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

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

Fingerprint

ASJC Scopus subject areas

Clinical Neurology

Cite this

Brown, F. R., Shimizu, H., McDonald, J. M., Moser, A. B., Marquis, P., Chen, W. W., & Moser, H. W. (1981). Auditory evoked brainstem response and high-performance liquid chromatography sulfatide assay as early indices of metachromatic leukodystrophy. Neurology, 31(8), 980-985. https://doi.org/10.1212/wnl.31.8.980

@article{c97ea6d5cea8437c8a8f57eefdf6fe53,

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

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.",

author = "Brown, {Frank R.} and Hiroshi Shimizu and McDonald, {James M.} and Moser, {Ann B.} and Paul Marquis and Chen, {Winston W.} and Moser, {Hugo W.}",

year = "1981",

month = aug,

doi = "10.1212/wnl.31.8.980",

language = "English (US)",

volume = "31",

pages = "980--985",

journal = "Neurology",

issn = "0028-3878",

publisher = "Lippincott Williams and Wilkins",

number = "8",

}

TY - JOUR

T1 - Auditory evoked brainstem response and high-performance liquid chromatography sulfatide assay as early indices of metachromatic leukodystrophy

AU - Brown, Frank R.

AU - Shimizu, Hiroshi

AU - McDonald, James M.

AU - Moser, Ann B.

AU - Marquis, Paul

AU - Chen, Winston W.

AU - Moser, Hugo W.

PY - 1981/8

Y1 - 1981/8

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=0019453538&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0019453538&partnerID=8YFLogxK

U2 - 10.1212/wnl.31.8.980

DO - 10.1212/wnl.31.8.980

M3 - Article

C2 - 6115337

AN - SCOPUS:0019453538

VL - 31

SP - 980

EP - 985

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 8

ER -

Access to Document

10.1212/wnl.31.8.980