TY - JOUR
T1 - A neurophysiological study in children and adolescents with Crigler-Najjar syndrome type I
AU - Rubboli, G.
AU - Ronchi, F.
AU - Cecchi, P.
AU - Rizzi, R.
AU - Gardella, Elena
AU - Meletti, S.
AU - Zaniboni, Anna
AU - Volpi, Lilia
AU - Tassinari, C. A.
PY - 1997/10
Y1 - 1997/10
N2 - 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.
AB - 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.
KW - Bilirubin neurotoxicity
KW - Crigler-Najjar syndrome
KW - Electroencephalography
KW - Kernicterus
UR - http://www.scopus.com/inward/record.url?scp=0030700772&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0030700772&partnerID=8YFLogxK
U2 - 10.1055/s-2007-973715
DO - 10.1055/s-2007-973715
M3 - Article
C2 - 9413009
AN - SCOPUS:0030700772
VL - 28
SP - 281
EP - 286
JO - Neuropediatrics
JF - Neuropediatrics
SN - 0174-304X
IS - 5
ER -