Corticobasal degeneration

Corticobasal degeneration (CBC) is an increasingly recognized neurodegenera tive disease with both motor and cognitive dysfunction. The diagnosis is pr obably underestimated because of the heterogeneity of clinical features, ov erlap with symptoms: and pathologic findings of other neurodegenerative dis eases. The most characteristic initial motor symptoms are akinesia, rigidit y, and apraxia. Dystonia and alien limb phenomena are frequently observed. There is often a parkinsonian picture with failure or lack of efficacy of d opaminergic medical therapy. Cognitive decline, prompting the diagnosis of dementia, may be the most common presentation of CBD that is misdiagnosed. Pathology is characterized by an asymmetric frontoparietal neuronal loss an d gliosis with ballooned, achromatic cortical neurons, nigral degeneration, and variable subcortical involvement. Neuroimaging and electrophysiologic studies may help with the diagnosis but are not specific. Treatment is prim arily symptomatic and minimally effective, especially after the first sever al years of symptoms. CBD should be considered in the differential diagnosi s of patients with motor and cognitive dysfunction presenting with cortical and subcortical features. Further studies to elucidate molecular abnormali ties and biological markers associated with CBD are needed to improve clini cal diagnosis and treatment of patients with this disorder.