Jankowicz E, Drozdowski W, Halicka D
Kliniki Neurologii AM w Białymstoku.
Neurol Neurochir Pol. 2000 Jan-Feb;34(1):121-31.
Cortico-basal degeneration (CBD) or cortico-basal ganglionic degeneration is a condition characterised by selective cortical atrophy of parietal and in a lesser extent, frontal lobe associated with dysfunction of the basal ganglia. The clinical symptoms of CBD, predominantly extrapyramidal signs (bradykinesia and rigidity) and apraxia, affect often only one body side in the onset phase, with the left one being more frequent. Neuropathological studies reveal neuronal loss, gliosis, and achromasia chiefly in frontal and parietal cortex, as well as in basal ganglia and substantia nigra. Functional investigations, such as SPECT, disclose similar distribution of abnormalities (hypometabolism). The aetiology and causative treatment of CBD are unknown. The authors highlight the diagnostic difficulties in CBD including a necessity of a prolonged patient's observation in order to ascertain the differential diagnosis of other neurodegenerative disorders, in particular progressive supranuclear palsy, Alzheimer's disease and Parkinson's disease.
皮质基底节变性(CBD)或皮质基底节神经节变性是一种以顶叶选择性皮质萎缩为特征的疾病,额叶萎缩程度较轻,伴有基底节功能障碍。CBD的临床症状主要为锥体外系症状(运动迟缓及强直)和失用症,在发病初期通常仅影响身体一侧,左侧更为常见。神经病理学研究显示,神经元丢失、胶质细胞增生和染色质溶解主要见于额叶和顶叶皮质,以及基底节和黑质。功能检查,如单光子发射计算机断层扫描(SPECT),显示异常(代谢减低)分布相似。CBD的病因及针对性治疗方法尚不清楚。作者强调了CBD的诊断困难,包括需要对患者进行长期观察,以明确与其他神经退行性疾病的鉴别诊断,特别是进行性核上性麻痹、阿尔茨海默病和帕金森病。