Neurological and neurophysiological follow-up in patients with Crigler-Najjar syndrome type I and possible implications for the timing of liver transplantation

In children and adolescents suffering from Crigler-Najjar syndrome type I, the most worrisome complication is the possible appearance of neurological disorders with aging. We report the results of a neurological and neurophysiological assessment in 6 patients (age range 5-26 years) suffering from Crigler-Najjar syndrome type I. Our results indicate that, in children and adolescents with Crigler-Najjar syndrome type I, neurophysiological tests, such as EEG-polygraphic recordings, are sensitive techniques to demonstrate the involvement of the central nervous system and to evaluate the efficacy of the treatment (i.e., liver transplantation in one of our patients), therefore suggesting a possible usefulness of these neurophysiological techniques for the timing of liver transplantation.