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Anil Dhuna, MD; Camilo Toro, MD; Fernado Torres, MD; William R. Kennedy, MD; William Krivit, MD, PhD

Arch Neurol. 1992;49(10):1088-1092.

Abstract
• We describe a girl with late infantile metachromatic leukodystrophy. The patient has been followed up with serial neurologic and neurophysiologic examinations for 8 years following bone marrow transplantation, which she underwent when she was 43/4 years old. Her older sister died from metachromatic leukodystrophy at the age of 8 years, whereas our patient has retained significant cognitive and motor skills. Serial neurophysiologic studies initially demonstrated continued deterioration after the bone marrow transplantation, but since then, most results have remained stable or improved. Although, to our knowledge, there have been no previous serial studies of metachromatic leukodystrophy, individual case studies suggest that these findings in our patient are very unusual. With the advent of possible treatment for this condition, there is a need for further serial neurophysiologic studies to characterize the natural progression and the possible detection of progression or reversal with treatment.

Author Affiliations
From the Departments of Neurology (Drs Dhuna, Torres, and Kennedy) and Pediatrics (Dr Krivit), University of Minnesota, and the Neurology Service, Veterans Affairs Medical Center (Dr Dhuna), Minneapolis, Minn, and the Human Cortical Physiology Unit, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Md (Dr Toro).

Footnotes
Accepted for publication June 25, 1992.

Reprint requests to Department of Neurology, University of Minnesota, Box 295 UMHC, 20 Delaware St SE, Minneapolis, MN 55455 (Dr Dhuna).

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