Abnormal neuromuscular transmission in an infantile myasthenic syndrome

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dc.contributor.author Albers, James W. en_US
dc.contributor.author Faulkner, John A. en_US
dc.contributor.author Dorovini-Zis, Katerina en_US
dc.contributor.author Barald, Kate F. en_US
dc.contributor.author Must, Rachel E. en_US
dc.contributor.author Ball, Richard D. en_US
dc.date.accessioned 2007-04-06T18:50:08Z
dc.date.available 2007-04-06T18:50:08Z
dc.date.issued 1984-07 en_US
dc.identifier.citation Albers, James W.; Faulkner, John A.; Dorovini-Zis, Katerina; Barald, Kate F.; Must, Rachel E.; Ball, Richard D. (1984)."Abnormal neuromuscular transmission in an infantile myasthenic syndrome." Annals of Neurology 16(1): 28-34. <http://hdl.handle.net/2027.42/50308> en_US
dc.identifier.issn 0364-5134 en_US
dc.identifier.issn 1531-8249 en_US
dc.identifier.uri http://hdl.handle.net/2027.42/50308
dc.identifier.uri http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=retrieve&db=pubmed&list_uids=6087719&dopt=citation en_US
dc.description.abstract A term infant required intubation for respiratory depression. Examination revealed hypotonia and areflexia with intact extraocular movements. Electrodiagnostic studies demonstrated defective neuromuscular transmission characterized by borderline low motor evoked amplitudes, profound decremental responses at all stimulation rates, and moderate facilitation (50 to 740%) 15 seconds after 5 seconds of 50 Hz stimulation. Repetitive muscle action potential responses were not recorded following stimulation of nerves by single shocks. Sensory evoked responses and needle electromyographic findings were normal, as were acetylcholine receptor antibody levels. Results of muscle histochemical analyses, including acetylcholinesterase stains, were normal. End-plate histometric analyses demonstrated only a slight reduction in mean synaptic vesicle diameter compared with that in an adult control subject. In vitro muscle contractile properties, stimulating the muscle directly, were normal. Anticholinesterase medications were ineffective. Guanidine produced clinical deterioration. The amplitude of motor evoked responses progressively declined, whereas the percentage of decrement and amount of post-tetanic facilitation increased. Although the nature of the transmission defect was not identified, the data are consistent with abnormal acetylcholine resynthesis, mobilization, or storage without abnormality of release or receptors. en_US
dc.format.extent 1836904 bytes
dc.format.extent 3118 bytes
dc.format.mimetype application/pdf
dc.format.mimetype text/plain
dc.publisher Wiley Subscription Services, Inc., A Wiley Company en_US
dc.subject.other Life and Medical Sciences en_US
dc.subject.other Neuroscience, Neurology, and Psychiatry en_US
dc.title Abnormal neuromuscular transmission in an infantile myasthenic syndrome en_US
dc.type Article en_US
dc.rights.robots IndexNoFollow en_US
dc.subject.hlbsecondlevel Psychiatry en_US
dc.subject.hlbtoplevel Health Sciences en_US
dc.description.peerreviewed Peer Reviewed en_US
dc.contributor.affiliationum The Neuromuscular Section, The University of Michigan Medical Center, Ann Arbor, MI 48109 ; Department of Neurology, B4952, Clinical Faculty Office Bldg, The University of Michigan Medical Center, Ann Arbor, MI 48109 en_US
dc.contributor.affiliationum The Neuromuscular Section, The University of Michigan Medical Center, Ann Arbor, MI 48109 en_US
dc.contributor.affiliationum The Neuromuscular Section, The University of Michigan Medical Center, Ann Arbor, MI 48109 en_US
dc.contributor.affiliationum The Neuromuscular Section, The University of Michigan Medical Center, Ann Arbor, MI 48109 en_US
dc.contributor.affiliationum The Neuromuscular Section, The University of Michigan Medical Center, Ann Arbor, MI 48109 en_US
dc.contributor.affiliationum The Neuromuscular Section, The University of Michigan Medical Center, Ann Arbor, MI 48109 en_US
dc.identifier.pmid 6087719 en_US
dc.description.bitstreamurl http://deepblue.lib.umich.edu/bitstream/2027.42/50308/1/410160107_ftp.pdf en_US
dc.identifier.doi http://dx.doi.org/10.1002/ana.410160107 en_US
dc.identifier.source Annals of Neurology en_US
dc.owningcollname Interdisciplinary and Peer-Reviewed
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