遗传性痉挛性截瘫伴胼胝体发育不良的临床分析及文献回顾  

Clinical Analysis and Literature Review on the Hereditay Spastic Paraplegia with a Thin Corpus Callosum

作　　者：杨任民[1] 张立新[1] 侯焕喜[1] 

机构地区：[1]安徽中医学院神经病学研究所附院,230061

出　　处：《中国临床神经科学》2005年第1期55-57,82,共4页Chinese Journal of Clinical Neurosciences

摘　　要：目的:分析遗传性痉挛性截瘫伴胼胝体发育不良(HSP-TCC)的临床特点,以提高对此病的认识。方法:结合文献复习并报告1 例HSP-TCC患者的临床和影像学表现。结果:本例HSP-TCC的双亲为表兄妹婚配,临床以缓慢进行性痉挛性截瘫为主要表现,MRI 示胼胝体发育不良和脑室周围白质疏松,侧脑室对称轻度扩大;胸腰髓明显变细,但脊髓本身未见明显异常信号。结论:建议凡家族性痉挛性截瘫患者均应常规行MRI检查,以判定是否并有胼胝体发育不良,可提高本病的诊断率。Aim: To explore the feature of hereditary spastic paraplegia with thin corpus callosum ( HSP-TCC) for the profound recognition of this disease. Methods: Relevant refrences were reviewed in combination with the report of both the clinical and magnetic resonance imaging (MRI) finding of a patient with HSP-TCC. Results: The patient's parents are early stage.The main clinical finding was the slowly progressive spastic paraplegia; MRI demonstrated a thin corpus callosum; the spinal cord in the thoracolumbar segment was apparently thin without obvious abnormality within the cord. Conclusion: Routine MRI scare may be discovered for the patient with HSP-TCC.

关 键 词：胼胝体发育不良 遗传性痉挛性截瘫 HSP TCC 临床分析 诊断率 白质疏松 婚配 腰髓 脊髓 

分 类 号：R682.22[医药卫生—骨科学] R737[医药卫生—外科学]