{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T15:28:51Z","timestamp":1780414131412,"version":"3.54.1"},"reference-count":18,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2011,3,8]],"date-time":"2011-03-08T00:00:00Z","timestamp":1299542400000},"content-version":"vor","delay-in-days":35,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[2011,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To improve the accuracy of genotype prediction and guide genetic testing in patients with muscle channelopathies we applied and refined specialized electrophysiological exercise test parameters.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We studied 56 genetically confirmed patients and 65 controls using needle electromyography, the long exercise test, and short exercise tests at room temperature, after cooling, and rewarming.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Concordant amplitude\u2010and\u2010area decrements were more reliable than amplitude\u2010only measurements when interpreting patterns of change during the short exercise tests. Concordant amplitude\u2010and\u2010area pattern I and pattern II decrements of &gt;20% were 100% specific for paramyotonia congenita and myotonia congenita, respectively. When decrements at room temperature and after cooling were &lt;20%, a repeat short exercise test after rewarming was useful in patients with myotonia congenita. Area measurements and rewarming distinguished true temperature sensitivity from amplitude reduction due to cold\u2010induced slowing of muscle fiber conduction. In patients with negative short exercise tests, symptomatic eye closure myotonia predicted sodium channel myotonia over myotonia congenita. Distinctive \u201ctornado\u2010shaped\u201d neuromyotonia\u2010like discharges may be seen in patients with paramyotonia congenita. In the long exercise test, area decrements from pre\u2010exercise baseline were more sensitive than amplitude decrements\u2010from\u2010maximum\u2013compound muscle action potential (CMAP) in patients with Andersen\u2010Tawil syndrome. Possible ethnic differences in the normative data of the long exercise test argue for the use of appropriate ethnically\u2010matched controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Concordant CMAP amplitude\u2010and\u2010area decrements of &gt;20% allow more reliable interpretation of the short exercise tests and aid accurate DNA\u2010based diagnosis. In patients with negative exercise tests, specific clinical features are helpful in differentiating sodium from chloride channel myotonia. A modified algorithm is suggested. Ann Neurol 2011<\/jats:p><\/jats:sec>","DOI":"10.1002\/ana.22238","type":"journal-article","created":{"date-parts":[[2011,3,8]],"date-time":"2011-03-08T17:46:01Z","timestamp":1299606361000},"page":"328-340","source":"Crossref","is-referenced-by-count":90,"title":["Refined exercise testing can aid dna\u2010based diagnosis in muscle channelopathies"],"prefix":"10.1002","volume":"69","author":[{"given":"S. Veronica","family":"Tan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Matthews","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa","family":"Barber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James A.","family":"Burge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanjeev","family":"Rajakulendran","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doreen","family":"Fialho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richa","family":"Sud","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Haworth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Koltzenburg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael G.","family":"Hanna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,3,8]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20241"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20905"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1002\/1097-4598(200007)23:7<1089::AID-MUS12>3.0.CO;2-Q"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20856"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880090805"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2003.013870"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880180418"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20865"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.56.3.352"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1097\/00029330-200810010-00012"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880100704"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880070709"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880050909"},{"key":"e_1_2_7_15_2","first-page":"315","article-title":"Paramyotonia congenita: clinical and electrophysiologic studies","volume":"23","author":"Streib EW","year":"1983","journal-title":"Electromyogr Clin Neurophysiol"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2008.01.007"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000143383.91137.00"},{"key":"e_1_2_7_18_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-83-1-63"},{"key":"e_1_2_7_19_2","doi-asserted-by":"publisher","DOI":"10.1089\/thy.1994.4.441"}],"container-title":["Annals of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fana.22238","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ana.22238","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,16]],"date-time":"2023-10-16T18:43:21Z","timestamp":1697481801000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ana.22238"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,2]]},"references-count":18,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2011,2]]}},"alternative-id":["10.1002\/ana.22238"],"URL":"https:\/\/doi.org\/10.1002\/ana.22238","archive":["Portico"],"relation":{},"ISSN":["0364-5134","1531-8249"],"issn-type":[{"value":"0364-5134","type":"print"},{"value":"1531-8249","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,2]]}}}