsukuba, Tsukuba, Japan. kazishii@md.tsukuba.ac.jp
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