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

A neurophysiological study in children and adolescents with Crigler-Najjar syndrome type I. Research Abstract Details 

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A neurophysiological study in children and adolescents with Crigler-Najjar syndrome type I. Abstract Text:

G Rubboli,F Ronchi,P Cecchi,R Rizzi,E Gardella,S Meletti,A Zaniboni,L Volpi,C A Tassinari,G Rubboli,F Ronchi,P Cecchi,R Rizzi,E Gardella,S Meletti,A Zaniboni,L Volpi,C A Tassinari,

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 performed 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.

A neurophysiological study in children and adolescents with Crigler-Najjar syndrome type I. Publishing Authors By Initials

G Rubboli,F Ronchi,P Cecchi,R Rizzi,E Gardella,S Meletti,A Zaniboni,L Volpi,CA Tassinari,G Rubboli,F Ronchi,P Cecchi,R Rizzi,E Gardella,S Meletti,A Zaniboni,L Volpi,CA Tassinari,

For similar diagnosis: prognosis research abstracts see: diagnosis: prognosis research

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A neurophysiological study in children and adolescents with Crigler-Najjar syndrome type I. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Neuropediatrics

VOLUME: 28

Page Numbers: 281-6

Journal Abbreviation: Neuropediatrics

ISSN: 0174-304X

DAY: 16

MONTH: Oct

YEAR: 1997

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

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LANGUAGE: eng

NlmUniqueID: 8101187

A neurophysiological study in children and adolescents with Crigler-Najjar syndrome type I. Keywords Mesh Terms:

KEYWORDS: Prognosis

MESH TERMS: physiopathology

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Grant and Affiliation Information for A neurophysiological study in children and adolescents with Crigler-Najjar syndrome type I.

AFFILIATION: Department of Neurology, Bellaria Hospital, University of Bologna, Italy.

Country: GERMANY

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MEDLINETA: Neuropediatrics

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