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近端受累型平山病的影像学和电生理特征:一项回顾性研究。

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

作者信息

Wang Hongwei, Tian Ye, Wu Jianwei, Sun Chi, Nie Cong, Zheng Chaojun, Zou Fei, Xia Xinlei, Ma Xiaosheng, Lyu Feizhou, Jiang Jianyuan, Wang Hongli

机构信息

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

Spine Center Fudan University, Shanghai, China.

出版信息

Front Neurol. 2022 Aug 11;13:969484. doi: 10.3389/fneur.2022.969484. eCollection 2022.

DOI:10.3389/fneur.2022.969484
PMID:36034284
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9406812/
Abstract

PURPOSE

Hirayama disease (HD) has been largely believed to affect only distal muscles. However, the proximal upper extremities have been affected in some cases, which can be confused with motor neuron diseases.

METHODS

Baseline data, deep tendon reflex, Hoffmann sign, cervical curvature, sagittal Cobb angle, atrophied spinal cord, high signal intensity, loss of attachment, and affected muscles and segments on electromyography (EMG) were retrospectively obtained and compared between patients with HD with proximal involvement and patients with simple distal HD in one center from September 2007 to April 2022.

RESULTS

In this study, fifteen patients with proximal HD and 30 patients with simple distal HD were included. The proximal group had a larger proportion of patients with decreased biceps reflex, decreased triceps reflex, brisk or hyperactive knee reflex, positive Hoffmann sign, and cervical kyphosis. The curvatures of the upper part of the cervical spine (C2-4) were lost to a greater degree in the proximal group. More affected segments were observed on magnetic resonance imaging (MRI) and electromyography in the proximal group.

CONCLUSION

The injured segments were longer and the upper curvature of the cervical spine was poorer in patients with HD with proximal involvement. These findings indicated that proximal involvement may indicate more serious HD.

摘要

目的

平山病(HD)一直被广泛认为仅累及远端肌肉。然而,在某些病例中,近端上肢也会受到影响,这可能会与运动神经元病相混淆。

方法

回顾性获取2007年9月至2022年4月期间在一个中心就诊的近端受累的HD患者和单纯远端HD患者的基线数据、深腱反射、霍夫曼征、颈椎曲度、矢状面Cobb角、脊髓萎缩、高信号强度、附着丧失以及肌电图(EMG)检查中受累的肌肉和节段,并进行比较。

结果

本研究纳入了15例近端HD患者和30例单纯远端HD患者。近端组中肱二头肌反射减弱、肱三头肌反射减弱、膝反射亢进或活跃、霍夫曼征阳性以及颈椎后凸的患者比例更高。近端组颈椎上部(C2 - 4)的曲度丧失程度更大。在近端组的磁共振成像(MRI)和肌电图检查中观察到更多受累节段。

结论

近端受累的HD患者损伤节段更长,颈椎上曲度更差。这些发现表明近端受累可能提示HD病情更严重。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/3297409fd4a1/fneur-13-969484-g0005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/9f6f01678607/fneur-13-969484-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/13f1355bc3ec/fneur-13-969484-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/a3464b25b66a/fneur-13-969484-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/527b770e4ae4/fneur-13-969484-g0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/3297409fd4a1/fneur-13-969484-g0005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/9f6f01678607/fneur-13-969484-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/13f1355bc3ec/fneur-13-969484-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/a3464b25b66a/fneur-13-969484-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/527b770e4ae4/fneur-13-969484-g0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a5/9406812/3297409fd4a1/fneur-13-969484-g0005.jpg

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Surg Neurol Int. 2021 Dec 20;12:622. doi: 10.25259/SNI_1081_2021. eCollection 2021.
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Interobserver and Intraobserver Reproducibility and Reliability of the Huashan Clinical Classification System for Hirayama Disease.华山平山病临床分类系统的观察者间及观察者内可重复性和可靠性
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