Cerebellar hemorrhage complicating isovaleric acidemia: A case report

Asma Q. Fischer, Venkata R. Challa*, Barbara K. Burton, William T. McLean

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

25 Scopus citations

Abstract

Isovaleric acidemia (IVA) is an inborn error of leucine metabolism, resulting in an accumulation of isovaleric acid in the body fluids. The neuropathologic findings in an 11-day-old infant with IVA consisted of diffuse cerebral edema, massive cerebellar hemor-rhage, upward transtentorial herniation, and focal degeneration of clusters of glial cells in white and gray matter. Although abnormal myelination has been described in different aminoacidopathies, the above findings have not been reported previously in IVA.

Original languageEnglish (US)
Pages (from-to)746-748
Number of pages3
JournalNeurology
Volume31
Issue number6
StatePublished - Jun 1981

ASJC Scopus subject areas

  • Clinical Neurology

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