Bluett Brent, Pantelyat Alexander Y, Litvan Irene, Ali Farwa, Apetauerova Diana, Bega Danny, Bloom Lisa, Bower James, Boxer Adam L, Dale Marian L, Dhall Rohit, Duquette Antoine, Fernandez Hubert H, Fleisher Jori E, Grossman Murray, Howell Michael, Kerwin Diana R, Leegwater-Kim Julie, Lepage Christiane, Ljubenkov Peter Alexander, Mancini Martina, McFarland Nikolaus R, Moretti Paolo, Myrick Erica, Patel Pritika, Plummer Laura S, Rodriguez-Porcel Federico, Rojas Julio, Sidiropoulos Christos, Sklerov Miriam, Sokol Leonard L, Tuite Paul J, VandeVrede Lawren, Wilhelm Jennifer, Wills Anne-Marie A, Xie Tao, Golbe Lawrence I
Neurology, Pacific Central Coast Health Center, Dignity Health, San Luis Obispo, CA, United States.
Neurology, Stanford University, Stanford, CA, United States.
Front Neurol. 2021 Jul 1;12:694872. doi: 10.3389/fneur.2021.694872. eCollection 2021.
Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS; the most common phenotype of corticobasal degeneration) are tauopathies with a relentless course, usually starting in the mid-60s and leading to death after an average of 7 years. There is as yet no specific or disease-modifying treatment. Clinical deficits in PSP are numerous, involve the entire neuraxis, and present as several discrete phenotypes. They center on rigidity, bradykinesia, postural instability, gait freezing, supranuclear ocular motor impairment, dysarthria, dysphagia, incontinence, sleep disorders, frontal cognitive dysfunction, and a variety of behavioral changes. CBS presents with prominent and usually asymmetric dystonia, apraxia, myoclonus, pyramidal signs, and cortical sensory loss. The symptoms and deficits of PSP and CBS are amenable to a variety of treatment strategies but most physicians, including many neurologists, are reluctant to care for patients with these conditions because of unfamiliarity with their multiplicity of interacting symptoms and deficits. CurePSP, the organization devoted to support, research, and education for PSP and CBS, created its CurePSP Centers of Care network in North America in 2017 to improve patient access to clinical expertise and develop collaborations. The directors of the 25 centers have created this consensus document outlining best practices in the management of PSP and CBS. They formed a writing committee for each of 12 sub-topics. A 4-member Steering Committee collated and edited the contributions. The result was returned to the entire cohort of authors for further comments, which were considered for incorporation by the Steering Committee. The authors hope that this publication will serve as a convenient guide for all clinicians caring for patients with PSP and CBS and that it will improve care for patients with these devastating but manageable disorders.
进行性核上性麻痹（PSP）和皮质基底节综合征（CBS；皮质基底节变性最常见的表型）是具有无情病程的tau蛋白病，通常始于60岁左右，平均7年后导致死亡。目前尚无特异性或疾病修饰治疗方法。PSP的临床缺陷众多，累及整个神经轴，表现为几种不同的表型。它们主要集中在僵硬、运动迟缓、姿势不稳、步态冻结、核上性眼球运动障碍、构音障碍、吞咽困难、尿失禁、睡眠障碍、额叶认知功能障碍以及各种行为改变。CBS表现为突出且通常不对称的肌张力障碍、失用症、肌阵挛、锥体束征和皮质感觉丧失。PSP和CBS的症状和缺陷适合多种治疗策略，但大多数医生，包括许多神经科医生，由于不熟悉这些疾病相互作用的多种症状和缺陷，不愿照料患有这些疾病的患者。致力于为PSP和CBS提供支持、研究和教育的CurePSP组织于2017年在北美创建了其CurePSP护理中心网络，以改善患者获得临床专业知识的机会并开展合作。25个中心的主任制定了这份共识文件，概述了PSP和CBS管理的最佳实践。他们为12个分主题分别成立了一个写作委员会。一个由4名成员组成的指导委员会整理并编辑了这些稿件。结果返回给所有作者以供进一步评论，指导委员会考虑将这些评论纳入其中。作者希望本出版物能为所有照料PSP和CBS患者的临床医生提供便利指南，并改善对这些具有毁灭性但可控制疾病患者的护理。
