Anterior cingulate dysfunction in presenile dementia due to progressive supranuclear palsy.

We report neuropsychological and neuroimaging investigations performed in a patient suffering from presenile onset degenerative dementia and subsequent mild extrapyramidal and pyramidal signs. Early neuropsychological testing revealed frontal lobe dysfunction. Neuroanatomical pictures were not contributive. After four years of evolution, the clinical pattern was consistent with progressive supranuclear palsy. Statistical parametric mapping analysis of functional imaging revealed a highly significant metabolic impairment in the anterior cingulate gyrus, that might be a key feature of subcortico-frontal dementia in progressive supranuclear palsy.