{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,2,13]],"date-time":"2023-02-13T10:09:55Z","timestamp":1676282995921},"reference-count":17,"publisher":"FapUNIFESP (SciELO)","issue":"2a","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arq. Neuro-Psiquiatr."],"published-print":{"date-parts":[[2006,6]]},"abstract":"<jats:p>A s\u00edndrome do canal lento \u00e9 uma das s\u00edndromes miast\u00eanicas cong\u00eanitas atribu\u00edda a desordem din\u00e2mica do canal i\u00f4nico do receptor de acetilcolina da jun\u00e7\u00e3o neuromuscular. Descrevemos o caso de um homem de 25 anos com progressiva ptose palpebral e limita\u00e7\u00e3o da movimenta\u00e7\u00e3o ocular desde inf\u00e2ncia, que evoluiu h\u00e1 6 anos com piora da oftalmoparesia externa e diminui\u00e7\u00e3o da for\u00e7a muscular em ombros e m\u00e3os. O estudo da condu\u00e7\u00e3o nervosa motora ap\u00f3s est\u00edmulo \u00fanico demonstrou duplo potencial de a\u00e7\u00e3o muscular composto (PAMC) com desaparecimento do segundo ap\u00f3s esfor\u00e7o de 30 segundos. Ao est\u00edmulo repetitivo dos nervos facial e acess\u00f3rio observou-se um decremento da amplitude do PAMC maior que 10% com desaparecimento do segundo potencial. O paciente fez uso de fluoxetina mostrando discreta melhora da for\u00e7a muscular, por\u00e9m persiste com: ptose palpebral, limita\u00e7\u00e3o dos movimentos oculares e PAMC repetitivo ao estudo da condu\u00e7\u00e3o nervosa motora. As caracter\u00edsticas da doen\u00e7a s\u00e3o discutidas.<\/jats:p>","DOI":"10.1590\/s0004-282x2006000200028","type":"journal-article","created":{"date-parts":[[2006,8,2]],"date-time":"2006-08-02T13:56:37Z","timestamp":1154526997000},"page":"318-321","source":"Crossref","is-referenced-by-count":4,"title":["Estudo neurofisiol\u00f3gico na s\u00edndrome miast\u00eanica cong\u00eanita do canal lento: relato de caso"],"prefix":"10.1590","volume":"64","author":[{"given":"Paulo Jos\u00e9","family":"Lorenzoni","sequence":"first","affiliation":[{"name":"Universidade Federal do Paran\u00e1, Brasil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cl\u00e1udia Suely Kamoi","family":"Kay","sequence":"additional","affiliation":[{"name":"Universidade Federal do Paran\u00e1, Brasil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Walter Oleschko","family":"Arruda","sequence":"additional","affiliation":[{"name":"Universidade Federal do Paran\u00e1, Brasil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosana Herminia","family":"Scola","sequence":"additional","affiliation":[{"name":"Universidade Federal do Paran\u00e1, Brasil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lineu C\u00e9sar","family":"Werneck","sequence":"additional","affiliation":[{"name":"Universidade Federal do Paran\u00e1, Brasil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"530","reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1212\/WNL.45.5.982","article-title":"A leucine-to-phenylalanine substituition in the acetylcholine receptor ion channel in a family with the slow-channel syndrome","volume":"45","author":"Gomez CM","year":"1995","journal-title":"Neurology","ISSN":"http:\/\/id.crossref.org\/issn\/0028-3878","issn-type":"print"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.1002\/(SICI)1097-4598(199609)19:9<1127::AID-MUS7>3.0.CO;2-1","article-title":"Repetitive CMAPs: mechanisms of neural and synaptic genesis","volume":"19","author":"Van Dijk JG","year":"1996","journal-title":"Muscle Nerve","ISSN":"http:\/\/id.crossref.org\/issn\/0148-639X","issn-type":"print"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1002\/ana.410110603","article-title":"A newly recognized congenital myasthenic syndrome attributed to a prolonged open time of the acetylcholine-induced ion channel","volume":"11","author":"Engel AG","year":"1982","journal-title":"Ann Neurol","ISSN":"http:\/\/id.crossref.org\/issn\/0364-5134","issn-type":"print"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1016\/S0733-8619(18)30104-X","article-title":"Congenital myasthenic syndromes","volume":"12","author":"Engel AG","year":"1994","journal-title":"Neurol Clin","ISSN":"http:\/\/id.crossref.org\/issn\/0733-8619","issn-type":"print"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"1710","DOI":"10.1212\/01.WNL.0000061483.11417.1B","article-title":"Treatment of slow-channel congenital myasthenic syndrome with fluoxetine","volume":"60","author":"Harper CM","year":"2003","journal-title":"Neurology","ISSN":"http:\/\/id.crossref.org\/issn\/0028-3878","issn-type":"print"},{"issue":"S5","key":"ref6","doi-asserted-by":"crossref","first-page":"S28","DOI":"10.1212\/WNL.48.Suppl_5.28S","article-title":"Congenital