多系统萎缩17例临床分析和一例尸检报告

Clinical and pathological research on 17 cases of multi plesystem atrophy

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.