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Neuropediatrics 1978; 9(4): 338-350
DOI: 10.1055/s-0028-1091493
Original article

© 1978 by Thieme Medical Publishers, Inc.

Metachromatic Leukodystrophy: A Comparative Study of the Ultrastructural Findings in the Peripheral Nervous System of Three Cases, One of the Late Infantile, One of the Juvenile and one of the Adult Form of the Disease

J. A. F. M. Luijten1 , W. Straks2 , L. F. Blikkendaal-Lieftinck2 , G. E. J. Staal3 , J. Willemse4
  • 1Dept. of Neurology, State University Hospital, Utrecht, The Netherlands
  • 2Dept. of Pathology, State University Hospital, Utrecht, The Netherlands
  • 3Unit of Medical Enzymology, State University Hospital, Utrecht, The Netherlands
  • 4Dept. of Child Neurology, State University Hospital, Utrecht, The Netherlands
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Publication History

1978

1978

Publication Date:
18 November 2008 (online)

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The ultrastructural findings in the peripheral nerve of three cases of M. L. D., namely of the late infantile, the juvenile and the adult form are described.

The pathological changes consist of two phenomena: segmental demyelination and lysosomal storage of sulfatides, both probably separate results of a deficiency of the enzyme Arylsulfatase A. Some differences are found in the degree of segmental demyelination and the type of lysosomal storage products. A possible relation between these differences and the supposed difference in the defect of the enzyme Arylsulfatase A, responsible for the different subtypes of the disease, is postulated.

Metachromatic leukodystrophy - late infantile - juvenile and adult form - ultrastructural findings - peripheral nervous system

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