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远端上肢青少年型脊髓性肌萎缩症(平山病)的动态F波研究

A study of dynamic F-waves in juvenile spinal muscular atrophy of the distal upper extremity (Hirayama disease).

作者信息

Zheng Chaojun, Zhu Yu, Yang Shuo, Lu Feizhou, Jin Xiang, Weber Robert, Jiang Jianyuan

机构信息

Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai 200040, China.

Department of Physical Medicine and Rehabilitation, Upstate Medical University, State University of New York at Syracuse, Syracuse, NY 10212, USA.

出版信息

J Neurol Sci. 2016 Aug 15;367:298-304. doi: 10.1016/j.jns.2016.06.032. Epub 2016 Jun 16.

DOI:10.1016/j.jns.2016.06.032
PMID:27423607
Abstract

OBJECTIVE

The study aimed to analyse changes in the upper limb F-waves during neck flexion in patients with Hirayama disease (HD).

MATERIAL AND METHODS

This study included 41 healthy subjects, 38 HD patients and 24 patients with amyotrophic lateral sclerosis (ALS). Bilateral F-waves were consecutively recorded 20 times with the neck both in the standard position and after persistent neck flexion for 30min. The persistence, minimal latencies, chronodispersion, F/M ratios and amplitudes of the F-waves and repeater F-waves were compared between the standard neck and neck flexion positions.

RESULTS

During neck flexion, repeater F-waves were found in more HD patients, the percentage of both the ulnar and median repeater F-waves increased significantly, and higher F/M ratios were observed on the symptomatic side (P<0.05). No differences in the F-waves were observed in the healthy subjects or the ALS patients during neck flexion (P>0.05).

DISCUSSION

HD might be more likely to present as a position-related dysfunction rather than a spinal cord-intrinsic disease. Thus, HD patients could be counselled to avoid neck flexion for long periods of time to prevent further damage, especially in the progressive stage of the disease.

摘要

目的

本研究旨在分析平山病(HD)患者颈部屈曲时上肢F波的变化。

材料与方法

本研究纳入41名健康受试者、38例HD患者和24例肌萎缩侧索硬化症(ALS)患者。在颈部处于标准位置以及持续颈部屈曲30分钟后,双侧F波连续记录20次。比较标准颈部位置和颈部屈曲位置之间F波和重复F波的出现率、最小潜伏期、时限离散度、F/M比值及波幅。

结果

颈部屈曲时,更多HD患者出现重复F波,尺神经和正中神经重复F波的百分比均显著增加,且在症状侧观察到更高的F/M比值(P<0.05)。颈部屈曲时,健康受试者和ALS患者的F波未观察到差异(P>0.05)。

讨论

HD可能更倾向于表现为与姿势相关的功能障碍而非脊髓原发性疾病。因此,建议HD患者避免长时间颈部屈曲以防止进一步损伤,尤其是在疾病进展阶段。

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