Department of Neurology, Mayo Clinic, Rochester, MN, USA.
J Neurol. 2018 Feb;265(2):439-445. doi: 10.1007/s00415-017-8644-3. Epub 2017 Oct 23.
Corticobasal degeneration (CBD) was first described by Rebeiz et al. in 1967, and was called corticodentatonigral degeneration with neuronal achromasia [1]. Since then, our knowledge of the clinical features and underlying tau pathology has grown tremendously. Clinical antemortem diagnosis of CBD pathology remains challenging and has led to the development of revised diagnostic criteria. As various clinical phenotypes may have CBD pathology, accurate prevalence studies are lacking. Recently, pooled prevalence of fronto-temporal lobar degeneration, PSP and CBS was reported as 10.6 per 100,000 [2]. Although rare, CBD is an important disease to understand because it provides a model of a specific proteinopathy (tauopathy) and, therefore, opportunity to study pathophysiology of tauopathies and efficacy of tau-directed therapies. In the past few years, identification of tau specific ligands has advanced neuroimaging of tauopathies such as CBD and progressive supranuclear palsy. However, clinical prediction of CBD pathology remains challenging and an active are of research. In this review, we highlight key emerging issues in CBD pathophysiology, genetics and novel neuroimaging techniques with tau ligands.
皮质基底节变性（CBD）于 1967 年由 Rebeiz 等人首次描述，当时称为皮质纹状体神经元变性伴神经元丧失[1]。从那时起，我们对其临床特征和潜在的 tau 病理学的了解有了巨大的增长。CBD 病理学的临床生前诊断仍然具有挑战性，这导致了修订后的诊断标准的发展。由于各种临床表型可能存在 CBD 病理学，因此缺乏准确的患病率研究。最近，据报道额颞叶变性、 PSP 和 CBS 的总患病率为每 10 万人中有 10.6 人[2]。尽管 CBD 较为罕见，但了解这种疾病很重要，因为它提供了一种特定蛋白病（tau 病）的模型，从而有机会研究 tau 病的病理生理学和 tau 靶向治疗的疗效。在过去几年中，tau 特异性配体的鉴定推动了 CBD 和进行性核上性麻痹等 tau 病的神经影像学研究。然而，CBD 病理学的临床预测仍然具有挑战性，是一个活跃的研究领域。在这篇综述中，我们重点介绍了 CBD 病理生理学、遗传学和新型 tau 配体神经影像学技术方面的新出现问题。
