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Visual evoked potentials (VEP) and brain stem acoustic evoked potentials (BAEP) were studied in eight children with tuberous sclerosis (four boys and four girls, average age 4.5 years) comparing them with a control group of 21 healthy children (ten boys and 11 girls). The patients had an increased average latency of VEP (IV component or P2; p < .01694) and BAEP (right V wave = p < .001145; left V wave = p < 013792; right I-V interpeak = p < .000543; left I-V interpeak ? p < .009854). Impaired responses were common in both VEP (80%) and BAEP (85%). In addition, altered BAEP were also present in patients without subtentorial structural abnormalities. Evoked potentials serve as a sensitive test for early diagnosis of tuberous sclerosis.

Visual evoked potentials (VEP) and brain stem acoustic evoked potentials (BAEP) were studied in 19 children with neurofibromatosis (13 males and six females aged from seven months to 17 years; average age 6.3 years) comparing them with those of a control group of 43 healthy children (25 males and 18 females). The patients had an increased average VEP latency (III wave = p < .01559; IV wave = p < .00001) and I-V interpeak of BAEP (right I-V interpeak = p < .04083; left I-V interpeak ? p < .00852). Impaired responses were relatively common in both VEP (40%) and BAEP (35%), with a moderate but not constant relation to neuroradiological findings. Altered potentials were also present in patients without structural abnormalities. Evoked potentials serve as a sensitive test for early diagnosis of neurofibromatosis.

Evoked potentials (EP) allow the assessment of the functional integrity and degree of maturation of the main pathways of sensory afference (visual, acoustic and somaesthetic). For this reason, EP can be used in the study of patients with tuberous sclerosis. In clinical practice, EP serve to give information for early diagnosis and a better prognostic assessment of the individual patient. A recent analysis of visual evoked potentials (VEP) and brain stem acoustic evoked potentiials (BAEP) in children with tuberous sclerosis disclosed an increased average latency of some components of both VEP (component IV or P2) and BAEP (V wave and I-V interpeak). Impaired responses were present in 80% of VEP and 85% of BAEP. In addition, impaired BAEP were also present in patients without subtentorial structural abnormalities. EP serve as a useful instrument for early diagnosis of tuberous sclerosis. Given the anatomofunctional differences of the systems stimulated and the preferential location of lesions in certain areas, the various different EP have varying degrees of sensitivity in disclosing abnormalities: visual and brain stem acoustic EP are more sensitive than somatosensory EP.