Delayed posthypoxic demyelination
- 1 November 1997
- journal article
- case report
- Published by Ovid Technologies (Wolters Kluwer Health) in Neurology
- Vol. 49 (5), 1400-1404
- https://doi.org/10.1212/wnl.49.5.1400
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.This publication has 7 references indexed in Scilit:
- Problems in Pharmacokinetic Analysis of Alcohol Disposition: A Trial of the Bayesian Least‐Squares MethodAlcohol: Clinical and Experimental Research, 1996
- Amyloid and the nervous systemZeitschrift für Neurologie, 1995
- Migration and Accumulation of Silicone in the Liver of Women with Silicone Gel‐Filled Breast ImplantsMagnetic Resonance in Medicine, 1995
- Megadose corticosteroids in multiple sclerosisNeurology, 1994
- Bibliography on ceroid-lipofuscinosesAmerican Journal of Medical Genetics, 1988
- Neuroanatomical and Neurophysiologic StudiesArchives of Neurology, 1974
- Publishers and PrintersArchives of Internal Medicine, 1962