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Neurological Complications of Kernicterus

Published online by Cambridge University Press:  02 December 2014

Suad F. AlOtaibi ,
Susan Blaser  and
Daune L. MacGregor
Suad F. AlOtaibi
Affiliation:
Department of Paediatrics, Division of Neurology, The Hospital for Sick Children, Toronto, ON, Canada
Susan Blaser
Affiliation:
Department of Paediatrics, Division of Neurology, The Hospital for Sick Children, Toronto, ON, Canada
Daune L. MacGregor*
Affiliation:
Department of Paediatrics, Division of Neurology, The Hospital for Sick Children, Toronto, ON, Canada
*
Department of Paediatrics, Division of Neurology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada.
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Abstract:

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Objective and Background:

Prevention of bilirubin encephalopathy relies on the detection of newborns who are at risk of developing serious hyperbilirubinemia. The objective of this study was to reassess the clinical syndrome of kernicterus as neurodiagnostic studies have become more readily available and can be used to evaluate these infants.

Methods:

The study population was neonates born at term or near term admitted to The Hospital for Sick Children in Toronto, Ontario, Canada, between January 1990 and May 2000. During the study period, there were 9776 admissions (average number of admissions per year – 888 infants). The inclusion criteria were that patients had total serum bilirubin levels of > 400μmol/L at the time of diagnosis and no evidence of hypoxic ischemic encephalopathy. Records were reviewed to establish neurodevelopment outcomes.

Results:

Twelve neonates (nine males) were identified. Bilirubin levels at the time of diagnosis ranged from 405 to 825μmol/L. Causes of these elevated levels included glucose-6-phosphate dehydrogenase deficiency (seven patients), dehydration (three patients), sepsis (one patient), and was undetermined in one patient. Abnormal visual evoked potentials were found in three of nine patients and abnormal brainstem auditory evoked potentials in seven of ten patients. Abnormal electroencephalograms were documented in five patients studied. Brain magnetic resonance imaging results were abnormal in three of four patients.

Conclusions:

Magnetic resonance imaging typically showed an increased signal in the posteromedial aspect of the globus pallidus and was, therefore, useful in the assessment of the structural changes of chronic bilirubin encephalopathy after kernicterus.

Résumé:

RÉSUMÉ:

La prévention de l’encéphalopathie par hyperbilirubinémie est basée sur le dépistage des nouveau-nés qui sont à risque de développer une hyperbilirubinémie sévère. L’objectif de cette étude était de réévaluer le syndrome clinique du kernictère étant donné que les examens neurodiagnostiques sont de plus en plus disponibles et peuvent être utilisés pour évaluer ces enfants. La population à l’étude comprend les nouveau-nés à terme ou près du terme admis au Hospital for Sick Children de Toronto, Ontario, Canada, entre janvier 1990 et mai 2000, soit 9776 admissions (moyenne annuelle de 888 nouveau-nés). Les critères d’inclusion étaient un taux de bilirubine sérique total de plus de 400 mmol/L au moment du diagnostic, sans évidence d’encéphalopathie ischémique hypoxique. Les dossiers ont été révisés pour vérifier l’issue neurodéveloppementale. Douze nouveau-nés, dont 9 garçons et 3 filles, ont été identifiés. Les taux de bilirubine au moment du diagnostic étaient de 405 à 825 mmol/L, dont la cause était un déficit en glucose-6-phosphate-déshydrogénase chez 7 patients, une déshydratation chez 3 patients, une septicémie chez 1 patient et indéterminée chez 1 patient. Les potentiels évoqués visuels étaient anormaux chez 3 patients sur 9 et les potentiels évoqués auditifs au niveau du tronc cérébral étaient anormaux chez 7 patients sur 10. Des anomalies à l’ÉEG ont été observées chez 5 patients. L’IRM était anormale chez 3 patients sur 4. La présentation typique à l’IRM était une augmentation du signal dans la région postéro-médiale du globus pallidus et s’est avérée utile dans l’évaluation des changements structuraux de l’encéphalopathie chronique après un kernictère.

