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Pediatrics · Neonatology: chronic bilirubin encephalopathy
Sample itemA 4-year-old who had very high unconjugated bilirubin as a newborn now has athetoid movements, high-frequency sensorineural deafness and limited upward gaze. Which brain region is preferentially damaged?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Globus pallidus and subthalamic nucleus
Unconjugated bilirubin stains and damages the basal ganglia, especially the globus pallidus and subthalamic nucleus, along with brainstem auditory and oculomotor nuclei. The chronic picture is athetoid cerebral palsy, sensorineural deafness, upward gaze palsy and enamel hypoplasia. Prevention depends on timely phototherapy and exchange transfusion.
Option by option
A. Parasagittal watershed cortex
Watershed cortical injury follows hypotension and gives spastic quadriparesis.
B. Periventricular white matter
Periventricular white matter injury produces spastic diplegia in preterm infants.
C. Globus pallidus and subthalamic nucleus (correct)
Correct. Bilirubin deposits in the globus pallidus and subthalamic nucleus, giving athetosis.
D. Cerebellar Purkinje cell layer
Purkinje cell loss is not the typical site and would give ataxia, not athetosis with deafness.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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