PSYCHOPATHOLOGY IN STURGE-WEBER SYNDROME

Sturge-Weber syndrome is a rare congenital disease which can impair a child's brain functions and psychosocial development. Its psychiatric aspects have been ignored in the literature. Two male Chinese patients who presented with paranoid disorders and one female Chinese patient who presented with depressive pseudo-dementia are described. Biological and psychosocial aspects of pathogenesis are discussed, especially in the light of a ''shame-humiliation model'' of paranoid processes, since shame and humiliation are engrained in Chinese culture. These case descriptions suggest an overlooked association of Sturge-Weber syndrome with psychopathology and have important implications for the prevention and treatment of such potentially crippling psychiatric complications in subjects with this syndrome as well as other visible physical handicaps.