Linked Life Data

1664789

Source:http://linkedlifedata.com/resource/pubmed/id/1664789

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
7
pubmed:dateCreated
1992-3-20
pubmed:abstractText
Peripheral neuropathy (PN) associated with mitochondrial encephalomyopathy (MEM) has been reported in adult patients, while children with both conditions are rare. Electrophysiological and pathological studies disclosed evidence of PN in a 3-year-old girl and an 8-year-old boy with MEM. In both patients, peripheral nerve conduction velocities were reduced, while amplitudes of evoked potentials were normal. No ragged red fibers were found in the biopsy muscle, while most of the muscle fibers showed poor activity with histochemical staining for cytochrome c oxidase (CCO). Biochemical studies revealed deficiency of CCO in both cases. In the latter patient, CCO activity was also absent in the intramuscular peripheral nerve using CCO staining, and histopathological studies of the sural nerve revealed a marked decrease in the number of large myelinated fibers and an unusual accumulation of the mitochondria in the Schwann cell cytoplasm. These results may support the hypothesis that a common pathogenesis exists in both peripheral nerve and muscle due to mitochondrial dysfunction.
pubmed:language
jpn
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
0009-918X
pubmed:author
pubmed:issnType
Print
pubmed:volume
31
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
772-4
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1991
pubmed:articleTitle
[Peripheral neuropathy in two children with mitochondrial encephalomyopathy].
pubmed:affiliation
Department of Pediatrics, Osaka University Medical School.
pubmed:publicationType
Journal Article, English Abstract, Case Reports