DePold Hohler Anna, Ransom Bruce R, Chun Michael R, Tröster Alexander I, Samii Ali
Department of Neurology, Madigan Army Medical Center, Tacoma, WA, USA.
Parkinsonism Relat Disord. 2003 Oct;10(1):47-50. doi: 10.1016/s1353-8020(03)00046-4.
Corticobasal degeneration (CBD) is a movement disorder characterized by early apraxia and asymmetric parkinsonism that responds poorly to anti-Parkinson medications. There are clinical manifestations reflecting dysfunction in both the cerebral cortex and the basal ganglia. Patients typically present between the sixth and seventh decades. Previously, the youngest clinically diagnosed individual was 40 years old. Here we describe a 34-year-old woman who meets the clinical diagnostic criteria for CBD with onset of symptoms at age 28. In this patient, the first symptom was an 'uncooperative' right hand. This was soon followed by right hand dystonia. Symptoms progressed rapidly, and she developed generalized bradykinesia, rigidity, and corticospinal tract signs with preservation of the initial asymmetry. Her symptoms did not respond to a daily dose of 1200 mg of immediate release levodopa. Extensive laboratory workup and brain imaging were normal. Neuropsychological evaluation revealed mild deficits consistent with frontal-subcortical dysfunction. The chronic, progressive course, asymmetric limb rigidity, apraxia, focal dystonia, and lack of response to levodopa suggest CBD. To our knowledge, this is the youngest reported case of clinically probable CBD.
皮质基底节变性（CBD）是一种运动障碍性疾病，其特征为早期失用症和不对称性帕金森综合征，对抗帕金森药物反应不佳。存在反映大脑皮质和基底节功能障碍的临床表现。患者通常在60至70岁之间发病。此前，临床上诊断出的最年轻患者为40岁。在此，我们描述一名34岁女性，她在28岁时出现症状，符合CBD的临床诊断标准。该患者的首发症状是右手“不配合”。随后不久出现右手肌张力障碍。症状迅速进展，她出现了全身性运动迟缓、僵硬和皮质脊髓束征，且最初的不对称性依然存在。她的症状对每日1200毫克速释左旋多巴剂量无反应。广泛的实验室检查和脑部影像学检查均正常。神经心理学评估显示存在与额叶 - 皮质下功能障碍相符的轻度缺陷。慢性、进行性病程、不对称肢体僵硬、失用症、局灶性肌张力障碍以及对左旋多巴无反应提示为CBD。据我们所知，这是报告的临床上可能为CBD的最年轻病例。
