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Query: UMLS:C0033377 (prolapse)
 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Light and electronmicroscopic findings in two cases. Neuropediatrics 12: 215-31 (1981). Two cases of infantile spinal muscular atrophy (Werdnig-Hoffmann disease) are described in unrelated children deceased at 11 months (acute clinical onset at 6 months) and 2 years (onset at birth). Severe respiratory difficulties, hypotonia, muscular weakness and depressed tendon reflexes were the main clinical features. Bulbar palsy, bilateral ptosis, pale optic discs and atactic movements of the hands were observed in the child deceased at 11 months. Besides severe loss of anterior horn cells and neurogenic muscle atrophy there was evidence of an extensive sensory involvement in both cases. Shrinkage, vacuolation as well as chromatolytic changes of dorsal root ganglion cells, together with the evidence of a primary axonal damage in sural nerve biopsies were interpreted in terms of ganglioneuropathy of the primary sensory neurons. An invasion of fibrous astrocytes into dorsal roots constituted another striking anomaly in one case as well as a pronounced degeneration of cranial nerves V and VIII in the other case, a finding not hitherto reported in Werdnig-Hoffmann disease.
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PMID:Sensory ganglioneuropathy in infantile spinal muscular atrophy. Light and electronmicroscopic findings in two cases. 729 Mar 43

Although pathological changes are observed in both the oculomotor nucleus and abducens nucleus in autopsied cases of infantile progressive spinal muscular atrophy, external and internal ocular palsy and ptosis have not been previously reported clinically. We presented here two long-surviving cases on respirators which gradually developed ophthalmoplegia and ptosis were presented. From our observation of these cases, it was suggested that there are certain periods of latencies between the occurrence of pathological changes and their clinical manifestation and that the lack of clinical signs of upper cranial nerve involvement in cases with Werdnig-Hoffmann type I is due to their short survival length.
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PMID:[Werdnig-Hoffmann disease type I with progressive ophthalmoplegia and ptosis]. 875 33