Walterfang, M. and Patenaude, B. and Abel, L. A. and Kluenemann, H. and Bowman, E. A. and Fahey, M. C. and Desmond, P. and Kelso, W. and Velakoulis, D. (2013) Subcortical Volumetric Reductions in Adult Niemann-Pick Disease Type C: A Cross-Sectional Study. AMERICAN JOURNAL OF NEURORADIOLOGY, 34 (7). pp. 1334-1340. ISSN 0195-6108, 1936-959X
Full text not available from this repository. (Request a copy)Abstract
BACKGROUND AND PURPOSE: Voxel-based analysis has suggested that deep gray matter rather than cortical regions is initially affected in adult Niemann-Pick type C. We sought to examine a range of deep gray matter structures in adults with NPC and relate these to clinical variables. MATERIALS AND METHODS: Ten adult patients with NPC (18-49 years of age) were compared with 27 age- and sex-matched controls, and subcortical structures were automatically segmented from normalized T1-weighted MR images. Absolute volumes (in cubic millimeters) were generated for a range of deep gray matter structures and were compared between groups and correlated with illness variables. RESULTS: Most structures were smaller in patients with NPC compared with controls. The thalamus, hippocampus, and striatum showed the greatest and most significant reductions, and left hippocampal volume correlated with symptom score and cognition. Vertex analysis of the thalamus, hippocampus, and caudate implicated regions involved in memory, executive function, and motor control. CONCLUSIONS: Thalamic and hippocampal reductions may underpin the memory and executive deficits seen in adult NPC. Volume losses in other subcortical regions may also be involved in the characteristic range of motor, psychiatric, and cognitive deficits seen in the disease.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | NEUROFIBRILLARY TANGLES; NEUROAXONAL DYSTROPHY; THALAMUS; NUCLEUS; ORGANIZATION; CHOLESTEROL; NEURONS; MEMORY; |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Psychiatrie und Psychotherapie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 06 Apr 2020 12:42 |
| Last Modified: | 06 Apr 2020 12:42 |
| URI: | https://pred.uni-regensburg.de/id/eprint/16389 |
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