Linked Life Data

6837264

Source:http://linkedlifedata.com/resource/pubmed/id/6837264

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1983-5-27
pubmed:abstractText
Electromyography was performed, and motor and sensory nerve conduction velocities were measured in 19 patients definitely affected by Friedreich's ataxia. Biopsy of the sural nerve was also performed in 9 patients. Most patients presented a moderate to severe loss of motor units, a significant increase in mean duration of motor unit potentials, and in the incidence of polyphasic potentials. Short-lasting spontaneous activity was rarely seen. Conduction velocity along the motor and sensory fibres of the median and tibial nerves was moderately slowed, while distal conduction time to muscle was significantly increased and the sensory orthodromically-evoked response markedly reduced. Intraoperative electrophysiological recordings obtained during biopsy of the sural nerve in 4 patients were consistent with the changes conventionally observed in the median, tibial and sural (6 patients) nerves. Quantitative histology revealed a reduced number of total myelinated fibres with a severe loss of large fibres, and a moderate loss of fibres of less than 7 microns in diameter. In teased nerve fibre preparations, the most evident abnormality consisted of fibres with uniformly short internodal length, while signs of remyelination were less prominent. Signs of active axonal degeneration were rarely observed in electron microscopy. Electrophysiological and histological findings were uniformly distributed, and the changes were neither related to the duration nor to the severity of the clinical condition.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Jan
pubmed:issn
0001-6314
pubmed:author
pubmed:issnType
Print
pubmed:volume
67
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
26-40
pubmed:dateRevised
2006-8-16
pubmed:meshHeading
pubmed:year
1983
pubmed:articleTitle
Friedreich's ataxia: electrophysiological and histological findings.
pubmed:publicationType
Journal Article