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

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

doi:10.3389/fneur.2022.969484

Frontiers in Neurology (Aug 2022)

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

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

Affiliations

Hongwei Wang: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Hongwei Wang: Spine Center Fudan University, Shanghai, China
Ye Tian: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Ye Tian: Spine Center Fudan University, Shanghai, China
Jianwei Wu: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Jianwei Wu: Spine Center Fudan University, Shanghai, China
Chi Sun: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Chi Sun: Spine Center Fudan University, Shanghai, China
Cong Nie: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Cong Nie: Spine Center Fudan University, Shanghai, China
Chaojun Zheng: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Chaojun Zheng: Spine Center Fudan University, Shanghai, China
Fei Zou: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Fei Zou: Spine Center Fudan University, Shanghai, China
Xinlei Xia: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Xinlei Xia: Spine Center Fudan University, Shanghai, China
Xiaosheng Ma: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Xiaosheng Ma: Spine Center Fudan University, Shanghai, China
Feizhou Lyu: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Feizhou Lyu: Spine Center Fudan University, Shanghai, China
Feizhou Lyu: Department of Orthopedics, Shanghai Fifth People's Hospital, Fudan University, Shanghai, China
Jianyuan Jiang: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Jianyuan Jiang: Spine Center Fudan University, Shanghai, China
Hongli Wang: Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, China
Hongli Wang: Spine Center Fudan University, Shanghai, China

DOI: https://doi.org/10.3389/fneur.2022.969484
Journal volume & issue: Vol. 13

Abstract

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PurposeHirayama 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.MethodsBaseline 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.ResultsIn 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.ConclusionThe 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.

Published in Frontiers in Neurology

ISSN: 1664-2295 (Online)
Publisher: Frontiers Media S.A.
Country of publisher: Switzerland
LCC subjects: Medicine: Internal medicine: Neurosciences. Biological psychiatry. Neuropsychiatry: Neurology. Diseases of the nervous system
Website: https://www.frontiersin.org/journals/neurology/

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