Kernicterus

Case contributed by Dr Sepehr Haghighi

Presentation

Bilateral sensorineural hearing loss with intellectual disability. Hyperbilirubinaemia.

Patient Data

Age: 8 months
Gender: Female
MRI

Bilateral symmetrical T2 abnormal signal intensity at both globus pallidi, in a patient with history of infantile hyperbilirubinemia, is in-keeping with kernicterus.

Case Discussion

Chronic bilirubin encephalopathy or kernicterus is a consequence of central nervous system deposition of unconjugated bilirubin in an infant. The incidence of this very rare disease is unknown 1,2.

The posteromedial border of both globus pallidi is the most sensitive region for deposition of unconjugated bilirubin which shows high signal in T2 sequences, although it could be normal in early stage of disease 3-6.

The main radiographic differential diagnoses for kernicterus are :

  • hypoxic ischemic encephalopathy
  • hypoglycemia
  • carbon monoxide poisoning
  • encephalitis
  • dysmyelinating disorders
  • various degenerative disease
  • inborn errors of metabolism
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Case information

rID: 65441
Published: 14th Jan 2019
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: Children's medical center

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