Solomon G, Labar D, Galbraith R A, Schaefer J, Kappas A
New York Hospital-Cornell University Medical Center, NY 10021.
Electroencephalogr Clin Neurophysiol. 1990 Nov;76(5):473-5. doi: 10.1016/0013-4694(90)90100-x.
We report neurophysiological abnormalities in two adolescents with type I Crigler-Najjar syndrome, an autosomal recessive disorder characterized by severe unconjugated hyperbilirubinemia. Electroencephalograms (EEGs) demonstrated frequent generalized single and polyspikes, and background slowing. Normal pattern reversal evoked responses (PRVERs) and normal central brain-stem auditory evoked responses (BAERs) were recorded in both patients. These findings differ from the EEG triphasic wave pattern seen in the EEG in hepatic encephalopathy and the central BAER abnormalities seen in many infants with kernicterus. Thus these findings most likely result from complex multifactorial processes and are not simply the result of the hyperbilirubinemia which is common to all 3 conditions.
我们报告了两名患有I型克里格勒-纳贾尔综合征的青少年的神经生理异常情况，这是一种常染色体隐性疾病，其特征为严重的非结合性高胆红素血症。脑电图（EEG）显示频繁出现全身性单棘波和多棘波，以及背景减慢。两名患者均记录到正常的模式反转诱发电位（PRVER）和正常的中枢脑干听觉诱发电位（BAER）。这些发现不同于肝性脑病脑电图中所见的三相波模式以及许多患有核黄疸的婴儿中所见的中枢BAER异常。因此，这些发现很可能是由复杂的多因素过程导致的，而不仅仅是这三种情况所共有的高胆红素血症的结果。
