Auditory evoked brainstem response and high-performance liquid chromatography sulfatide assay as early indices of metachromatic leukodystrophy

Frank R. Brown, Hiroshi Shimizu, James M. McDonald, Ann B. Moser, Paul Marquis, Winston W. Chen, Hugo W. Moser

Research output: Contribution to journalArticle


We studied three patients with metachromatic leukodystrophy, two with late infantile onset and one with juvenile onset. Auditory brainstem responses were abnormal in all patients, including a 14-month-old child who was asymptomatic, with normal neurologic examination and peripheral nerve conduction studies. Auditory brainstem responses therefore indicated early pathology. A new high-performance liquid chromatography assay was used to quantitate the urinary sulfatide excretion at the picomole level.

Original languageEnglish (US)
Pages (from-to)980-985
Number of pages6
Issue number8
StatePublished - Aug 1981


ASJC Scopus subject areas

  • Clinical Neurology

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