Hughes A J, Daniel S E, Blankson S, Lees A J
Department of Neurology, Middlesex Hospital, London, England.
Arch Neurol. 1993 Feb;50(2):140-8. doi: 10.1001/archneur.1993.00540020018011.
The clinical details of 100 cases of histologically confirmed Parkinson's disease were examined and correlated with pathologic findings. Age at disease onset (mean, 62.4 years), disease duration (mean, 13.1 years), and age at death (mean, 75.5 years) were similar to those in previous smaller series. Asymmetric, tremulous onset was most common, although 23% of patients had no rest tremor. Motor fluctuations and dyskinesias occurred in 60% of levodopa-treated patients. All patients had clinical parkinsonism; however, 12 had atypical clinical features of Parkinson's disease, including severe early dementia, fluctuating confusional states, no response to levodopa, and early marked autonomic disturbance. Neuropathologic examination found coexistent Alzheimer-type change in 17 cases and striatal abnormality--mainly vascular--in 34 cases. Cortical Lewy bodies were present in all cases, but only four satisfied proposed criteria for diffuse Lewy body disease. Dementia occurred in 44% of cases; 29% had Alzheimer's disease, 10% had numerous cortical Lewy bodies, and 6% had a possible vascular cause; in 55% no definite pathologic cause was found. Nigral cell loss correlated with disease duration and severity. Although the general pattern of disease conformed to traditional descriptions, the findings broaden the present clinical and pathologic spectrum of Parkinson's disease.
对100例经组织学确诊的帕金森病患者的临床细节进行了检查,并与病理结果进行了关联分析。发病年龄(平均62.4岁)、病程(平均13.1年)和死亡年龄(平均75.5岁)与之前较小系列研究中的情况相似。不对称、震颤起病最为常见,不过23%的患者无静止性震颤。60%接受左旋多巴治疗的患者出现运动波动和异动症。所有患者均有临床帕金森综合征表现;然而,12例患者具有帕金森病的非典型临床特征,包括严重的早期痴呆、波动性意识模糊状态、对左旋多巴无反应以及早期明显的自主神经功能障碍。神经病理学检查发现,17例患者并存阿尔茨海默型改变,34例患者存在纹状体异常——主要是血管性异常。所有病例均存在皮质路易小体,但只有4例符合弥漫性路易体病的拟定标准。44%的病例出现痴呆;29%患有阿尔茨海默病,10%有大量皮质路易小体,6%可能由血管原因引起;55%未发现明确的病理原因。黑质细胞丢失与病程和严重程度相关。尽管疾病的总体模式符合传统描述,但这些发现拓宽了目前帕金森病的临床和病理谱。