Pharmacological interventions in corticobasal degeneration: a review

ABSTRACT. Corticobasal degeneration (CBD) is a sporadic tauopathy that presents with a varied combination of motor, cognitive, and behavioral features, making its diagnosis difficult. CBD has high morbidity and poor prognosis, with no effective therapy at present. We searched the PubMed/MEDLINE database for articles published from 1990 to 2019, using the keywords “corticobasal degeneration” AND “treatment.” The PRISMA method was adopted. Retrieved articles were characterized as having one of two methodological approaches: (1) studies aimed at primary tauopathy treatment and (2) symptomatic management. Review articles (based on CBD expert groups), case reports, case series, and pilot clinical trials were selected. Few attempts have been made to study drug options and drug efficacy in CBD systematically, and an effective treatment is not yet available. Treatment is symptomatic and based on similarity with other diseases due to the scarcity of studies specifically addressing CBD. CBD seems not to spark interest in more clinical trials for its low prevalence and reliability in clinical diagnosis.摘要：皮质基底节变性（Corticobasal degeneration, CBD）是一类散发性tau蛋白病，其临床表现为运动、认知与行为特征的多样化组合，致使诊断难度较高。该病具有高发病率与不良预后的特点，目前尚无有效治疗手段。本研究以"皮质基底节变性"与"治疗"为检索关键词，检索了1990年至2019年间PubMed/MEDLINE数据库收录的相关文献，并采用PRISMA法开展文献筛选与分析。检索得到的文献可归为两类方法论范畴：（1）针对原发性tau蛋白病治疗的研究；（2）症状管理相关研究。本研究纳入了基于CBD专家小组的综述文章、病例报告、病例系列研究及先导性临床试验。目前，针对CBD系统性开展药物选择与疗效评估的研究极为有限，尚未出现有效的治疗方案。由于针对CBD的专项研究匮乏，当前治疗仅能参考其他相似疾病的诊疗经验，采取对症治疗策略。鉴于CBD患病率较低且临床诊断可靠性不足，该领域尚未引发足够的临床试验研究兴趣。