Presentation
Bilateral sensorineural hearing loss with intellectual disability. Hyperbilirubinemia.
Patient Data
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 globi 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