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K. Kompoliti, MD; C. G. Goetz, MD; B. F. Boeve, MD; D. M. Maraganore, MD; J. E. Ahlskog, PhD, MD; C. D. Marsden, FRCP; K. P. Bhatia, MD; P. E. Greene, MD; S. Przedborski, MD; E. C. Seal, MD; R. S. Burns, MD; R. A. Hauser, MD; L. L. Gauger, BA; S. A. Factor, DO; E. S. Molho, MD; D. E. Riley, MD

Arch Neurol. 1998;55:957-961.

Background  To date, to our knowledge, there is no systematic presentation of treatment outcome in large series of patients clinically diagnosed as having corticobasal degeneration.

Objective  To evaluate the clinical presentation and treatment outcome of patients clinically diagnosed as having corticobasal degeneration.

Subjects  We gathered case patients seen in 8 major movement disorder clinics during the last 5 years who were diagnosed as having corticobasal ganglionic degeneration.

Methods  Using a chart review method, we recorded the clinical presentation, medications used, response to medications, and adverse effects.

Results  A total of 147 case patients were reviewed, 7 were autopsy proven. Parkinsonian features were present in all, other movement disorders 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 55%. Higher cortical dysfunction included dyspraxia (82%), alien limb (42%), cortical sensory loss (33%), and dementia (25%). Ninety-two percent of the case patients received dopaminergic drugs, which resulted in a beneficial effect for 24%. Parkinsonian signs were the elements improving the most and levodopa was the most effective drug. Benzodiazepines, primarily clonazepam, were administered to 47 case patients, which resulted in improvement of myoclonus in 23% and dystonia in 9%. The most frequent disabling adverse effects of drug trials in these case patients were somnolence (n=24), gastrointestinal complaints (n=23), confusion (n=16), dizziness (n=12), hallucinations (n=5), and dry mouth (n=5).

Conclusions  Pharmacological intervention was largely ineffective in the management of corticobasal degeneration, and new treatments are needed for ameliorating the symptoms of this syndrome.

From Rush-Presbyterian-St Luke's Medical Center, Chicago, Ill (Drs Kompoliti and Goetz); Mayo Clinic, Rochester, Minn (Drs Boeve, Maraganore, and Ahlskog); National Hospital for Neurology and Neurosurgery, Queen Square, London, England (Drs Marsden and Bhatia); Columbia University, New York, NY (Drs Greene and Przedborski); Cleveland Clinic Foundation, Cleveland, Ohio (Drs Seal and Burns); University of South Florida, Tampa (Dr Hauser and Ms Gauger); Albany Medical College, Albany, NY (Drs Factor and Molho); and Mt Sinai Medical Center, Cleveland (Dr Riley).

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