Literature DB >> 33676826

Quantitative assessment of motor impairment and surgical outcome in Hirayama disease with proximal involvement using motor unit number index.

Chaojun Zheng1, Yu Zhu2, Qifeng Yu1, Dongqing Zhu3, Jun Li4, Feizhou Lyu5, Robert Weber2, Kai Qiao3, Jianyuan Jiang6.   

Abstract

OBJECTIVE: To assess the feasibility of motor unit number index (MUNIX) in quantitatively evaluating Hirayama disease (HD) with proximal involvement and to identify the effectiveness of anterior cervical fusion (ACF) in treating atypical HD with proximal involvement.
METHODS: This study included 28 atypical HD patients with proximal involvement (proximal-distal vs. distal-proximal groups: 5 vs. 23) and 41 healthy controls. All patients underwent pre- and postoperative 1-year MUNIX tests on abductor pollicis brevis (APB), abductor digiti minimi (ADM), biceps brachii (BB) and deltoid (Del). The disabilities of arm, shoulder and hand (DASH) and Medical Research Council (MRC) scales were also performed in these patients before and one year after operation.
RESULTS: Preoperatively, the patients in the distal-proximal group showed reduced compound muscle action potential (CMAP), decreased MUNIX and increased motor unit size index (MUSIX) in bilateral distal muscles and symptomatic-side proximal muscles (P < 0.05), and similar abnormalities were also observed in ADM, BB and Del on the symptomatic side in the proximal-distal groups (P < 0.05). Postoperative follow-up analysis identified increased MUNIX in the symptomatic-side proximal muscles with improved motor function in the proximal-distal groups (P < 0.05), and distal-proximal group patients showed an increase in both CMAP and MUSIX in the symptomatic-side proximal muscles (P < 0.05).
CONCLUSIONS: MUNIX may serve as an available supplementary test to quantitatively evaluate the motor dysfunction and treatment outcome in HD with proximal involvement. ACF procedures can effectively treat these atypical HD patients, especially for those whose symptoms started in proximal muscles.
Copyright © 2021 Elsevier Masson SAS. All rights reserved.

Entities:  

Keywords:  Hirayama disease; Motor unit number index; Proximal muscle involvement; Quantitative assessment; Surgical treatment

Year:  2021        PMID: 33676826     DOI: 10.1016/j.neucli.2021.02.002

Source DB:  PubMed          Journal:  Neurophysiol Clin        ISSN: 0987-7053            Impact factor:   3.734


  3 in total

Review 1.  Juvenile muscular atrophy of the distal upper extremity (Hirayama syndrome): a systematic review.

Authors:  Henrik C Bäcker; Jacob Bock; Peter Turner; Michael A Johnson; John Cunningham; Patrick Chan; Richard Gerraty
Journal:  Eur Spine J       Date:  2022-06-21       Impact factor: 3.134

Review 2.  Update on the Pathogenesis, Clinical Diagnosis, and Treatment of Hirayama Disease.

Authors:  Hongwei Wang; Ye Tian; Jianwei Wu; Sushan Luo; Chaojun Zheng; Chi Sun; Cong Nie; Xinlei Xia; Xiaosheng Ma; Feizhou Lyu; Jianyuan Jiang; Hongli Wang
Journal:  Front Neurol       Date:  2022-02-01       Impact factor: 4.003

3.  The radiological and electrophysiological characteristics of Hirayama disease with proximal involvement: A retrospective study.

Authors:  Hongwei Wang; Ye Tian; Jianwei Wu; Chi Sun; Cong Nie; Chaojun Zheng; Fei Zou; Xinlei Xia; Xiaosheng Ma; Feizhou Lyu; Jianyuan Jiang; Hongli Wang
Journal:  Front Neurol       Date:  2022-08-11       Impact factor: 4.086

  3 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.