Progressive supranuclear palsy combined with Alzheimer's disease: A clinicopathological study of two autopsy cases
Progressive supranuclear palsy combined with Alzheimer's disease: A clinicopathological study of two autopsy cases
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DOI：
10.1111/j.1440-1789.2008.00968.x
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发表时间：
2009-06-01
期刊：
影响因子：
2.3
通讯作者：
Mimura, Masaru
中科院分区：
文献类型：
--
作者：
Sakamoto, Rieko;Tsuchiya, Kuniaki;Mimura, Masaru
本文报告两例合并进行性核上性麻痹(PSP)和阿尔茨海默病(AD)的尸检确诊病例的临床病理特征。在组织病理学上，基底节和脑干中神经原纤维缠结(NFT)的数量和分布符合美国国家神经疾病和卒中学会(NINDS-SPSP)提出的PSP的病理标准。1例老年斑块和NFT的Braak分期为C期和V期，2例为C期和IV期。这些神经病理结果证实了这两例患者合并了PSP和AD。我们的患者临床表现为执行功能障碍，早期症状为记忆障碍。除步态障碍、核上性眼肌麻痹、假性球麻痹等神经症状外，情绪、个性改变及精神错乱也较突出。因此，在这两名患者中，PSP的皮质下痴呆的症状比AD相关的症状更明显。PSP和AD的并存使临床表现更加复杂，临床诊断更加困难。
We present here the clinicopathological characteristics of two autopsy-confirmed cases comorbid of progressive supranuclear palsy (PSP) and Alzheimer's disease (AD). Histopathologically, the amount and distribution of neurofibrillary tangles (NFTs) in the basal ganglia and brainstem fulfilled the pathological criteria of PSP proposed by the National Institute of Neurological Disorders and Stroke - The Society for PSP (NINDS-SPSP). The Braak stages of senile plaques and NFTs were stage C and stage V in Case 1, and stage C and stage IV in Case 2. These neuropathological findings confirmed that the two patients had combined PSP with AD. Our patients presented clinically with executive dysfunction prior to memory disturbance as an early symptom. Not only neurological symptoms such as gait disturbance, supranuclear ophthalmoplegia and pseudobulbar palsy, but emotional and personality changes and delirium were prominent. Therefore, symptoms of subcortical dementia of PSP were more predominant than AD-related symptoms in the present two patients. Comorbid PSP and AD further complicates the clinical picture and makes clinical diagnosis even more difficult.