多系统萎缩17例临床分析和一例尸检报告

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；皮质兴髓束损害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。

Clinical and pathological research on 17 cases of multiple system atrophy

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.