Type
Original Article
Information
Canadian Journal of Neurological Sciences , Volume 32 , Issue 3 , August 2005 , pp. 311 - 315
Copyright
Copyright © The Canadian Journal of Neurological 2005

References

1. AAP Subcommittee on Neonatal hyperbilirubinemia. Neonataljaundice and Kernicterus. Pediatrics 2001; 108:763–765.CrossRefGoogle Scholar
2. Mckinney, A, Filice, R. Diffusion abnormalities of the globi pallidi inmanganese neurotoxicity. Neuroradiology 2004; 46 (4):291–295.Google Scholar
3. Ebbesen, F. Recurrence of kernicterus in term and near-term infantsin Denmark. Acta Paediatr 2000; 89:1213–1217.Google Scholar
4. Watchko, JF, Oski, FA. Kernicterus in preterm newborns: past,present, and future. Pediatrics 1992; 90:707–715.Google Scholar
5. Gourley, GR. Bilirubin metabolism and kernicterus. Adv Pediatr 1997; 44:173–229.Google Scholar
6. Maisels, MJ, Newman, TB. Kernicterus in otherwise healthy, breast-fed term newborns. Pediatrics 1995; 96:730–733.Google Scholar
7. Bertini, G, Dani, C, Pezzati, M, Rubaltelli, FF. Prevention of bilirubin encephalopathy. Biol Neonate 2001; 79:219–223.Google Scholar
8. MacDonald, MG. Hidden risks: early discharge and bilirubintoxicity due to glucose 6-phosphate dehydrogenase deficiency. Pediatrics 1995; 96:734–738.Google Scholar
9. Chisin, R, Perlman, M, Sohmer, H. Cochlear and brainstem responsesin hearing loss following neonatal hyperbilirubinemia. Ann Otol Rhinol Laryngol 1979; 88:352–357.Google Scholar
10. Vohr, BR. New approaches to assessing the risks ofhyperbilirubinemia. Clin Perinatol 1990; 17:293–306.CrossRefGoogle Scholar
11. Volpe, JJ, (Ed). Neurology Of The Newborn, 4th ed. Philadelphia: WB Saunders, 2000;490-510.Google Scholar
12. Vohr, BR, Karp, D, O’Dea, C, et al. Behavioral changes correlatedwith brainstem auditory evoked responses in term infants with moderate hyperbilirubinemia. J Pediatr 1990; 117:288–291.Google Scholar
13. Perlman, M, Frank, JW. Bilirubin beyond the blood-brain barrier. Pediatrics 1988; 81:304–315.Google Scholar
14. Funato, M, Tamai, H, Shimada, S, Nakamura, H. Vigintiphobia,unbound bilirubin, and auditory brainstem responses. Pediatrics 1994; 93:50–53.Google Scholar
15. Kaga, K, Kitazumi, E, Kodama, K. Auditory brainstem responses ofkernicterus infants. Int J Pediatr Otorhinolaryngol 1979; 1:255–264.Google Scholar
16. Solomon, G, Labar, D, Galbraith, RA, et al. Neurophysiologicalabnormalities in adolescents with type I Crigler-Najjar syndrome. Electroencephalogr Clin Neurophysiol 1990; 76:473–475.CrossRefGoogle ScholarPubMed
17. Perlstein, MA. Thelateclinicalsyndromeof postictericencephalopathy. Pediatr Clin North Am 1960; 7:665–687.CrossRefGoogle Scholar
18. Hayashi, M, Satoh, J, Sakamoto, K, Morimatsu, Y. Clinical andneuropathological findings in severe athetoid cerebral palsy: a comparative study of globo-Luysian and thalamo-putaminal groups. Brain Dev 1991; 13:47–51.Google Scholar
19. Yokochi, K. Magnetic resonance imaging in children withkernicterus. Acta Paediatr 1995; 84:937–939.Google Scholar
20. Byers, RK, Paine, RS, Crothers, B. Extrapyramidal cerebral palsywith hearing loss following erythroblastosis. Pediatrics 1955; 15:248–254.Google Scholar
21. Connolly, AM, Volpe, JJ. Clinical features of bilirubinencephalopathy. Clin Perinatal 1990; 17:371–379.Google Scholar