Clinicopathological Spectrum of Bilirubin Encephalopathy/Kernicterus

Sumit Das, Frank K H van Landeghem, Sumit Das, Frank K H van Landeghem

Abstract

Bilirubin encephalopathy/kernicterus is relatively rare, but continues to occur despite universal newborn screening. What is more interesting is the spectrum of clinical and even neuropathological findings that have been reported in the literature to be associated with bilirubin encephalopathy and kernicterus. In this review, the authors discuss the array of clinicopathological findings reported in the context of bilirubin encephalopathy and kernicterus, as well as the types of diagnostic testing used in patients suspected of having bilirubin encephalopathy or kernicterus. The authors aim to raise the awareness of these features among both pediatric neurologists and neuropathologists.

Keywords: bilirubin encephalopathy; diagnosis; kernicterus; neurological symptoms; neuropathology.

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Neuropathologic findings from a patient with kernicterus. (a) Yellowish discoloration of subthalamic nucleus and hippocampus; (b) discoloration of medullary tegmentum, inferior olives, and cerebellar tonsils; (c) cytoplasmic pigment in cells of choroid plexus (20× magnification); (d) Alzheimer’s type II astrocytes (arrow) in keeping with liver failure (20× magnification).
Figure 1
Figure 1
Neuropathologic findings from a patient with kernicterus. (a) Yellowish discoloration of subthalamic nucleus and hippocampus; (b) discoloration of medullary tegmentum, inferior olives, and cerebellar tonsils; (c) cytoplasmic pigment in cells of choroid plexus (20× magnification); (d) Alzheimer’s type II astrocytes (arrow) in keeping with liver failure (20× magnification).

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