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

doi:10.1212/wnl.31.8.980

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

Kennedy Krieger Institute

Research output: Contribution to journal › Article › peer-review

Abstract

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 language	English (US)
Pages (from-to)	980-985
Number of pages	6
Journal	Neurology
Volume	31
Issue number	8
DOIs	https://doi.org/10.1212/wnl.31.8.980
State	Published - Aug 1981

ASJC Scopus subject areas

Clinical Neurology

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Auditory evoked brainstem response and high-performance liquid chromatography sulfatide assay as early indices of metachromatic leukodystrophy. / Brown, Frank R.; Shimizu, Hiroshi; McDonald, James M.; Moser, Ann B.; Marquis, Paul; Chen, Winston W.; Moser, Hugo W.

In: Neurology, Vol. 31, No. 8, 08.1981, p. 980-985.

Research output: Contribution to journal › Article › peer-review

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

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