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Altered corticomotor‐cerebellar integrity in young ataxia telangiectasia patients

Overview of attention for article published in Movement Disorders, July 2014
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Title
Altered corticomotor‐cerebellar integrity in young ataxia telangiectasia patients
Published in
Movement Disorders, July 2014
DOI 10.1002/mds.25970
Pubmed ID
Authors

Ishani Sahama, Kate Sinclair, Simona Fiori, Kerstin Pannek, Martin Lavin, Stephen Rose

Abstract

Magnetic resonance imaging (MRI) research in identifying altered brain structure and function in ataxia-telangiectasia, an autosomal recessive neurodegenerative disorder, is limited. Diffusion-weighted MRI were obtained from 11 ataxia telangiectasia patients (age range, 7-22 years; mean, 12 years) and 11 typically developing age-matched participants (age range, 8-23 years; mean, 13 years). Gray matter volume alterations in patients were compared with those of healthy controls using voxel-based morphometry, whereas tract-based spatial statistics was employed to elucidate white matter microstructure differences between groups. White matter microstructure was probed using quantitative fractional anisotropy and mean diffusivity measures. Reduced gray matter volume in both cerebellar hemispheres and in the precentral-postcentral gyrus in the left cerebral hemisphere was observed in ataxia telangiectasia patients compared with controls (P < 0.05, corrected for multiple comparisons). A significant reduction in fractional anisotropy in the cerebellar hemispheres, anterior/posterior horns of the medulla, cerebral peduncles, and internal capsule white matter, particularly in the left posterior limb of the internal capsule and corona radiata in the left cerebral hemisphere, was observed in patients compared with controls (P < 0.05). Mean diffusivity differences were observed within the left cerebellar hemisphere and the white matter of the superior lobule of the right cerebellar hemisphere (P < 0.05). Cerebellum-localized gray matter changes are seen in young ataxia telangiectasia patients along with white matter tract degeneration projecting from the cerebellum into corticomotor regions. The lack of cortical involvement may reflect early-stage white matter motor pathway degeneration within young patients. © 2014 International Parkinson and Movement Disorder Society.

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Mendeley readers

The data shown below were compiled from readership statistics for 26 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 26 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 8 31%
Student > Bachelor 4 15%
Other 3 12%
Student > Doctoral Student 2 8%
Student > Ph. D. Student 2 8%
Other 4 15%
Unknown 3 12%
Readers by discipline Count As %
Medicine and Dentistry 9 35%
Neuroscience 4 15%
Nursing and Health Professions 2 8%
Agricultural and Biological Sciences 2 8%
Unspecified 1 4%
Other 2 8%
Unknown 6 23%