CLINICAL PRESENTATION AND PHARMACOLOGICAL THERAPY IN CORTICOBASAL DEGENERATION

Background: To date, to our knowledge, there is no systematic presenta tion of treatment outcome in large series of patients clinically diagn osed as having corticobasal degeneration. Objective: To evaluate the c linical presentation and treatment outcome of patients clinically diag nosed as having corticobasal degeneration. Subjects: We gathered case patients seen in 8 major movement disorder clinics during the last 5 y ears who were diagnosed as having corticobasal ganglionic degeneration . Methods: Using a chart review method, we recorded the clinical prese ntation, medications used, response to medications, and adverse effect s. Results: A total of 147 case patients were reviewed, 7 were autopsy proven. Parkinsonian features were present in all, other movement dis orders in 89%, and higher cortical dysfunction in 93%. The most common parkinsonian sign was rigidity (92%), followed by bradykinesia (80%), gait disorder (80%), and tremor (55%). Other movement disorders were dystonia in 71% and myoclonus in 5%. Higher cortical dysfunction inclu ded dyspraxia (82%), alien limb (42%), cortical sensory loss (33%), an d dementia (25%). Ninety-two percent of the case patients received dop aminergic drugs, which resulted in a beneficial effect for 24%. Parkin sonian signs were the elements improving the most and levodopa was the most effective drug. Benzodiazepines, primarily clonazepam, were admi nistered to 47 case patients, which resulted in improvement of myoclon us in 23% and dystonia in 9%. The most frequent disabling adverse effe cts of drug trials in these case patients were somnolence (n = 24), ga strointestinal complaints (n = 23), confusion (n = 16), dizziness (n = 12), hallucinations (n = 5), and dry mouth (n = 5). Conclusions: Phar macological intervention was largely ineffective in the management of corticobasal degeneration, and new treatments are needed for ameliorat ing the symptoms of this syndrome.