Sawle G V, Brooks D J, Marsden C D, Frackowiak R S
MRC Cyclotron Unit, Hammersmith Hospital, London, UK.
Brain. 1991 Feb;114 ( Pt 1B):541-56. doi: 10.1093/brain/114.1.541.
Corticobasal degeneration presents with an asymmetric akinetic-rigid syndrome, apraxia and combinations of supranuclear gaze palsy, myoclonus, and an alien limb. Six patients aged 59-77 yrs, diagnosed on clinical criteria as having corticobasal degeneration, have been studied with positron emission tomography using tracers of dopamine storage capacity and oxygen metabolism. Striatal 18F-6-fluorodopa uptake was reduced in an asymmetric pattern, caudate and putamen being involved in all cases. Uptake into medial frontal cortex was also impaired. Regional cortical oxygen metabolism was most significantly depressed in the superior and posterior temporal, inferior parietal, and occipital associated cortices. Within the frontal lobe, the hypometabolism was chiefly posterior. This unique combination of regional hypometabolism and disruption of the nigrostriatal system is discussed in relation to the clinical features of the disease and is compared with reported findings in other disorders of cognition and movement.
皮质基底节变性表现为不对称的运动不能-强直综合征、失用症以及核上性凝视麻痹、肌阵挛和异己肢体等症状的组合。对6例年龄在59至77岁之间、根据临床标准诊断为皮质基底节变性的患者，使用多巴胺储存能力和氧代谢示踪剂进行了正电子发射断层扫描研究。纹状体18F-6-氟多巴摄取呈不对称性降低，所有病例的尾状核和壳核均受累。内侧额叶皮质的摄取也受损。颞上回和颞下回、顶下小叶及枕叶联合皮质的区域皮质氧代谢最为显著降低。在额叶内，代谢减低主要位于后部。本文结合该疾病的临床特征讨论了这种区域代谢减低与黑质纹状体系统破坏的独特组合，并与其他认知和运动障碍的报道结果进行了比较。
