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

G. Rubboli, F. Ronchi, P. Cecchi, S. Meletti, E. Gardella, A. Zaniboni, G. D'Orsi, S. Testoni, A. Bisulli, C. A. Tassinari

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)95-98
Number of pages4
JournalGastroenterology International
Volume12
Issue number2
Publication statusPublished - 1999

Keywords

  • Crigler-Najjar syndrome
  • EEG
  • Kernicterus
  • Liver transplantation

ASJC Scopus subject areas

  • Gastroenterology

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    Rubboli, G., Ronchi, F., Cecchi, P., Meletti, S., Gardella, E., Zaniboni, A., D'Orsi, G., Testoni, S., Bisulli, A., & Tassinari, C. A. (1999). Neurological and neurophysiological follow-up in patients with Crigler-Najjar syndrome type I and possible implications for the timing of liver transplantation. Gastroenterology International, 12(2), 95-98.