Depletion and sizes of motor units in spinal muscular atrophy
β Scribed by Victoria Galea; Darcy Fehlings; Susan Kirsch; Alan McComas
- Publisher
- John Wiley and Sons
- Year
- 2001
- Tongue
- English
- Weight
- 136 KB
- Volume
- 24
- Category
- Article
- ISSN
- 0148-639X
- DOI
- 10.1002/mus.1128
No coin nor oath required. For personal study only.
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## Abstract The hereditary neurodegenerative disease spinal muscular atrophy (SMA) with childhood onset is one of the most common genetic causes of infant mortality. The disease is characterized by selective loss of spinal cord motor neurons leading to muscle atrophy and is the result of mutations
## Abstract Reliable outcome measures that reflect the underlying disease process and correlate with motor function in children with SMA are needed for clinical trials. Maximum ulnar compound muscle action potential (CMAP) data were collected at two visits over a 4β6βweek period in children with SM