Mizuno Y, Yokochi F, Ohta S, Mori H, Takubo H
Department of Neurology, Juntendo University School of Medicine, Tokyo.
No To Shinkei. 1996 Apr;48(4):381-93.
We report a 65-year-old man with parkinsonism, supranuclear gaze palsy, and dementia. The patient was well until 58 years of the age (1984) when he noted an onset of tremor in his right hand. He visited our neurology service two years after the onset; neurologic examination at that time revealed moderate restriction in down ward gaze, horizontal gaze nystagmus in left and right gaze, stooped posture with loss of arm swing when he walked, slight rigidity in the neck and the right upper and lower extremities, and resting tremor in his right hand and foot; mentation was intact. He was treated with 600 mg of levodopa with carbidopa; his tremor partially improved. He received left Vim thalamotomy on March 14 of 1987. His tremor disappeared after the thalamotomy. Post-operative course was complicated by transient clouding of consciousness due to subdural hematoma which developed after the surgery. Six months after the surgery, he noted increase in the unsteadiness of gait; he also experienced urinary incontinence once in a while, and he became mentally dull. In November of 1988, he had episodes of stiffening of his body. Although his spontaneous speech was very much reduced, he repeatedly hummed a same tune; no one could make him stop humming. In June of 1989, he was totally unable to move his eyes in the vertical direction. He was hospitalized to another hospital in May of 1990 where he died six month after admission because of pneumonia. The clinical course of this patient was characterized by the onset with parkinsonian resting tremor, and supranuclear gaze palsy and dementia in the later course. The patient was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had an overlap syndrome of progressive supranuclear palsy (PSP) and diffuse Lewy body disease. Majority of the participants thought that the patient had progressive supranuclear palsy, but many of them had an impression that parkinsonian tremor responding to levodopa as the initial symptom for PSP is rather unusual. Postmortem examination revealed severe loss of neurons in the substantia nigra, the globus pallidus, and the subthalamic nucleus with reactive gliosis; formy spheroids were seen in the substantia nigra, however, no Lewy bodies or neurofibrillary tangles were observed. Moderate neuronal loss was also seen in the dentate nucleus of the cerebellum. In addition, the precentral gyrus showed moderate neuronal loss, astrocytosis, and spongy change in the second layer; ballooned neurons were seen in the third and the fifth layers. Histologic characteristics were consistent with the pathologic diagnosis of corticobasal degeneration.
我们报告一名65岁男性，患有帕金森症、核上性凝视麻痹和痴呆症。该患者在58岁（1984年）之前情况良好，当时他注意到右手出现震颤。震颤发作两年后他前来我们的神经科就诊；当时的神经系统检查发现向下凝视有中度受限，左右凝视时出现水平凝视性眼球震颤，行走时姿势弯腰驼背且手臂摆动消失，颈部以及右上肢和下肢有轻度僵硬，右手和右脚有静止性震颤；精神状态正常。他接受了600毫克左旋多巴与卡比多巴的治疗；震颤部分改善。1987年3月14日他接受了左侧丘脑腹中间核切开术。丘脑切开术后他的震颤消失。术后病程因术后出现的硬膜下血肿导致意识短暂模糊而复杂化。手术后六个月，他注意到步态不稳加重；他还偶尔出现尿失禁，并且变得精神迟钝。1988年11月，他出现身体僵硬发作。尽管他的自发言语大幅减少，但他反复哼唱同一曲调；没人能让他停止哼唱。1989年6月，他完全无法在垂直方向移动眼睛。1990年5月他因肺炎在另一家医院住院，入院六个月后死亡。该患者的临床病程特点是起病时有帕金森病静止性震颤，后期出现核上性凝视麻痹和痴呆症。该患者在一次神经科临床病理讨论会（CPC）上被讨论，主要讨论者得出结论，该患者患有进行性核上性麻痹（PSP）和弥漫性路易体病的重叠综合征。大多数参与者认为该患者患有进行性核上性麻痹，但他们中的许多人觉得帕金森病震颤对左旋多巴有反应作为PSP的初始症状相当不寻常。尸检显示黑质、苍白球和丘脑底核有严重的神经元丧失并伴有反应性胶质增生；在黑质中可见丝状球体，然而，未观察到路易小体或神经原纤维缠结。小脑齿状核也有中度神经元丧失。此外，中央前回显示中度神经元丧失、星形细胞增多以及第二层的海绵状改变；在第三层和第五层可见气球样神经元。组织学特征与皮质基底节变性的病理诊断一致。
