Dissociated leg muscle atrophy in amyotrophic lateral sclerosis/motor neuron disease: the ‘split-leg’ sign

Disproportionate muscle atrophy is a distinct phenomenon in amyotrophic lateral sclerosis (ALS); however, preferentially affected leg muscles remain unknown. We aimed to identify this split-leg phenomenon in ALS and determine its pathophysiology. Patients with ALS (n = 143), progressive muscular atr...

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Bibliographic Details
Published in:Scientific reports 2020-09, Vol.10 (1), p.15661-15661, Article 15661
Main Authors: Min, Young Gi, Choi, Seok-Jin, Hong, Yoon-Ho, Kim, Sung-Min, Shin, Je-Young, Sung, Jung-Joon
Format: Article
Language:English
Subjects:
692/617/375/1917
692/617/375/365
Amyotrophic Lateral Sclerosis - complications
Amyotrophic Lateral Sclerosis - pathology
Amyotrophic Lateral Sclerosis - physiopathology
Electrophysiological Phenomena
Female
Humanities and Social Sciences
Humans
Leg
Male
Middle Aged
multidisciplinary
Muscular Atrophy - complications
Retrospective Studies
Science
Science (multidisciplinary)
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Summary:Disproportionate muscle atrophy is a distinct phenomenon in amyotrophic lateral sclerosis (ALS); however, preferentially affected leg muscles remain unknown. We aimed to identify this split-leg phenomenon in ALS and determine its pathophysiology. Patients with ALS (n = 143), progressive muscular atrophy (PMA, n = 36), and age-matched healthy controls (HC, n = 53) were retrospectively identified from our motor neuron disease registry. We analyzed their disease duration, onset region, ALS Functional Rating Scale-Revised Scores, and results of neurological examination. Compound muscle action potential (CMAP) of the extensor digitorum brevis (EDB), abductor hallucis (AH), and tibialis anterior (TA) were reviewed. Defined by CMAP EDB /CMAP AH (SI EDB ) and CMAP TA /CMAP AH (SI TA ), respectively, the values of split-leg indices (SI) were compared between these groups. SI EDB was significantly reduced in ALS (p 
ISSN:2045-2322
2045-2322
DOI:10.1038/s41598-020-72887-7