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学科主题: 临床医学
题名:
Clinical features of Hirayama disease in mainland China
作者: Zhou, Bo1; Chen, Lei1; Fan, Dongsheng2; Zhou, Dong1
关键词: Hirayama disease ; muscular atrophy ; cervical spinal cord
刊名: AMYOTROPHIC LATERAL SCLEROSIS
发表日期: 2010
DOI: 10.3109/17482960902912407
卷: 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
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/55829
Appears in Collections:北京大学第三临床医学院_神经内科_期刊论文

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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

Recommended Citation:
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.
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