کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
5044531 1475437 2017 12 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Cognitive decline in Huntington's disease expansion gene carriers
ترجمه فارسی عنوان
کاهش شناختی در حاملان ژن های گسترش بیماری هانتینگتون
کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری علم عصب شناسی علوم اعصاب رفتاری
چکیده انگلیسی


- Cognitive decline is mediated by CAG length in HD.
- Stroop Word and Color test are sensitive in tracking cognitive decline in HD.
- Cognitive decline starts early in the course of HD.

BackgroundIn Huntington's Disease (HD) cognitive decline can occur before unequivocal motor signs become apparent. As cognitive decline often starts early in the course of the disease and has a progressive nature over time, cognition can be regarded as a key target for symptomatic treatment. The specific progressive profile of cognitive decline over time is unknown.ObjectiveThe aim of this study is to quantify the progression of cognitive decline across all HD stages, from pre-motormanifest to advanced HD, and to investigate if CAG length mediates cognitive decline.MethodsIn the European REGISTRY study 2669 HD expansion gene carriers underwent annual cognitive assessment. General linear mixed models were used to model the cognitive decline for each cognitive task across all disease stages. Additionally, a model was developed to evaluate the cognitive decline based on CAG length and age rather than disease stage.ResultsThere was significant cognitive decline on all administered tasks throughout pre-motormanifest (close to estimated disease onset) participants and the subsequent motormanifest participants from stage 1 to stage 4. Performance on the Stroop Word and Stroop Color tests additionally declined significantly across the two pre-motormanifest groups: far and close to estimated disease onset.The evaluation of cognition performance in relation to CAG length and age revealed a more rapid cognitive decline in participants with longer CAG length than participants with shorter CAG length over time.ConclusionCognitive performance already shows decline in pre-motormanifest HD gene expansion carriers and gradually worsens to late stage HD. HD gene expansion carriers with certain CAG length have their own cognitive profile, i.e., longer CAG length is associated with more rapid decline.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Cortex - Volume 95, October 2017, Pages 51-62
نویسندگان
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