Mitani K, Uchihara T, Tamaru F, Endo K, Tsukagoshi H
Department of Neurology, Tokyo Metropolitan Geriatric Hospital.
Rinsho Shinkeigaku. 1993 Feb;33(2):155-61.
We reported two patients with clinical features of corticobasal degeneration (CBD), one with autopsy observations. Their illness began in their sixties, ran progressive course and ended in death in about five years. The initial symptom was difficulty in manipulating with the left hands. Gradually the left lower limbs and the right limbs were also involved and the left became useless. They developed Parkinsonism, myoclonus, dystonia, hyperreflexia and vertical gaze palsy. They also showed the dysfunction of the fronto-parietal cortices, including grasping reflex, motor neglect, hemispatial neglect, constructional disturbance and cortical sensory loss. Dementia did not manifest until the terminal stage. Neuro-radiological studies demonstrated mild dilatation of the right central sulcus and marked reduction in cerebral blood flow in the fronto-parietal cortices, predominant in the right. Pathological examination in one of the case confirmed the the clinical diagnosis of CBD. There was a neuronal loss affecting second and third layers of the fronto-parietal cortices, especially the right parasylvian region, associated with spongiosis in these laminae and marked gliosis in deeper layers. Swollen, poorly staining (achromatic) neurons were observed in fifth layer. The hippocampus was unaffected. In the substantia nigra, there was extensive loss of pigmented cells. No Pick bodies, senile plaques, neurofibrillary tangles were observed in any region of the brain. The initial manifestations of their illness, the motor disturbances in the left upper extremities, were distinctive features in our patients. We could find no detailed description of these symptoms in the literature. We analysed these manifestations and described the results.(ABSTRACT TRUNCATED AT 250 WORDS)
我们报告了两名具有皮质基底节变性（CBD）临床特征的患者，其中一名进行了尸检观察。他们的病情始于六十多岁，呈进行性发展，约五年后死亡。最初的症状是左手操作困难。逐渐地，左下肢和右下肢也受到影响，左侧肢体失去功能。他们出现了帕金森综合征、肌阵挛、肌张力障碍、反射亢进和垂直凝视麻痹。他们还表现出额顶叶皮质功能障碍，包括抓握反射、运动忽视、半侧空间忽视、结构障碍和皮质感觉丧失。直到疾病晚期才出现痴呆症状。神经放射学研究显示右侧中央沟轻度扩张，额顶叶皮质脑血流量显著减少，以右侧为主。其中一例的病理检查证实了CBD的临床诊断。额顶叶皮质的第二层和第三层存在神经元丢失，尤其是右侧岛周区域，这些层伴有海绵状变性，深层有明显的胶质增生。在第五层观察到肿胀、染色不佳（无色）的神经元。海马未受影响。在黑质中，色素细胞大量丢失。在大脑的任何区域均未观察到Pick小体、老年斑和神经原纤维缠结。他们疾病的最初表现，即左上肢的运动障碍，是我们患者的独特特征。我们在文献中未找到对这些症状的详细描述。我们分析了这些表现并描述了结果。（摘要截短至250字）
