Progressive Supranuclear Palsy and Corticobasal Syndrome

This article describes the approach to diagnosis and management of patients with progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). The clinical criteria for both PSP and CBS have expanded to include the broad phenotypic spectrum of these disorders. There is significant overlap among features of PSP and CBS, both clinically and neuropathologically, and early recognition of these disorders remains challenging. Use of novel clinical criteria increases the sensitivity and accuracy of clinician diagnosis. Advances in brain imaging techniques, such as MRI and positron emission tomography (PET), as well as fluid biomarkers, may help in diagnosis. For CBS, there is increasing recognition of varied neuropathology and differences in tau filaments, which may differentiate the disorder from PSP and other tauopathies. Careful attention to historical presentation, clinical features, and evolving diagnostic criteria and brain imaging techniques will help the clinician recognize the various PSP and CBS phenotypes. Early recognition is critical to provide appropriate treatment and supportive care, which ideally should involve a multidisciplinary team of allied health professionals, inclusive of the patient and the caregiver.