Globoid Cell Leukodystrophy: Cranial Computed Tomography and Evoked Potentials

Author:

Darras Basil T.1,Kwan Eddie S.2,Gilmore Herbert E.1,Ehrenberg Bruce L.3,Rabe Edward F.1

Affiliation:

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

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

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

Abstract

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)

Publisher

SAGE Publications

Subject

Clinical Neurology,Pediatrics, Perinatology, and Child Health

Reference11 articles.

1. A NEW FAMILIAL, INFANTILE FORM OF DIFFUSE BRAIN-SCLEROSIS

2. Suzuki K., Suzuki Y.: Galactosylceramide lipidosis: globoid cell leukodystrophy (Krabbe's disease) . In Stanbury JB, Wyngaarden BJ, Fredrickson DS, et al (eds): The Metabolic Basis of Inherited Disease . New York, McGraw-Hill, 1983, pp 857-880.

3. Cortical electrical responses to visual stimulation in the human infant

4. Christensen EE, Curry TS, Dowdey JE: Computed tomography, in Christensen EE, Curry TS, Dowdey JE (eds): An Introduction to the Physics of Diagnostic Radiology . Philadelphia, Lea & Febiger , 1978, pp 348-350.

5. Brainstem auditory evoked responses in leukodystrophies

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