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

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 languageEnglish (US)
Pages (from-to)980-985
Number of pages6
JournalNeurology
Volume31
Issue number8
StatePublished - 1981

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Metachromatic Leukodystrophy
Sulfoglycosphingolipids
Brain Stem Auditory Evoked Potentials
High Pressure Liquid Chromatography
Neural Conduction
Neurologic Examination
Peripheral Nerves
Pathology
Hearing
Onset
High-performance Liquid Chromatography
Nerve

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology
  • Neuroscience(all)

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.

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, 1981, p. 980-985.

Research output: Contribution to journalArticle

Brown, FR, Shimizu, H, McDonald, JM, Moser, AB, Marquis, P, Chen, WW & Moser, HW 1981, 'Auditory evoked brainstem response and high-performance liquid chromatography sulfatide assay as early indices of metachromatic leukodystrophy', Neurology, vol. 31, no. 8, pp. 980-985.
Brown, Frank R. ; Shimizu, Hiroshi ; McDonald, James M. ; Moser, Ann B. ; Marquis, Paul ; Chen, Winston W. ; Moser, Hugo W. / Auditory evoked brainstem response and high-performance liquid chromatography sulfatide assay as early indices of metachromatic leukodystrophy. In: Neurology. 1981 ; Vol. 31, No. 8. pp. 980-985.
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