Linked Life Data

2168777

Source:http://linkedlifedata.com/resource/pubmed/id/2168777

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
1990-10-17
pubmed:abstractText
The pathology of the peripheral nervous system (PNS) in 2 autopsied cases of group A xeroderma pigmentosum (De Sanctis Cacchione syndrome) are presented. Motor nerves including those of the oculomotor systems were severely affected, but involvement of the sensory system was even more marked. Minor hypertrophic changes were present in the distal portions of the peripheral nerve trunks, but there was no appreciable difference in the density of myelinated nerve fibres between proximal and distal levels. Morphometric data including teased fibre analyses and g ratio scattergrams suggest that the underlying pathogenetic mechanism is that of a neuronopathy. Unmyelinated axons were also severely depleted. Review of the previous literature revealed that the pathological changes of the PNS in group A xeroderma pigmentosum are thought to be slowly progressive, which is also suggested by the severe and widespread sclerotic changes of the CNS in the present 2 cases.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
0006-8950
pubmed:author
pubmed:issnType
Print
pubmed:volume
113 ( Pt 4)
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1025-44
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed:year
1990
pubmed:articleTitle
Peripheral neuropathy in xeroderma pigmentosum.
pubmed:affiliation
Department of Neurology, Tokyo Medical and Dental University, Japan.
pubmed:publicationType
Journal Article, Case Reports