PEDIATRICS Vol. 22 No. 6 December 1958, pp. 1064-1073
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METACHROMATIC LEUKO-ENCEPHALOPATHY

Review with Illustrative Case Report

Raymond F. Hain M.D.1 and Gerald D. LaVeck M.D.2

1 Department of Pathology (RFH), University of Washington School of Medicine
2 Department of Pediatrics (GDL), University of Washington School of Medicine

Metachromatic leuko-encephalopathy is a familial degenerative disease of the central nervous system included with Schilder's disease as a type of diffuse cerebral sclerosis. The disease usually has its onset early in childhood and is characteried by progressive motor and mental deterioration with ataxia, muscular weakness, spasticity, optic atrophy, convulsions and finally dementia. The concentration of protein of the cerebrospinal fluid is frequently elevated.

The pathologic findings consist of demyelination, destruction of axons, gliosis and the accumulation of metachromatic granules in the brain and other organs. The metachromasia can be demonstrated in formalin-fixed frozen sections with a toluidine blue stain. It is not demonstrable in paraffin sections. Histochemical studies indicate this abnormal material is probably a complex of glycolipids and protein. It has been reported elsewhere that early diagnosis can be established by demonstrating the metachromatic materials in urinary sediment or in a real biopsy. An illustrative case has been presented.

Submitted on March 31, 1958
Accepted on June 3, 1958