Document Detail


Magnetic resonance spectroscopy of the cerebellum in patients with spinocerebellar ataxia type 3/Machado-Joseph disease.
MedLine Citation:
PMID:  21743142     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Objective To evaluate the metabolite pattern and the severity in patients with spinocerebellar ataxia type 3/ Machado-Joseph disease (SCA3/MJD) by (1)H magnetic resonance spectroscopy ((1)H-MRS) on different cerebellar regions, including cerebellar vermis, cerebellar peduncles, cerebellar cortex, and dentatum. Methods Thirty-six SCA3/MJD patients, and 27 sex, age-matched healthy controls were scanned with (1)H-MRS for N-acetylaspartate (NAA), choline (Cho) and creatine (Cr). We made cerebellar vermis, cerebellar peduncles, cerebellar cortex, and dentatum as the region of interests (ROI), and finally got access to NAA/Cr, Cho/Cr, and NAA/Cho ratios. We also examined the CAG repeat numbers of MJD1 gene, scored the 36 patients by the scale for the assessment and rating of ataxia (SARA), analyzed the differences in ratios between SCA3/MJD patients and the control group, and explored whether relevance existed between these ratios and duration of the disease, age of onset, CAG repeat times, and SARA scores respectively. Results The ratio of NAA/Cr in SCA3/MJD patients showed a significant reduction in the cerebellar cortex, dentatum, cerebellar vermis and medipeduncle (P<0.01) compared with the controls. The ratio of NAA/Cho also showed significant reduction in the dentatum and cerebellar vermis (P<0.01). A number of correlations were found between the metabolite ratios of (1)H-MRS and duration of the disease, age of onset, expanded CAG and SARA score in SCA3/MJD patients. Conclusion (1)H-MRS, which shows the neural metabolic changes in the cerebella of SCA3/MJD patients, provides useful information about the severity of SCA3/MJD.
Authors:
Lifang Lei; Yunjie Liao; Weihua Liao; Jie Zhou; Yi Yuan; Junling Wang; Hong Jiang; Lu Shen; Beisha Tang
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences     Volume:  36     ISSN:  1672-7347     ISO Abbreviation:  Zhong Nan Da Xue Xue Bao Yi Xue Ban     Publication Date:  2011 Jun 
Date Detail:
Created Date:  2011-07-11     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101230586     Medline TA:  Zhong Nan Da Xue Xue Bao Yi Xue Ban     Country:  China    
Other Details:
Languages:  eng     Pagination:  511-9     Citation Subset:  IM    
Affiliation:
Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008,China.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:

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


Previous Document:  Serum concentrations of NSE and S100B in spinocerebellar ataxia type 3/Machado-Joseph disease.
Next Document:  Clinical, familial and hereditary analysis of myotonic dystrophy.