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MRI T2 Hypointensities in basal ganglia of premanifest Huntington's disease.
MedLine Citation:
PMID:  20877453     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Increased iron levels have been demonstrated in the basal ganglia of manifest Huntington's disease (HD). An excess in iron accumulation correlates with MRI T2-weighted hypointensity. Determination of the amount of hypointensities in the basal ganglia in the premanifest phase of HD may give more insight in the role of iron in the pathogenesis of HD. Therefore, the present study assessed whether the degree of hypointensities on T2-w MRI in the basal ganglia of premanifest gene carriers differs from non-carriers. Seventeen HD gene carriers without clinical motor signs and 15 non-carriers underwent clinical evaluation and MRI scanning. The amount of T2-w hypointensities was determined using a computer-assisted quantitative method that classified each pixel in the basal ganglia as hypointense or not, resulting in a total of hypointense pixels for each individual. Carriers showed an increased amount of hypointensities in the basal ganglia compared to non-carriers. More hypointensities were furthermore associated with a higher UHDRS total motor score, a longer CAG repeat length and a greater probability of developing symptoms within 5 years. We concluded that the increased amount of hypointensities in the basal ganglia of premanifest carriers of the HD gene may reflect excessive iron deposition and a role for iron in the neuropathology of HD. Furthermore, this phenomenon is associated with clinical and biological disease characteristics. An increased amount of hypointensities on T2-w MRI in the basal ganglia may be considered a biomarker for HD.
Authors:
Caroline K Jurgens; Radu Jasinschi; Ahmet Ekin; Marie-Noëlle W Witjes-Ané; Huub Middelkoop; Jeroen van der Grond; Raymund A C Roos
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Publication Detail:
Type:  Journal Article     Date:  2010-09-08
Journal Detail:
Title:  PLoS currents     Volume:  2     ISSN:  2157-3999     ISO Abbreviation:  PLoS Curr     Publication Date:  2010  
Date Detail:
Created Date:  2010-09-29     Completed Date:  2010-10-19     Revised Date:  2013-02-27    
Medline Journal Info:
Nlm Unique ID:  101515638     Medline TA:  PLoS Curr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  -     Citation Subset:  -    
Affiliation:
Leiden University Medical Center, Leiden; Bronovo Hospital, The Hague, The Netherlands.
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