Corticobasal degeneration (CBGD) is also known as or subsumes Cortical-basal ganglionic degeneration, Corticonigral degeneration, and Cortico-dentato-nigral degeneration with neuronal achromasia. -ed.
Corticobasal degeneration is one of the Parkinsonism-plus syndromes that can mimic Parkinson disease but is distinct because of the added features of apraxia, dysphasia, cortical sensory signs, unusual dystonic postures, and autonomous movements--including the “alien limb” sign. In this clinical article, Dr. Samuel Frank, Associate Professor of Neurology at Boston University, reviews the clinical features of the disease as well as the pathological findings. New potential genetic etiologies of the disease are presented. Although treatment remains symptomatic, accurate diagnosis can help prepare families and help clinicians better understand and treat this progressive, fatal disorder.