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Journal of Child Neurology
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Ultrastructural Pathology of Eccrine Sweat Gland Epithelial Cells in Globoid Cell Leukodystrophy

Hans H. Goebel, MD

Division of Neuropathology, University of Mainz, Mainz, Germany

Seiji Kimura, MD

Department of Pediatrics, Yokohama University Medical School, Yokohama, Japan

Klaus Harzer, MD

Institute of Brain, Research University of Tübingen, Tübingen, Germany

Harold Klein, MD

Institute of Clinical Neuropathology, Krankenhaus Bremen Ost, Bremen, Germany

Three of four children were recognized by deficient β-galactocerebrosidase activities as having globoid cell leukodystrophy inclusions in sweat gland epithelial cells, similar in ultrastructure to those seen in Schwann cells. This observation in globoid cell leukodystrophy emphasizes the need to include sweat gland epithelial cells in examinations of skin in globoid cell leukodystrophy, as well as in any neurometabolic disorder. (J Child Neurol 1993;8:171-174).

Journal of Child Neurology, Vol. 8, No. 2, 171-174 (1993)
DOI: 10.1177/088307389300800211


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J Child NeurolHome page
A. Prasad, E. M. Kaye, and J. Alroy
Electron Microscopic Examination of Skin Biopsy as a Cost-Effective Tool in the Diagnosis of Lysosomal Storage Diseases
J Child Neurol, July 1, 1996; 11(4): 301 - 308.
[Abstract] [PDF]