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NEUROLOGY 1981;31:980
© 1981 American Academy of Neurology

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

Frank R. Brown, III, Ph.D., M.D., Hiroshi Shimizu, M.D., James M. McDonald, Sc.D., Ann B. Moser, B.A., Paul Marquis, M.D., Winston W. Chen, Ph.D. and Hugo W. Moser, M.D.

John F. Kennedy Institute (Drs. Brown, Marquis, Chen, and Moser, and Ms. Moser), and the Departments of Pediatrics (Drs. Brown, Marquis, and Moser), Otolaryngology (Drs. Shimizu and McDonald), and Neurology (Drs. Chen and Moser), Johns Hopkins University School of Medicine, Baltimore, MD.

Address correspondence and reprint requests to Dr. Brown, John F. Kennedy Institute, 707 N. Broadway, Baltimore, MD 21205.

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.




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