[Neurocutaneous syndrome with a ventricular tumor]

It is reported on a 19 year-old female patient with a mild mental retardation and a neurocutaneous syndrome. A tumour of a lateral ventricle had caused a partial blockade of the Foramen of Monro without symptoms of brain pressure. The patient shows lesions on the skin corresponding to a neurofibromatosis. Neurologically there was a choreatic syndrome, which can occur in transitional forms from neurofibromatosis to tuberous sclerosis (TS) of the brain. Psychologically a psychorganic syndrome, typical for TS, with disturbances of affectivity and motive developed. There was no essential improvement of the neurological and psychical symptoms by surgical removal of the brain tumour nor were there changes in the EEG except for an acceleration of the basic rhythm.