myasthenic syndrome caused by mutations in acetylcholine receptor genes","volume":"48","author":"Engel AG","year":"1997","journal-title":"Neurology","ISSN":"http:\/\/id.crossref.org\/issn\/0028-3878","issn-type":"print"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1001\/archneur.56.2.163","article-title":"Congenital myasthenic syndrome: recent advances","volume":"56","author":"Engel AG","year":"1999","journal-title":"Arch Neurol","ISSN":"http:\/\/id.crossref.org\/issn\/0003-9942","issn-type":"print"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1002\/mus.10269","article-title":"Congenital myasthenic syndromes: progress over the past decade","volume":"27","author":"Engel AG","year":"2003","journal-title":"Muscle Nerve","ISSN":"http:\/\/id.crossref.org\/issn\/0148-639X","issn-type":"print"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"712","DOI":"10.1002\/ana.410390607","article-title":"A b-subunit mutation in the acetylcholine receptor channel gate causes severe slow-channel syndrome","volume":"39","author":"Gomez CM","year":"1996","journal-title":"Ann Neurol","ISSN":"http:\/\/id.crossref.org\/issn\/0364-5134","issn-type":"print"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1002\/ana.10077","article-title":"Novel delta subunit mutation in slow-channel syndrome causes severe weakness by novel mechanisms","volume":"51","author":"Gomez CM","year":"2002","journal-title":"Ann Neurol","ISSN":"http:\/\/id.crossref.org\/issn\/0364-5134","issn-type":"print"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1073\/pnas.92.3.758","article-title":"Congenital myasthenic syndrome caused by prolonged acetylcholine receptor channel openings due to a mutation in the M2 domain of the epsilon subunit","volume":"92","author":"Ohno K","year":"1995","journal-title":"Proc Natl Acad Sci USA","ISSN":"http:\/\/id.crossref.org\/issn\/0027-8424","issn-type":"print"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/0896-6273(95)90080-2","article-title":"Mutation of the acetylcholine receptor a subunit causes alpha slow-channel myasthenic syndrome by enhancing agonist binding affinity","volume":"15","author":"Sine SM","year":"1995","journal-title":"Neuron","ISSN":"http:\/\/id.crossref.org\/issn\/0896-6273","issn-type":"print"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"1582","DOI":"10.1002\/1097-4598(200010)23:10<1582::AID-MUS16>3.0.CO;2-H","article-title":"Acdquired slow-channel syndrome","volume":"23","author":"Scola RH","year":"2000","journal-title":"Muscle Nerve","ISSN":"http:\/\/id.crossref.org\/issn\/0148-639X","issn-type":"print"},{"key":"ref14","series-title":"Pediatric clinical electromyography","first-page":"372","volume-title":"Neuromuscular transmission disorders in childhood","author":"Harper CM","year":"1996"},{"key":"ref15","series-title":"Electrodiagnostic medicine","first-page":"1197","volume-title":"Neuromuscular junction disorders","author":"Amato AA","year":"2002","edition":"2nd"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1002\/ana.410440412","article-title":"Acquired slowchannel syndrome: a form of myasthenia gravis with prolonged open time of the acetylcholine receptor channel","volume":"44","author":"Wintzen AR","year":"1998","journal-title":"Ann Neurol","ISSN":"http:\/\/id.crossref.org\/issn\/0364-5134","issn-type":"print"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1002\/ana.410430411","article-title":"Quinidine sulfate therapy for the slow-channel congenital myasthenic syndrome","volume":"43","author":"Harper CM","year":"1998","journal-title":"Ann Neurol","ISSN":"http:\/\/id.crossref.org\/issn\/0364-5134","issn-type":"print"}],"container-title":["Arquivos de Neuro-Psiquiatria"],"original-title":[],"link":[{"URL":"http:\/\/www.scielo.br\/pdf\/anp\/v64n2a\/a28v642a.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,4,20]],"date-time":"2019-04-20T09:31:44Z","timestamp":1555752704000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.scielo.br\/scielo.php?script=sci_arttext&pid=S0004-282X2006000200028&lng=pt&tlng=pt"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,6]]},"references-count":17,"journal-issue":{"issue":"2a","published-print":{"date-parts":[[2006,6]]}},"alternative-id":["S0004-282X2006000200028"],"URL":"https:\/\/doi.org\/10.1590\/s0004-282x2006000200028","relation":{},"ISSN":["0004-282X"],"issn-type":[{"value":"0004-282X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,6]]}}}