Yamamoto Ryoko, Iseki Eizo, Murayama Norio, Minegishi Michiko, Kimura Michihiro, Eto Ko, Arai Heii, Ohbu Sadayoshi, Hatanaka Daisuke, Hino Hiroaki, Fujisawa Koshiro
Department of Psychiatry, Juntendo Tokyo Koto Geriatric Medical Center, Juntendo University School of Medicine, Tokyo, Japan.
Brain Nerve. 2007 Mar;59(3):263-9.
Dementia with motor neuron disease (D-MND) is characterized clinically by frontal and neurological signs, and pathologically by localized atrophy of the fronto-temporal lobes and neuronal ubiquitin(Ub)-positive inclusions. In this study, we compared the clinico-pathological findings of two patients with D-MND. Case 1 (55-year-old male): At the age of 51, he developed personality change and disinhibition, lacking neurological signs. Brain MRI exhibited localized atrophy of the frontal lobes. At the age of 54, he showed dysphagia and died after a disease duration of 4 years. Neuropathologically, the cerebrum showed localized atrophy of the dorsal area of the frontal lobes. The atrophied cerebral cortex demonstrated moderate neuronal loss with spongy change and gliosis in the superficial layers. The brainstem and spinal cord revealed moderate neuronal loss in the substantia nigra, severe neuronal loss with Bunina bodies in the hypoglossal nucleus, and moderate neuronal loss in the cervical anterior horn. There were some Ub-positive neuronal inclusions in the atrophied cortex and many in the dentate gyrus. Case 2 (68-year-old female): At the age of 64, she developed personality change, and then gait disturbance and dysarthria. Brain MRI exhibited localized atrophy of the fronto-temporal lobes. At the age of 67, she showed dysphagia with Babinski signs and died after a disease duration of 4 years. Neuropathologically, the cerebrum showed localized atrophy of the basal area of the temporal lobes, especially on the right side. The atrophied cerebral cortex demonstrated moderated neuronal loss with spongy change and gliosis in the superficial layers. The pre-central cortex revealed severe loss of Betz cells. The brainstem and spinal cord showed mild neuronal loss without Bunina bodies in the hypoglossal nucleus and cervical anterior horn, accompanied by severe degeneration of the bilateral pyramidal tracts. There were many Ub-positive neuronal inclusions with a few neurites in the atrophied cortex and some in the dentate gyrus. Cases 1 and 2 were clinically diagnosed as Pick's disease (PiD) and D-MND, respectively, although pathological diagnoses were both D-MND. Case 1 showed neuropathological findings typical to D-MND, whereas case 2 showed neuropathological findings common to atypical Pick's disease (aPiD). D-MND and aPiD are should be clinico-pathologically differentiated, although they are included in the frontotemporal lobar degeneration with motor neuron disease-type inclusions.
运动神经元病伴发的痴呆（D-MND）临床特征为额叶及神经学体征，病理特征为额颞叶局限性萎缩及神经元泛素（Ub）阳性包涵体。在本研究中，我们比较了2例D-MND患者的临床病理表现。病例1（55岁男性）：51岁时出现人格改变及行为脱抑制，无神经学体征。脑部MRI显示额叶局限性萎缩。54岁时出现吞咽困难，病程4年后死亡。神经病理学检查显示，大脑额叶背侧区域局限性萎缩。萎缩的大脑皮质浅层可见中度神经元丢失，伴有海绵状改变及胶质细胞增生。脑干和脊髓可见黑质中度神经元丢失，舌下神经核重度神经元丢失并伴有布尼亚小体，颈前角中度神经元丢失。萎缩皮质中有一些Ub阳性神经元包涵体，齿状回中有许多。病例2（68岁女性）：64岁时出现人格改变，随后出现步态障碍和构音障碍。脑部MRI显示额颞叶局限性萎缩。67岁时出现吞咽困难伴巴宾斯基征，病程4年后死亡。神经病理学检查显示，大脑颞叶底部区域局限性萎缩，右侧尤为明显。萎缩的大脑皮质浅层可见中度神经元丢失，伴有海绵状改变及胶质细胞增生。中央前回皮质可见贝茨细胞严重丢失。脑干和脊髓显示舌下神经核和颈前角轻度神经元丢失，无布尼亚小体，双侧锥体束严重变性。萎缩皮质中有许多Ub阳性神经元包涵体，伴有少量神经突，齿状回中有一些。病例1和病例2临床分别诊断为匹克病（PiD）和D-MND，尽管病理诊断均为D-MND。病例1表现出D-MND典型的神经病理学表现，而病例2表现出非典型匹克病（aPiD）常见的神经病理学表现。D-MND和aPiD应在临床病理上进行鉴别，尽管它们都属于伴有运动神经元病型包涵体的额颞叶变性。
