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Journal of Child Neurology
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Globoid Cell Leukodystrophy: Cranial Computed Tomography and Evoked Potentials

Basil T. Darras, MD

Departments of pediatrics (neurology), New England Medical Center University School of Medicine

Eddie S. Kwan, MD

Department of neuroradiology, New Engalnd Medical Center and Tufts University School of Medicine

Herbert E. Gilmore, MD

Departments of pediatrics (neurology), New England Medical Center University School of Medicine

Bruce L. Ehrenberg, MD

Department of Neurology Boston Floating Hospital, New England Medical Center and Tufts University School of Medicine, Boston

Edward F. Rabe, MD

Departments of pediatrics (neurology), New England Medical Center University School of Medicine

The evoked potentials and cranial computed tomographic (CT) scan findings in a case of early infantile globoid cell leukodystrophy are presented. The brain stem auditory evoked responses (BAERs) and the flash visual evoked potentials (VEPs) were abnormal. Repeated cranial CT scans showed multiple areas of increased attenuation and progressive cerebral atrophy. These evoked potential and CT scan patterns occurring concomitantly in an infant are suggestive of globoid cell leukodystrophy. (J Child Neurol 1986;1:126-130)

Journal of Child Neurology, Vol. 1, No. 2, 126-130 (1986)
DOI: 10.1177/088307388600100206
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