The behavioral, neuropsychological and single photon emission computerized tomography characteristics of 5 patients with progressive degeneration of the right hemisphere are described. In all, the brain regions with greatest involvement were right-frontal and temporal. Psychosis, compulsions and behavioral disinhibition were the dominant, and often first, symptoms. Affect was flattened and the patients seemed distant and remote. Neuropsychological testing did not reveal a consistent pattern that helped localize the abnormality to the right frontotemporal region. These patients contrast dramatically to those with left frontotemporal degeneration in whom behavior and psychiatric status is often normal. This study suggests that the right hemisphere may be primary for the control of social conduct.

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