Abnormal neuromuscular transmission in an infantile myasthenic syndrome
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 | https://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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