Sato S, Hattori N, Matsumine H, Imai H, Mizuno Y
Department of Neurology Juntendo University School of Medicine, Tokyo.
Rinsho Shinkeigaku. 1998 Mar;38(3):246-51.
We report a 73-year-old woman who presented progressive motor clumsiness, cortical sensory loss, and focal parietal lobe atrophy. She was well until one year prior to the present admission when she suffered from what appeared to be mild Fisher syndrome from which she showed excellent recovery. However, soon she noted a gradual onset of difficulty in hand movements and in the recognition of objects by hands. Neurologic examination revealed an alert and well oriented Japanese woman without dementia. Cranial nerves were unremarkable. Although, she did not have aphasia, apraxia, or agnosia, she showed marked clumsiness in skilled hand movements such as using chopsticks, hand writing, and buttoning. She had no motor weakness, ataxia, rigidity, or spasticity. Deep tendon reflexes were symmetrically diminished. Sensory examination revealed cortical sensory loss such as disturbances of two point discrimination, weight sensation, and stereotactic sensations. Her motor clumsiness appeared to be caused by her cortical sensory loss. MRI revealed marked focal atrophy in the bilateral parietal lobe, particularly in the postcentral gyrus and the adjacent association areas. Recently, neurodegenerative disorders with focal brain atrophy such as corticobasal degeneration, Pick's disease, and dementia of frontal lobe type have been reported, however, our patient does not fit to any of these known disorders nor clinical features are distinctly different from Alzheimer's disease. Our patient may be another example of progressive cerebral degeneration with emphasis on the parietal cortex.
我们报告了一位73岁的女性,她出现了进行性运动笨拙、皮质感觉丧失和局灶性顶叶萎缩。在本次入院前一年,她一直状况良好,当时她患了看似轻度的费舍尔综合征,恢复情况良好。然而,很快她就注意到手部运动和通过手部识别物体逐渐出现困难。神经系统检查显示,这是一位神志清醒、定向力良好的日本女性,无痴呆症状。颅神经未见异常。虽然她没有失语、失用症或失认症,但在诸如使用筷子、书写和扣纽扣等精细手部运动中表现出明显的笨拙。她没有运动无力、共济失调、僵硬或痉挛。深腱反射对称减弱。感觉检查显示皮质感觉丧失,如两点辨别觉、重量觉和实体觉障碍。她的运动笨拙似乎是由皮质感觉丧失引起的。磁共振成像(MRI)显示双侧顶叶有明显的局灶性萎缩,特别是中央后回和相邻的联合区域。最近,已经报道了一些伴有局灶性脑萎缩的神经退行性疾病,如皮质基底节变性、皮克病和额叶型痴呆,然而,我们的患者不符合任何这些已知疾病,其临床特征也与阿尔茨海默病没有明显不同。我们的患者可能是另一个以顶叶皮质为重点的进行性脑变性的例子。