ABSTRACT
Corticobasal degeneration (CBG) is an increasingly recognized neurodegenerative disease
with both motor and cognitive dysfunction. The diagnosis is probably underestimated
because of the heterogeneity of clinical features, overlap with symptoms, and pathologic
findings of other neurodegenerative diseases. The most characteristic initial motor
symptoms are akinesia, rigidity, and apraxia. Dystonia and alien limb phenomena are
frequently observed. There is often a parkinsonian picture with failure or lack of
efficacy of dopaminergic 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 and 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 primarily symptomatic and minimally effective, especially after
the first several years of symptoms. CBD should be considered in the differential
diagnosis of patients with motor and cognitive dysfunction presenting with cortical
and subcortical features. Further studies to elucidate molecular abnormalities and
biological markers associated with CBD are needed to improve clinical diagnosis and
treatment of patients with this disorder.
KEYWORD
Corticobasal degeneration - atypical parkinsonism - neurodegenerative disease
