Neuropsychiatric illness and neuropathological findings in a case of Klinefelter's syndrome

Abstract
SUMMARY A case of Klinefelter's syndrome with XXY karyotype and a dementing neuropsychiatric illness associated with a diffuse degeneration of the deep cerebral white matter and adrenal cortical atrophy is presented. The possible relationships between genetic abnormalities and disordered function and structure of the brain are discussed in the light of the clinical and pathological observations.