Inagaki T, Seno H I, Iijima M, Nagai A, Bokura H, Kobayashi S, Wada M, Harada T
Department of Psychiatry, Shimane Medical University, Izumo, Japan.
No To Shinkei. 1998 Mar;50(3):263-71.
A 62-year-old man developed clumsiness, vertical ophthalmoplegia, right-side dominant parkinsonism, pyramidal signs, limb-kinetic apraxia and dementia. His brain MRI and SPECT revealed mild fronto-parietal atrophy and hypoperfusion predominately on the right side. At the age of 65, the patient died of sepsis. The duration of his illness was approximately 3 years. Clinical diagnosis was corticobasal degeneration (CBD). On neuropathological examination, there was no neuronal loss and many neurofibrillary tangles (NFTs) in the cerebral cortices. Basal ganglia and substantia nigra showed moderate to severe neuronal loss. And there were many NFTs and argyrophillic threads (threads) in the subthalamic nucleus and nuclei of the brainstem. These findings were almost compatible to those of PSP. However, the following findings were atypical for PSP, 1) mild atrophy and absence of gliosis in the tegmentum of the brain stem, 2) Mild neuronal loss in the subthalamic nucleus and the dentate nucleus without grumose degeneration. Furthermore, characteristic findings were noted on modified Gallyas-Braak stain. Numerous threads and many astrocytic plaques (AP) were shown in cerebral cortices, which were particular to CBD cases. So this case demonstrated neuropathological findings of both PSP and CBD. Therefore, it was difficult to classify this case as either PSP or CBD, and we diagnosed this case as a neuropathological overlapping case of both PSP and CBD.
一名62岁男性出现肢体运动笨拙、垂直性眼肌麻痹、右侧为主的帕金森综合征、锥体束征、肢体运动性失用和痴呆。他的脑部MRI和SPECT显示轻度额顶叶萎缩以及主要在右侧的灌注不足。65岁时,患者死于败血症。病程约3年。临床诊断为皮质基底节变性(CBD)。神经病理学检查显示,大脑皮质无神经元丢失,但有许多神经原纤维缠结(NFTs)。基底节和黑质显示中度至重度神经元丢失。丘脑底核和脑干核中有许多NFTs和嗜银纤维(纤维)。这些发现几乎与进行性核上性麻痹(PSP)的发现相符。然而,以下发现对于PSP来说是非典型的:1)脑干被盖轻度萎缩且无胶质细胞增生;2)丘脑底核和齿状核轻度神经元丢失,无颗粒样变性。此外,改良的Gallyas-Braak染色有特征性发现。大脑皮质显示大量纤维和许多星形细胞斑块(AP),这是CBD病例所特有的。所以该病例显示出PSP和CBD两者的神经病理学发现。因此,很难将该病例归类为PSP或CBD,我们将该病例诊断为PSP和CBD的神经病理学重叠病例。