Delayed posthypoxic demyelination: Association with arylsulfatase A deficiency and lactic acidosis on proton MR spectroscopy

Jay A. Gottfried, Stephan A. Mayer*, Dikoma C. Shungu, Yuan Chang, Jozef H. Duyn

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

65 Scopus citations

Abstract

Delayed demyelination is a rare and poorly understood complication of hypoxic brain injury. A previous case report has suggested an association with mild-to-moderate deficiency of arylsulfatase A. We describe a 36-year- old man who recovered completely from an episode of hypoxia related to drug overdose, and 2 weeks later progressed from a confusional state to deep coma. MRI showed diffuse white matter signal changes, and brain biopsy demonstrated a noninflammatory demyelinating process. Proton magnetic resonance spectroscopy revealed elevated choline and lactate and reduced N-acetyl aspartate signal in the affected white matter, consistent with demyelination and a shift to anaerobic metabolism. Arylsulfatase A activity from peripheral leukocytes was approximately 50% of normal, consistent with a 'pseudodeficiency' phenotype. These findings confirm the hypothesis that relative arylsulfatase A deficiency predisposes susceptible individuals to delayed posthypoxic leukoencephalopathy and implicates lactic acidosis in the pathogenesis of this disorder.

Original languageEnglish (US)
Pages (from-to)1400-1404
Number of pages5
JournalNeurology
Volume49
Issue number5
DOIs
StatePublished - Jan 1 1997

ASJC Scopus subject areas

  • Clinical Neurology

Fingerprint Dive into the research topics of 'Delayed posthypoxic demyelination: Association with arylsulfatase A deficiency and lactic acidosis on proton MR spectroscopy'. Together they form a unique fingerprint.

Cite this