Neurologic symptoms of basal cell nevus syndrome

The neurological symptomatology of the basal cell nevus syndrome (Gorlin-Goltz syndrome) is described based on a personal observation and previously published cases. The case presented here, with dominant cerebellar symptomatology, optic atrophy and pyramidal signs, is discussed pathophysiologically either as a primary central nervous manifestation of the basic disturbance or as a secondary cerebral paraneoplastic process.