Department of Pathology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan Department of Pathology, Ebara Hospital, Tokyo, Japan Department of Pathology, Saitama Medical University, Saitama, Japan.
Department of Neurology, Ebara Hospital, Tokyo, Japan.
J Alzheimers Dis. 2014;40(1):51-5. doi: 10.3233/JAD-131676.
Corticobasal syndrome (CBS) is characterized by lateralized motor disturbance due to levodopa nonresponsive parkinsonism and progressive apraxia. Although CBS is neuropathologically heterogeneous, it remains unclear whether the clinical features of all CBS cases are the same. We report two autopsy cases diagnosed clinically as CBS and pathologically as Alzheimer's disease characterized by lateralized cerebral cortical degeneration and absence of significant nigrostriatial lesions. Cerebral cortical degeneration in both cases was contralateral to their motor disturbances. Thus, nigrostriatial lesions and contralateral cerebral cortical lesions can cause motor disturbances in CBS, necessitating the need for bedside examination in patients with CBS.
皮质基底节综合征（CBS）的特征是由于对左旋多巴反应不佳的帕金森病和进行性失用症导致的侧化运动障碍。尽管 CBS 在神经病理学上存在异质性，但目前尚不清楚所有 CBS 病例的临床特征是否相同。我们报告了两例临床诊断为 CBS 的尸检病例和病理学诊断为阿尔茨海默病的病例，这些病例的特征是侧化大脑皮质变性和没有明显的黑质纹状体病变。这两例病例的大脑皮质变性均与运动障碍相对应。因此，黑质纹状体病变和对侧大脑皮质病变都可能导致 CBS 中的运动障碍，这就需要对 CBS 患者进行床边检查。
