Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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December 3, 2015
Acute Kernicterus (Bilirubin Encephalopathy)
- Background:
- Kernicterus is a neurologic manifestation of hyperbilirubinemia in the newborn, with premature neonates being more susceptible.
- It results from cerebral deposition of unconjugated bilirubin - globus pallidus (GP), subthalamic nucleus, hippocampus, putamen, cerebellar nuclei, and thalamus
- Relevant Clinical Information:
- Newborn (2–5 days-old) with jaundice, lethargy, hypotonia, and high-pitched cry.
- Serum bilirubin levels > 20mg/dL. Our patient had bilirubinemia of 46mg/dL.
- Key Diagnostic Features: Imaging may be normal.
- Acute phase: Increased signal intensity on T1WI in GP and subthalamic nuclei
- Symmetrical high T2 signal of the GP (most common), subthalamic nuclei, and hippocampus and, less frequently, the thalamus, striatum, substantial nigra, cerebellar nuclei, and cranial nerve. Cerebral cortex and white matter are classically spared.
- T2 signal changes are more characteristic of the subacute and chronic phases.
- Differential Diagnosis:
- Hypoxic-Ischemic injury
- Inborn errors of metabolism
- Manganese deposition (total parental nutrition)
- Treatment:
- Phototherapy and exchange transfusion in severe cases
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