Corticobasal syndrome: a practical guide. Issue 4 (23rd July 2021)

• Record Type: Journal Article
• Authors: Wilson, Duncan; Le Heron, Campbell; Anderson, Tim
• Is Part Of: Practical neurology. Volume 21:Issue 4(2021)
• Page Start: 276
• Page End: 283
• Publication Date: 2021-07-23
• Subjects: alien hand -- corticobasal degeneration -- apraxia -- aphasia -- myoclonus; Neurology -- Periodicals; 616.8005
• DOI: 10.1136/practneurol-2020-002835
• Languages: English
• ISSNs: 1474-7758

Abstract

Abstract : Corticobasal syndrome is a disorder of movement, cognition and behaviour with several possible underlying pathologies, including corticobasal degeneration. It presents insidiously and is slowly progressive. Clinicians should consider the diagnosis in people presenting with any combination of extrapyramidal features (with poor response to levodopa), apraxia or other parietal signs, aphasia and alien-limb phenomena. Neuroimaging showing asymmetrical perirolandic cortical changes supports the diagnosis, while advanced neuroimaging may give insight into the underlying pathology. Identifying corticobasal syndrome carries some management implications (especially if protein-based treatments arise in the future) and prognostic significance. Its treatment is largely symptomatic and is best undertaken within a multidisciplinary setting, including a neurologist, physiotherapist, occupational therapist, speech language therapist, psychiatrist and, ultimately, a palliative care clinician. Corticobasal syndrome can be a confusing entity for neurologists, not least because it has over time evolved from being considered predominantly as a movement disorder to a condition spanning a wide range of cognitive and motor manifestations. In this practical review, we attempt to disentangle this syndrome and provide clarity around diagnosis, its underlying pathological substrates, key clinical features and potential treatments.