| Structural changes associated with progression of motor deficits in spinocerebellar ataxia 17. | |
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MedLine Citation:
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PMID: 20016963 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Spinocerebellar ataxia (SCA17) is a rare genetic disorder characterized by a variety of neuropsychiatric symptoms. Recently, using magnetic resonance imaging (MRI) voxel-based morphometry (VBM), several specific functional-structural correlations comprising differential degeneration related to motor and psychiatric symptoms were reported in patients with SCA17. To investigate gray matter volume (GMV) changes over time and its association to clinical neuropsychiatric symptomatology, nine SCA17 mutation carriers and nine matched healthy individuals underwent a detailed neuropsychiatric clinical examination and a high-resolution T1-weighted volume MRI scan, both at baseline and follow-up after 18 months. Follow-up images revealed a progressive GMV reduction in specific degeneration patterns. In contrast to healthy controls, SCA17 patients showed a greater atrophy not only in cerebellar regions but also in cortical structures such as the limbic system (parahippocampus, cingulate) and parietal precuneus. Clinically, progression of motor symptoms was more pronounced than that of psychiatric symptoms. Correlation with the clinical motor scores revealed a progressive reduction of GMV in cerebellar and cerebral motor networks, whereas correlation with psychiatric scores displayed a more widespread GMV impairment in frontal, limbic, parietal, and also cerebellar structures. Interestingly, changes in global functioning were correlated with bilateral atrophy within the para-/hippocampus. While there was a good temporal association between worsening of motor symptoms and progression in cerebral and cortical neurodegeneration, the progression in psychiatric related neurodegeneration seemed to be more widespread and complex, showing progressive atrophy that preceded the further development of clinical psychiatric symptoms. |
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Authors:
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Kathrin Reetz; Rebekka Lencer; Johannes M Hagenah; Christian Gaser; Vera Tadic; Uwe Walter; Alexander Wolters; Susanne Steinlechner; Christine Zühlke; Katja Brockmann; Christine Klein; Arndt Rolfs; Ferdinand Binkofski |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Cerebellum (London, England) Volume: 9 ISSN: 1473-4230 ISO Abbreviation: Cerebellum Publication Date: 2010 Jun |
Date Detail:
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Created Date: 2010-05-10 Completed Date: 2010-08-12 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101089443 Medline TA: Cerebellum Country: United States |
Other Details:
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Languages: eng Pagination: 210-7 Citation Subset: IM |
Affiliation:
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Department of Neurology, RWTH Aachen University, Germany. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Brain Mapping Disease Progression Female Humans Image Processing, Computer-Assisted / methods Magnetic Resonance Imaging / methods Male Middle Aged Movement Disorders / etiology*, pathology* Mutation / genetics Neurologic Examination Spinocerebellar Ataxias / complications*, genetics, pathology* Statistics as Topic TATA-Box Binding Protein / genetics |
| Chemical | |
Reg. No./Substance:
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0/TATA-Box Binding Protein; 0/TBP protein, human |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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