Journal
NEUROMUSCULAR DISORDERS
Volume 19, Issue 1, Pages 53-58Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.nmd.2008.10.002
Keywords
Amyotrophic lateral sclerosis; Motor neuron disease; Imaging of; Neurobiology of; Dementia; TDP-43
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Funding
- Prinses Beatrix Fonds
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There is an ongoing discussion whether ALS is primarily a disease of upper motor neurons or lower motor neurons. We undertook a review to assess how new insights have contributed to solve this controversy. For this purpose we selected relevant publications from 1995 onwards focussing on (I) primary targets and disease progression in ALS and variants of ALS, (2) brain imaging markers for upper motor neuron lesion, and (3) evidence for ALS being a multisystem disorder. Clinically, upper motor and lower motor neuron symptoms can occur in any order over time. Brain imaging markers show upper motor neuron involvement in early disease. Overlap syndromes of ALS and dementia, and involvement of autonomic and sensory nerves occur frequently. PFT/SPECT scans, functional MRI and voxel based morphometry Studies clearly show abnormalities in extra-motor areas of the brain. Pathologically, the 43 kDa TAR DNA-binding protein (TDP-43) provides a Clue to these overlapping disorders. In Conclusion. evidence accumulates that ALS is a multisystem disorder rather than a pure lower and/or upper motor neuron disorder. (C) 2008 Elsevier B.V. All rights reserved.
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