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Morphological and morphometric studies were performed on the spinal cords and roots
in 6 typical cases of Werdnig-Hoffmann disease (WHD) and 3 age-matched controls to
gain further insight into the pathogenesis of this disease.
(1) We observed islands of glial bundles in the anterior spinal roots in all cases
and in the posterior roots in one case. (2) Loss of myelin was seen in the posterior
columns and spinocerebellar tracts at the thoracic and lumbar segments in two cases,
and in the anterior and lateral corticospinal tracts at the thoracic and lumbar segments
in one case. (3) A few figures of neuronophagia were observed in the Onufrowicz nuclei
in two cases, whereas the figures were hardly seen in other nuclei. (4) a) In the
lateral nuclei of C5, L3 and S2 segments of the spinal cords in WHD, the reduction
in the number of motor cells was almost symmetrical and diffuse throughout the spinal
cord, and it varied from one-eleventh to one-third of controls. b) In the anteromedial
nuclei of C5 and L3 segments, there was mild to moderate reduction in the number of
motor cells (50–75% and 36–72% of controls, respectively). c) There was no distinct
difference in the number and the mean diameter of the cells in the Onufrowicz nucleus
between WHD and controls, but some of the cells of the nucleus in WHD showed central
chromatolysis (3.1–4.9%) to the same extent as those of other nuclei. d) In controls,
the mean minimum diameter of the cells of the lateral, anteromedial and Onufrowicz
nuclei was comparatively large, medium and small, respectively.
These results suggested a tendency to initial degeneration of the clusters of the
large motor cells and later degeneration in those of the small or medium cells in
WHD. However, the cause remains unknown.
Key words
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Article info
Publication history
Accepted:
January 16,
1982
Received:
November 20,
1981
Identification
Copyright
© 1982 Published by Elsevier Inc.
