Electron microscopic investigation of inclusion material in a case of adult metachromatic leukodystrophy; observations on kidney biopsy, peripheral nerve and cerebral white matter.

Acta neuropathologica ·Vol. 33 ·No. 2 ·1975-12-08 ·Pages 165-71

Joosten E, Hoes M, Gabreëls-Festen A, Hommes O, Schuurmans Stekhoven H, Slooff JL

Abstract

The fine structural characteristics of storage products in peripheral nerve, kidney and cerebral white matter, from a case of adult metachromatic leukodystrophy are described. There were pronounced differences from the fine structural aspects in late infantile cases. A large proportion of the inclusions did not exhibit a unit membrane. An hypothesis is proposed to clarify the delayed manifestation of this type of metachromatic leukodystrophy until childhood.

MeSH Terms

Acid Phosphatase/analysis Adult Brain/ultrastructure Demyelinating Diseases Humans Inclusion Bodies/ultrastructure Kidney Tubules/ultrastructure Kidney Tubules, Distal/ultrastructure Leukodystrophy, Metachromatic/pathology Lysosomes Macrophages/ultrastructure Male Neurons/ultrastructure Peripheral Nerves/enzymology,ultrastructure Schwann Cells/ultrastructure Sulfoglycosphingolipids/metabolism

Chemicals

Sulfoglycosphingolipids Acid Phosphatase

Authors & Affiliations

6 authors, click to expand affiliations / ORCID

Joosten E

Hoes M

Gabreëls-Festen A

Hommes O

Schuurmans Stekhoven H

Slooff J L

References (13)

13 references, click to expand

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J Neurol Sci. 1972 Dec;17(4):383-7 PMID: 5086255
Enzyme replacement in cultured fibroblasts from metachromatic leukodystrophy.
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The sciatic nerve in juvenile metachromatic leucodystrophy: a quantitative evaluation.
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Article Info

Journal

Acta neuropathologica

Abbr.

Acta Neuropathol

ISSN

0001-6322

DOI

10.1007/BF00687542

Published

1975-12-08

Pages

165-71

Language

English

Region

Germany

NLM ID

0412041

Subset

External Links

PubMed source DOI: 10.1007/BF00687542 View journal details

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Electron microscopic investigation of inclusion material in a case of adult metachromatic leukodystrophy; observations on kidney biopsy, peripheral nerve and cerebral white matter.

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