|

RID5059 kernicterus

synonym

BIND Bilirubin encephalopathy
Kernikterus
bilirubin-induced encephalopathy
bilirubin-induced neurologic dysfunction
kemicterus

definition

A term used pathologically to describe bilirubin staining of the basal ganglia; brain stem; and cerebellum and clinically to describe a syndrome associated with HYPERbilirubinEMIA. Clinical features include athetosis, muscle spasticity or hypotonia, impaired vertical gaze, and deafness. Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the blood-brain barrier (e.g., sepsis). This condition occurs primarily in neonates (infant, newborn), but may rarely occur in adults. (Menkes, Textbook of Child Neurology, 5th ed, p613) [MeSH]