DIFFUSE LEWY BODY DISEASE - CLINICAL-FEATURES IN 15 CASES

DIFFUSE LEWY BODY DISEASE - CLINICAL-FEATURES IN 15 CASES
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DOI:
10.1136/jnnp.52.6.709
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发表时间:
1989-06-01
影响因子:
11
通讯作者:
GODWINAUSTEN, RB
GODWINAUSTEN, RB
中科院分区:
医学1区
文献类型:
--
作者:
BYRNE, EJ;LENNOX, G;GODWINAUSTEN, RB

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一个卫生区在一年内经病理诊断为弥漫性路易体病15例。临床特征的范围和频率与以往的报道形成鲜明对比。大多数病例表现为典型的左旋多巴反应性帕金森病(6例)或轻度认知障碍(3例);其余6例仅表现为认知障碍。随着时间的推移,几乎所有患者都患上了痴呆症和帕金森病。痴呆症的类型是皮质性的,但不寻常的是,大多数(12例)在疾病的某个阶段表现出严重程度的日常波动。这些发现表明弥漫性路易体病并不罕见,其表现形式多种多样,从痴呆伴帕金森病到帕金森病伴痴呆。后一种表现模式表明,它应被认为是帕金森病痴呆的重要病理底物。
Fifteen cases of diffuse Lewy body disease were diagnosed on pathological grounds during a single year in one health district. The range and frequency of clinical features contrast strikingly with previous reports. The majority of cases presented with classical levodopa-responsive Parkinson''s disease either alone (6 cases) or with mild cognitive impairment (3 cases); the remaining 6 cases presented with cognitive impairment alone. In time almost all patients developed both dementia and Parkinsonism. The dementia was cortical in type, but unusual in that most (12 cases) showed day-to-day fluctuation in severity at some point in their illness. These findings suggest that diffuse Lewy body disease is not rare, and that it presents in a range of ways from dementia with subsequent Parkinsonism to Parkinson''s disease with subsequent dementia. The latter mode of presentation suggests that it should be considered as a significant pathological substrate of dementia in Parkinson''s disease.