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Clinical features of hereditary spastic paraplegia with thin corpus callosum:report of 5 Chinese cases

唐北沙 陈昕 赵国华 沈璐 严新翔 江泓 罗巍

中华医学杂志(英文版)Issue(7):1002-1005,4.
中华医学杂志(英文版)Issue(7):1002-1005,4.

Clinical features of hereditary spastic paraplegia with thin corpus callosum:report of 5 Chinese cases

Clinical features of hereditary spastic paraplegia with thin corpus callosum:report of 5 Chinese cases

唐北沙 1陈昕 1赵国华 1沈璐 1严新翔 1江泓 1罗巍1

作者信息

  • 1. Department of Neurology, Xiangya Hospital, Central Southern University, Changsha 410008, China
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摘要

Abstract

Background Hereditary spastic paraplegia is a clinically and genetically heterogeneous group of neurodegenerative disorders of the motor system, characterized by slowly progressive spasticity and weakness of the lower extremities. This study was conducted to investigate the clinical features of hereditary spastic paraplegia with thin corpus callosum (HSP-TCC). Methods Clinical data from five patients and thirty-five previously published case reports of HSP-TCC were analyzed retrospectively. Results Most patients were adolescents at the onset of the disease, presenting with spastic paraparesis of the lower limbs and mental impairment. Some patients also had other clinical features, including spasticity of the upper limbs, cerebellar ataxia, and sensory disturbances. Cranial MRIs of the five patients revealed an extremely thin corpus callosum, sometimes with widened cerebral sulci and ventricles, as well as with cerebellar and cerebral atrophy. Conclusion The main clinical features of HSP-TCC include slowly progressive spastic paraplegia, mental impairment during the second decade of life, and an extremely thin corpus callosum as shown on cranial MRIs.

关键词

hereditary spastic paraplegia/corpus callosum/dementia/magnetic resonance imaging

Key words

hereditary spastic paraplegia/corpus callosum/dementia/magnetic resonance imaging

分类

医药卫生

引用本文复制引用

唐北沙,陈昕,赵国华,沈璐,严新翔,江泓,罗巍..Clinical features of hereditary spastic paraplegia with thin corpus callosum:report of 5 Chinese cases[J].中华医学杂志(英文版),2004,(7):1002-1005,4.

基金项目

This study was supported by a grant from the National 863 Project (No.2001AA227011). (No.2001AA227011)

中华医学杂志(英文版)

OACSTPCDMEDLINESCI

0366-6999

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