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1968年,Rebeiz首次报道了3例皮质基底节变性(Cortlcobasal degeneration,CBD;又称为Corit-cal-basal ganglionic degeneration,CBGD)患者的临床和病理特征。以后许多学者特别是最近几年对CBD进行了深入研究。CBD是一种主要累及额顶叶大脑皮质和基底节区变性的疾病,临床上以非对称性运动障碍、皮质功能受损为特征;在疾病的早期,常被误诊为Parkinson氏病或其它变性疾病。为提高CBD的诊断率,现将CBD的临床特征、诊断标准和实验室检查特点综述如下。 一、临床特征
In 1968, Rebeiz first reported the clinical and pathological features of 3 patients with Cortlcobasal degeneration (CBD; also known as Corit-cal-basal ganglionic degeneration, CBGD). Since then, many scholars have conducted in-depth studies on the CBD, especially in recent years. CBD is a disease that mainly affects the parietal cortex and basal ganglia degeneration. It is characterized clinically by asymmetric dyskinesia and impaired cortical function. Early in the disease, it is often misdiagnosed as Parkinson’s disease or other degeneration disease. In order to improve the diagnostic rate of CBD, the clinical features, diagnostic criteria and laboratory examination features of CBD are summarized as follows. First, the clinical features