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. 2016 Jul;95(28):e4210.
doi: 10.1097/MD.0000000000004210.

Correlations of clinical, neuroimaging, and electrophysiological features in Hirayama disease

Ming-Feng Liao  1 , Hong-Shiu Chang, Kuo-Hsuan Chang, Long-Sun Ro, Chun-Che Chu, Hung-Chou Kuo, Rong-Kuo Lyu
Affiliations

Correlations of clinical, neuroimaging, and electrophysiological features in Hirayama disease

Ming-Feng Liao et al. Medicine (Baltimore). 2016 Jul.

Abstract

Hirayama disease (HD) is characterized by development of asymmetric forearm muscle atrophy during adolescence with or without focal cervical spinal cord atrophy. The purpose of this study is to assess the correlation of clinical symptoms, disease progression, and electrophysiological findings with cervical spine magnetic resonance imaging (MRI) findings.The medical records, cervical spine MRIs, and electrophysiological findings of 44 HD patients were retrospectively reviewed and analyzed.Denervation changes in any single C5 to C7 root-innervated muscle (deltoid, biceps, triceps, or extensor digitorum communis) occurred more frequently in the 25 patients with cord atrophy than the 19 patients without cord atrophy (88% vs 53%, P = 0.02). Onset age, duration of disease progression, neurological examinations, nerve conduction study, and electromyographic findings from individual muscles were similar between patient groups.Compared with HD patients without cord atrophy, HD patients with cord atrophy experience a more severe denervation change in C5 to C7 root-innervated muscles.

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Conflict of interest statement

The authors have no conflicts of interest to disclose.

Figures

Figure 1
Figure 1
(A) Sagittal T2-weighted cervical magnetic resonance imaging (MRI) of the spinal cord atrophied at the C5 to C6 vertebral body levels (white arrow) in a 22-year-old man with progressive distal atrophy of the right hand for 2 years. (B) Sagittal T2-weighted cervical MRI of the spinal cord atrophied at the C6 vertebral level (white arrow) in a 17-year-old man with progressive distal atrophy of the right hand for 6 months. (C) Sagittal T2-weighted cervical MRI of the spinal cord without cord atrophy in a 19-year-old man with progressive distal atrophy of the right hand for 2 years.
Figure 2
Figure 2
Percentage of denervation change in individual muscles in Hirayama disease patients.
See this image and copyright information in PMC

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Supplementary concepts