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进行性核上性麻痹合并阿尔茨海默病:两例尸检病例的临床病理研究

Progressive supranuclear palsy combined with Alzheimer's disease: a clinicopathological study of two autopsy cases.

作者信息

Sakamoto Rieko, Tsuchiya Kuniaki, Yoshida Ryoichi, Itoh Yoshinori, Furuta Nobuo, Kosuga Asako, Sugai Yuichi, Mimura Masaru

机构信息

Department of Psychiatry, Yokufukai Geriatric Hospital, Showa University School of Medicine, Tokyo, Japan.

出版信息

Neuropathology. 2009 Jun;29(3):219-29. doi: 10.1111/j.1440-1789.2008.00968.x. Epub 2008 Oct 20.

Abstract

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.

摘要

我们在此呈现两例经尸检确诊的同时合并进行性核上性麻痹(PSP)和阿尔茨海默病(AD)病例的临床病理特征。组织病理学上,基底节和脑干中神经原纤维缠结(NFTs)的数量和分布符合美国国立神经疾病和中风研究所 - 进行性核上性麻痹协会(NINDS - SPSP)提出的PSP病理标准。病例1中,老年斑和NFTs的Braak分期为C期和V期,病例2中为C期和IV期。这些神经病理学发现证实这两名患者患有PSP合并AD。我们的患者临床上以执行功能障碍先于记忆障碍作为早期症状。不仅步态障碍、核上性眼肌麻痹和假性球麻痹等神经症状突出,而且情绪和人格改变以及谵妄也很明显。因此,在这两名患者中,PSP的皮质下痴呆症状比AD相关症状更为突出。PSP和AD合并存在使临床情况更加复杂,也使临床诊断更加困难。

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