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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

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