Wadia Pettarusp M, Lang Anthony E
Movement Disorders Centre, Toronto Western Hospital, University of Toronto, Canada.
Parkinsonism Relat Disord. 2007;13 Suppl 3:S336-40. doi: 10.1016/S1353-8020(08)70027-0.
Corticobasal degeneration (CBD) has characteristic neuropathological features, but is a clinically heterogeneous disorder. It can present with various clinical syndromes. The corticobasal syndrome (CBS) is the best recognized and over the course of the illness may be the most common manifestation. Dementia, progressive non-fluent aphasia, speech apraxia, progressive supranuclear palsy (PSP)-like syndrome and posterior cortical atrophy syndrome are other presentations of CBD. The CBS is not specific for the pathology of CBD. Patients presenting as CBS have CBD as the underlying pathology in about 55% of the cases, PSP pathology in 20%, Pick's disease in 7%, and non tau pathology in the remaining. CBS is a sensitive clinical phenotype for tau pathology. Patients with CBS could potentially be candidates for therapies aiming to modify tau biochemistry.
皮质基底节变性(CBD)具有特征性神经病理学特征,但却是一种临床异质性疾病。它可表现为多种临床综合征。皮质基底节综合征(CBS)是最广为人知的,在病程中可能是最常见的表现。痴呆、进行性非流利性失语、言语失用、进行性核上性麻痹(PSP)样综合征和后部皮质萎缩综合征是CBD的其他表现形式。CBS并非CBD病理学所特有。表现为CBS的患者中,约55%的潜在病理学为CBD,20%为PSP病理学,7%为皮克病,其余为非tau病理学。CBS是tau病理学的一种敏感临床表型。CBS患者可能是旨在改变tau生物化学疗法的潜在候选者。