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January 1959

Constitutional Nonhemolytic Hyperbilirubinemia with Findings Resembling Kernicterus

Author Affiliations

Thiells, N. Y.

From the Research Department, Letchworth Village, New York State Department of Mental Hygiene.

AMA Arch NeurPsych. 1959;81(1):55-64. doi:10.1001/archneurpsyc.1959.02340130075008

One of the most striking effects of liver diseases upon the central nervous system is the characteristic brain damage (kernicterus) which may occur in infants suffering from severe neonatal jaundice. Although the morphologic features of kernicterus have been known for half a century, there is still no completely satisfactory explanation as to the pathogenesis of the brain lesions. Numerous hypotheses have been advanced stressing the relevance of such diverse factors as anoxia, anemia, antigen-antibody reaction, infection, intoxication, and liver failure. In the attempt to contribute to the clarification of this problem, the clinical and pathologic features of an apparently unique case will be presented. The patient, a 44-year-old woman, was affected by constitutional nonhemolytic hyperbilirubinemia, associated since early infancy with an "extrapyramidal" symptom complex. Post mortem, lesions of the brain were found which are consistent with the pathologic diagnosis of sequelae of kernicterus. The main feature of this case will