Oda T, Ikeda K, Akamatsu W, Iwabuchi K, Akiyama H, Kondo H, Seta K, Kato Y, Kogure T, Hori K
Department of Neuropsychiatry, National Shimofusa Sanatorium.
Seishin Shinkeigaku Zasshi. 1995;97(9):757-69.
We report a 69-year-old woman who was clinically diagnosed as having a frontal lobe-type of Pick's disease. The initial symptoms were personality changes and problematic behaviors. The patient showed intellectual decline, "stehende Redensarten" and abnormal attitude in interpersonal situations such as inattentiveness and indifference in the course of the disease. Brain CT revealed a marked atrophy of the frontal lobes. In the terminal stage the patient had severe dementia, mutism, parkinsonism and cervical dystonia. Neuropathologically, there was a marked atrophy of the frontal lobes. The superior frontal gyrus was most severely atrophic. Histological study revealed mild to moderate loss of neurons, hyperplasia of protoplasmic astrocytes and many balooned neurons in the deep layers of the atrophied cerebral cortex. Severe neuronal loss was even seen only in a part of the superior frontal gyrus. The cerebral white manner showed marked diffuse fibrillary gliosis. There was neuronal loss with gliosis in the thalamus, lentiform nucleus, subthalamic nucleus, substantia nigra and inferior olivary nucleus. Marked gliosis was seen in the midbrain and pontine tegmentum. Sections from several levels of the spinal cord also showed marked gliosis of the gray matter. Antibodies against human tau stained massive argyrophilic thread-like structures and oligodendroglial microtubular masses in the affected lesions. Neurofibrillary tangles were localized in the hippocampus and parahippocampal region. Neither Pick's body nor senile plaque were observed. Corticobasal degeneration (CBD) is a neurodegenerative disease initially presenting with unilateral motor disturbances. Typical initial symptoms are rigidity, akinesia and apraxia of an affected arm. The clinical phenotype might depend upon the affected areas of the cerebral cortex. Our patient initially exhibited personality changes and was clinically diagnosed as having Pick's disease. Although our case had unusual distribution pattern of the cerebral atrophy, it was pathologically diagnosed as CBD. The review of the literature suggests the presence of clinical varieties in CBD.
我们报告一例69岁女性，临床诊断为额叶型皮克病。初始症状为性格改变和行为问题。患者在病程中出现智力衰退、“固定语句”以及人际情境中的异常态度，如注意力不集中和冷漠。脑部CT显示额叶明显萎缩。终末期患者出现严重痴呆、缄默、帕金森综合征和颈部肌张力障碍。神经病理学检查发现额叶明显萎缩。额上回萎缩最为严重。组织学研究显示，萎缩的大脑皮质深层神经元轻度至中度丢失、原浆性星形胶质细胞增生以及许多气球样神经元。甚至仅在部分额上回可见严重的神经元丢失。脑白质显示明显的弥漫性纤维性胶质增生。丘脑、豆状核、底丘脑核、黑质和下橄榄核存在神经元丢失伴胶质增生。中脑和脑桥被盖可见明显的胶质增生。脊髓不同节段切片也显示灰质明显胶质增生。抗人tau抗体染色显示，受累病变中有大量嗜银丝状结构和少突胶质细胞微管团块。神经原纤维缠结定位于海马和海马旁区域。未观察到皮克小体和老年斑。皮质基底节变性（CBD）是一种神经退行性疾病，最初表现为单侧运动障碍。典型的初始症状是受累手臂的僵硬、运动不能和失用症。临床表型可能取决于大脑皮质受累区域。我们的患者最初表现为性格改变，临床诊断为皮克病。尽管我们的病例脑萎缩分布模式不寻常，但病理诊断为CBD。文献回顾提示CBD存在临床变异型。
