Ultrastructural aspects of muscle and nerve in Werdnig-Hoffmann disease.

Abstract:

:The authors describe in biopsies from 6 cases of Werdnig-Hoffmann disease, including 2 of the more benign type, the ultrastructural typical aspects of denervation. They compare their findings with those of other workers. The striking points are the great variation in the diameter of the muscle fibres and the myofibrils, the disorganisation of the myofibrils, the sarcomeres and the filaments, with persistance of the relations between thick and thin filaments at various levels, the modifications of the Z-band and the triads in chains. The folds and the basement membrane are examined. Centrioles are present in a muscle fibre and in a satellite. Glycogen is very abundant. The nerves seem normal but some Schwann cells contain pi granules which are not observed usually at the age of the patient. The end plates and a muscle spindle are normal.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Szliwowski HB,Drochmans P

doi

10.1007/BF00687923

subject

Has Abstract

pub_date

1975-01-01 00:00:00

pages

281-96

issue

4

eissn

0001-6322

issn

1432-0533

journal_volume

31

pub_type

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