Utility of multimodal evoked potentials study in neurofibromatosis type 1 of childhood.

A group of 21 children affected by neurofibromatosis type 1 has been investigated with the aim of studying multimodal (visual, brainstem auditory, and somatosensory) evoked potentials and their correlations with neurologic, electroencephalographic, and cranial magnetic resonance imaging. In the present series, cranial magnetic resonance imaging and evoked potentials were the most frequently abnormal instrumental tests. In approximately two thirds of the cases at least one of the evoked potentials (particularly visual and auditory evoked potentials) was compromised, always without clinical signs of related sensory (visual, auditory, and somatosensory) pathway pathology and sometimes in the absence of magnetic resonance imaging signs of central nervous system involvement. This study indicates that in patients with neurofibromatosis type 1, multimodal evoked potentials are useful and should be part of the diagnostic protocol of encephalic lesions together with magnetic resonance imaging. The use of both methods could aid in early detection of central nervous system dysfunction in both the initial evaluation of disease and its follow-up.