Pattern Differences of Small Hand Muscle Atrophy in Amyotrophic Lateral Sclerosis and Mimic Disorders.

Jia Fang; Ming-sheng Liu; Yu-zhou Guan; Hua DU; Ben-hong LI; Bo Cui; Qing-yun Ding; Li-ying Cui

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Pattern Differences of Small Hand Muscle Atrophy in Amyotrophic Lateral Sclerosis and Mimic Disorders.

Author: Jia FANG ; Ming-Sheng LIU ; Yu-Zhou GUAN ; Hua DU ; Ben-Hong LI ; Bo CUI ; Qing-Yun DING ; Li-Ying CUI ¹
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1. Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
Publication Type:Journal Article
MeSH: Action Potentials; Adult; Aged; Amyotrophic Lateral Sclerosis; pathology; physiopathology; Diagnosis, Differential; Female; Hand; pathology; Humans; Male; Middle Aged; Muscle, Skeletal; physiopathology; Muscular Atrophy; pathology; physiopathology; Retrospective Studies; Spinal Muscular Atrophies of Childhood; pathology; Spondylosis; pathology
From: Chinese Medical Journal 2016;129(7):792-798
CountryChina
Language:English
Abstract:
BACKGROUNDAmyotrophic lateral sclerosis (ALS) and some mimic disorders, such as distal-type cervical spondylotic amyotrophy (CSA), Hirayama disease (HD), and spinobulbar muscular atrophy (SBMA) may present with intrinsic hand muscle atrophy. This study aimed to investigate different patterns of small hand muscle involvement in ALS and some mimic disorders.
METHODSWe compared the abductor digiti minimi/abductor pollicis brevis (ADM/APB) compound muscle action potential (CMAP) ratios between 200 ALS patients, 95 patients with distal-type CSA, 88 HD patients, 43 SBMA patients, and 150 normal controls.
RESULTSThe ADM/APB CMAP amplitude ratio was significantly higher in the ALS patients (P < 0.001) than that in the normal controls. The ADM/APB CMAP amplitude ratio was significantly reduced in the patients with distal-type CSA (P < 0.001) and the HD patients (P < 0.001) compared with that in the normal controls. The patients with distal-type CSA had significantly lower APB CMAP amplitude than the HD patients (P = 0.004). The ADM/APB CMAP amplitude ratio was significantly lower in the HD patients (P < 0.001) than that in the patients with distal-type CSA. The ADM/APB CMAP amplitude ratio of the SBMA patients was similar to that of the normal controls (P = 0.862). An absent APB CMAP and an abnormally high ADM/APB CMAP amplitude ratio (≥4.5) were observed exclusively in the ALS patients.
CONCLUSIONSThe different patterns of small hand muscle atrophy between the ALS patients and the patients with mimic disorders presumably reflect distinct pathophysiological mechanisms underlying different disorders, and may aid in distinguishing between ALS and mimic disorders.