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学科主题临床医学
Clinical features of Hirayama disease in mainland China
Zhou, Bo1; Chen, Lei1; Fan, Dongsheng2; Zhou, Dong1
关键词Hirayama Disease Muscular Atrophy Cervical Spinal Cord
刊名AMYOTROPHIC LATERAL SCLEROSIS
2010
DOI10.3109/17482960902912407
11期:1-2页:133-139
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Clinical Neurology
研究领域[WOS]Neurosciences & Neurology
关键词[WOS]JUVENILE MUSCULAR-ATROPHY ; DISTAL UPPER EXTREMITY ; SPINAL-CORD ; AMYOTROPHY ; MYELOPATHY ; TAIWAN
英文摘要

The aim of this study was to investigate patients with Hirayama disease in mainland China. A total of 192 patients (167 males, 25 females) collected from mainland China were included. Their clinical features, electrophysiology, imaging, muscle biopsy and laboratory tests, treatments, and prognosis were analysed. We compared the results with data from other countries or regions. The mean age at onset was 16.8 years. Onset was insidious, with symptoms of muscle weakness and atrophy in the distal muscles of the upper limb. Tremor on finger extension was noted in 77.6% of patients and cold paresis in 81.3%. The clinical course plateaued within five years in 89.1% of patients. Time from disease onset to definitive diagnosis of our series is longer than that from other countries or regions. There is no geographically based difference in the clinical presentation of Hirayama disease. Thus, our study supports the notion that Hirayama disease is a benign self-limited disorder with juvenile preponderance and asymmetric muscular atrophy of the distal portion of the upper limb. Hirayama disease is under-recognized in mainland China.

语种英语
WOS记录号WOS:000275059800019
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被引频次:28[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/55829
专题北京大学第三临床医学院_神经内科
作者单位1.Sichuan Univ, Huaxi Hosp, Dept Neurol, Chengdu 610064, Peoples R China
2.Peking Univ, Hosp 3, Dept Neurol, Beijing 100871, Peoples R China
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Zhou, Bo,Chen, Lei,Fan, Dongsheng,et al. Clinical features of Hirayama disease in mainland China[J]. AMYOTROPHIC LATERAL SCLEROSIS,2010,11(1-2):133-139.
APA Zhou, Bo,Chen, Lei,Fan, Dongsheng,&Zhou, Dong.(2010).Clinical features of Hirayama disease in mainland China.AMYOTROPHIC LATERAL SCLEROSIS,11(1-2),133-139.
MLA Zhou, Bo,et al."Clinical features of Hirayama disease in mainland China".AMYOTROPHIC LATERAL SCLEROSIS 11.1-2(2010):133-139.
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