Cerebellar Hypoplasia in Werdnig‐Hoffmann Disease
- 1 August 1975
- journal article
- case report
- Published by Wiley in Developmental Medicine and Child Neurology
- Vol. 17 (4), 511-516
- https://doi.org/10.1111/j.1469-8749.1975.tb03503.x
Abstract
The case of an infant with Werding-Hoffmann disease, who died at the age of 4 1/2 days, is reported. At autopsy there was severe cerebellar hypoplasia, associated with degenerative changes in the brain-stem nuclei. This case supports the concept that cerebellar hypoplasia may develop as a manifestation of the neuronal abiotrophy of Werdnig-Hoffmann disease. A similarity has been noted between the cerebellar lesion found in the child reported here and that produced by viral infection in experimental animals.Keywords
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