SENILE DEMENTIA OF LEWY BODY TYPE - A CLINICALLY AND NEUROPATHOLOGICALLY DISTINCT FORM OF LEWY BODY DEMENTIA IN THE ELDERLY

SENILE DEMENTIA OF LEWY BODY TYPE - A CLINICALLY AND NEUROPATHOLOGICALLY DISTINCT FORM OF LEWY BODY DEMENTIA IN THE ELDERLY
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DOI:
10.1016/0022-510x(90)90236-g
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发表时间:
1990-02-01
影响因子:
4.4
通讯作者:
PERRY, EK
PERRY, EK
中科院分区:
医学3区
文献类型:
--
作者:
PERRY, RH;IRVING, D;PERRY, EK

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在一组年龄为 71-90 岁的精神病病例中发现了一种痴呆综合征,最初表现为亚急性/急性混乱状态,经常波动,并伴有幻视和行为障碍。临床上,这些病例不符合阿尔茨海默氏病的诊断标准,尽管尸检时没有发现明显的缺血性病变,但许多病例被分配到多发梗塞性痴呆组。许多病例有明显的轻度锥体外系特征,但不存在帕金森病的特征性临床三联征,即震颤、强直和运动不能。详细的神经病理学检查显示,脑干和其他皮层下核团中存在路易体形成和选择性神经元丢失,同时新皮层和边缘皮层中也存在路易体形成,其密度远低于先前报道的弥漫性路易体病。存在不同程度的老年退行性改变;大多数情况下有大量的老年斑和极少的神经原纤维缠结。该群体的临床和神经病理学特征都不是帕金森病或阿尔茨海默病的典型特征,但表明是一种独特的神经退行性疾病,属于路易体疾病谱系的一部分,其中精神症状比运动障碍占主导地位,并最终导致精神老年病入院。在对临床评估的痴呆症患者进行的一系列连续尸检中,神经病理学分析表明,此类病例可能占 70 岁以上住院痴呆症老年人群的 20%,这一观察结果与老年人痴呆症的诊断和治疗明显相关。
A dementing syndrome has been identified in a group of psychiatric cases aged 71-90 years, presenting initially with a subacute/acute confusional state, often fluctuating and associated with visual hallucinations and behavioural disturbances. Clinically, these cases did not meet criteria for a diagnosis of Alzheimer''s disease, and many were assigned to the multiinfarct dementia group, although no significant ischaemic lesions were evident at autopsy. Mild extrapyramidal features were apparent in a number of cases but the characteristic clinical triad of Parkinson''s disease, i.e., tremor, rigidity, and akinesia, was absent. Detailed neuropathological examination revealed Lewy body formation and selective neuronal loss in brain stem and other subcortical nuclei, accompanied by Lewy body formation in neo- and limbic cortex, at densities well below those previously reported in diffuse Lewy body disease. A variable degree of senile degenerative change was present; numerous senile plaques and minimal neurofibrillary tangles in most cases. Neither the clinical nor the neuropathological features of this group are typical of Parkinson''s or Alzheimer''s disease, but suggest a distinct neurodegenerative disorder, part of the Lewy body disease spectrum, in which mental symptoms predominate over motor disabilities and lead to eventual psychogeriatric hospital admission. In a sequential series of autopsies conducted on clinically assessed demented patients, neuropathological analysis has indicated that such cases may comprise up to 20% of a hospitalized population of demented old people over the age of 70 years, an observation clearly relevant to the diagnosis and management of dementia in the elderly.