Document Detail

Clinical utility of electrophysiological evaluation in Crigler-Najjar syndrome.
MedLine Citation:
PMID:  18058623     Owner:  NLM     Status:  MEDLINE    
We evaluated the neurological and neurophysiological features in ten patients with genetically characterized Crigler-Najjar (CN) syndrome: four with typical type I CN had undergone orthotopic liver transplantation (OLT); six had type II CN, and three of them developed severe hyperbilirubinemia with a limited response to phenobarbital leading to an intermediate phenotype I/II. Clinical neurological and multimodal electrophysiological evaluations [electroencephalogram (EEG), visual (VEPs), motor (MEPs) and brainstem auditory (BAEPs) evoked potentials] were performed. Neurological examinations showed mild hand tremor in four patients (one pre-OLT and one post-OLT type I, two type I/II). EEG revealed high voltage paroxysmal discharges in four patients (three type I/II, and one type I with a marked improvement after OLT). VEPs showed P100 wave increased latency in five patients (three type I, and two type I/II considered for OLT evaluation). MEPs showed prolonged central motor conduction time in five patients (two type I; one type I/II; two type II). Only EEG and VEPs findings showed a correlation with high bilirubin levels. BAEPs were normal. In conclusion, VEPs and EEG contribute to identify and monitor bilirubin neurotoxic effects, and may play a decisional role in some cases of severe hyperbilirubinemia without overt neurologic damage.
A Perretti; G Crispino; L Marcantonio; S Lenta; M Caropreso; F Manganelli; S Scianguetta; R Iorio; A Iolascon; P Vajro
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Publication Detail:
Type:  Evaluation Studies; Journal Article    
Journal Detail:
Title:  Neuropediatrics     Volume:  38     ISSN:  0174-304X     ISO Abbreviation:  Neuropediatrics     Publication Date:  2007 Aug 
Date Detail:
Created Date:  2007-12-06     Completed Date:  2008-03-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8101187     Medline TA:  Neuropediatrics     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  173-8     Citation Subset:  IM    
Department of Neurological Sciences, University of Naples Federico II, Naples, Italy.
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MeSH Terms
Age Factors
Crigler-Najjar Syndrome / diagnosis,  physiopathology*,  surgery
Evoked Potentials / physiology*
Follow-Up Studies
Liver Transplantation / methods
Neurologic Examination
Physical Stimulation / methods
Reaction Time

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