Clinical Presentation and Pharmacological Therapy in Corticobasal Degeneration

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Abstract
IN 1967 and 1968, Rebeiz et al1,2 described the clinical and pathologic findings in 3 patients who presented with slowness, awkwardness of volitional movement, and superimposed involuntary movements. All 3 were asymmetrically involved at onset with relatively preserved intellect until death. The duration of the illness until death was 6 to 8 years. Pathologic evaluation showed frontoparietal atrophy, asymmetrical in 2 cases, characterized histologically by neuronal loss, gliosis, and a distinctive neuronal change consisting of swelling of the cell body and resistance to staining methods (achromasia). They called the syndrome cortico-dentato-nigral degeneration with neuronal achromasia.