A neurophysiological study in children and adolescents with Crigler-Najjar syndrome type I

G. Rubboli, F. Ronchi, P. Cecchi, R. Rizzi, Elena Gardella, S. Meletti, Anna Zaniboni, Lilia Volpi, C. A. Tassinari

Research output: Contribution to journalArticlepeer-review

Abstract

We studied the neurophysiological features of five patients (age range: 4-20 years) suffering from Crigler-Najjar syndrome type I (CNsI) by means of multimodal (brainstem, somatosensory, motor) evoked potentials and periodic EEG-polygraphic recordings (follow-up: 3 months-4.5 years). Two patients presented with neurological disturbances, consisting mainly of mental slowing, motor impairment and seizures. Both of them presented an abnormal EEG, characterized by slowing of background activity associated with paroxysmal discharges. Liver transplantation was performend in one of these two patients and was followed by improvement of both the neurological picture and EEG activity. In a third patient, clinically normal, after two years of follow-up, the EEG started to show paroxysmal activity during sleep or when evoked by intermittent photic stimulation. In these three patients, multimodal evoked potentials were unremarkable. The remaining two younger subjects did not show any clinical or EEG abnormality. Our findings suggest that, whereas in newborns and infants evoked potentials have been demonstrated as reliable techniques to monitor bilirubin neurotoxicity, in children and adolescents with CNsI, EEG seems to be more sensitive in evaluating patients for neurological damage and effectiveness of therapeutic strategies adopted.

Original languageEnglish
Pages (from-to)281-286
Number of pages6
JournalNeuropediatrics
Volume28
Issue number5
DOIs
Publication statusPublished - Oct 1997

Keywords

  • Bilirubin neurotoxicity
  • Crigler-Najjar syndrome
  • Electroencephalography
  • Kernicterus

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Fingerprint Dive into the research topics of 'A neurophysiological study in children and adolescents with Crigler-Najjar syndrome type I'. Together they form a unique fingerprint.

Cite this