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Motor unit number index in children with later-onset spinal muscular atrophy.
- Source :
-
Muscle & Nerve . Nov2020, Vol. 62 Issue 5, p633-637. 5p. - Publication Year :
- 2020
-
Abstract
- <bold>Background: </bold>Motor unit number index (MUNIX) is a validated electrophysiological biomarker in amyotrophic lateral sclerosis. MUNIX studies in spinal muscular atrophy (SMA) are limited.<bold>Methods: </bold>Later-onset SMA children (n = 13; three SMN2 copy number) were evaluated for Hammersmith Motor Function Scale Expanded (HMFSE) and MUNIX of right abductor pollicis brevis (APB) and abductor digiti minimi (ADM) muscles. Age-matched healthy controls (n = 8) were enrolled to obtain normative APB and ADM MUNIX values.<bold>Results: </bold>Mean APB and ADM MUNIX values in SMA subjects were significantly reduced (P < .001) compared with controls. HMFSE scores strongly correlated with ADM MUNIX (r 0.63).<bold>Conclusions: </bold>APB and ADM muscle MUNIX studies are feasible in SMA type 2 children. ADM MUNIX correlated with disease severity on motor function testing. MUNIX studies in later-onset SMA could be a potential biomarker of motor neuron loss. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 0148639X
- Volume :
- 62
- Issue :
- 5
- Database :
- Academic Search Index
- Journal :
- Muscle & Nerve
- Publication Type :
- Academic Journal
- Accession number :
- 146507500
- Full Text :
- https://doi.org/10.1002/mus.26909