摘要
目的 分析多系统萎缩的临床、病理特点和诊断标准。方法 按Gilman诊断标准 ,回顾性分析了 17例多系统萎缩的临床资料和其中 1例病理资料。结果 按Gilman诊断标准 ,本组 17例中 ,确诊多系统萎缩 1例 ,拟诊 12例 ,可疑者 4例。首发症状表现为植物神经功能障碍者 8例 ,锥体外系体征 8例。病程中出现姿位性低血压的有 12例 ;排尿障碍 16例 ,阳痿 9例 ;帕金森综合征症状12例 ;共济失调 13例 ;皮质脊髓束损害 12例。头颅MRI发现 10例脑萎缩。 1例经尸检证实存在少突胶质细胞包涵体。结论 多系统萎缩是累及植物神经、锥体外系、小脑和皮质脊髓束 ,并具少突胶质细胞包涵体等特定神经病理表现的变性疾病。Gilman诊断标准具有较强的临床可操作性。
Objective To analyze the clinical features, pathological manifestation and diagnostic crit eria of multiple system atrophy(MSA). Methods 1 case autopsy d ata and 17 cases retrospective analysis clinical material based on Gilman diag nostic criteria were reported. Results According to Gilman diagnostic crite r ia, 1 case was diagnosed as definite MSA, 12 were probable MSA, 4 cases were pos sible MSA. By initiative symptoms and signs, 8 cases showed autonomic dysfuncti o n and another 8 showed extrapyramidal motor disturbance. In duration of disease , 12 cases presented orthostatic hypotension, 16 urinary incontinence, 9 impotenc e, 12 Parkinson syndrome, 13 cerebellar dysfunction and 12 pyramidal tract inv olvement. 10 cases were found cerebral atrophy on brain MRI. Glial cytoplasmic i nclusions were found in one autopsy case. Conclusion MSA is a neurodegenerative disease involving the autonomic nervous sy stem, extrapyramidal, cerebellar and pyramidal system, in which glial cytoplasmi c inclusions finding is a specific neuropathological manifestation. Gilman d iagnosis criteria are strongly practicable in clinics.
出处
《中华神经科杂志》
CAS
CSCD
北大核心
2001年第5期287-290,共4页
Chinese Journal of Neurology