{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T12:31:04Z","timestamp":1773923464429,"version":"3.50.1"},"reference-count":19,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2012,12,4]],"date-time":"2012-12-04T00:00:00Z","timestamp":1354579200000},"content-version":"vor","delay-in-days":33,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"UILDM-Telethon","award":["GUP05001"],"award-info":[{"award-number":["GUP05001"]}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2012,11]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Fatigue and pain have been previously shown to be important determinants for decreasing quality of life (<jats:styled-content style=\"fixed-case\">Q<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>) in one report in patients with non\u2010dystrophic myotonia. The aims of our study were to assess <jats:styled-content style=\"fixed-case\">Q<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content> in skeletal muscle channelopathies (<jats:styled-content style=\"fixed-case\">SMC<\/jats:styled-content>) using <jats:styled-content style=\"fixed-case\">INQ<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content> (individualized <jats:styled-content style=\"fixed-case\">Q<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>) and <jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>\u201036 questionnaires.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We administered <jats:styled-content style=\"fixed-case\">INQ<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>\u201036 to 66 Italian patients with <jats:styled-content style=\"fixed-case\">SMC<\/jats:styled-content> (26: periodic paralysis, 36: myotonia congenita and 4: Andersen\u2010Tawil) and compared the results in 422 patients with myotonic dystrophies (<jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>1: 382; and <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>2: 40).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>(i) <jats:styled-content style=\"fixed-case\">INQ<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content> index in <jats:styled-content style=\"fixed-case\">SMC<\/jats:styled-content> is similar to that in <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>s (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.79). (ii) Patients with myotonia congenita have the worst perception of <jats:styled-content style=\"fixed-case\">Q<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>. (iii) Myotonia has the most detrimental effect on patients with myotonia congenita, followed by patients with <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>2 and then by patients with <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>1 and hyperkalemic periodic paralysis. (iv) Pain is a significant complaint in patients with myotonia congenita, hypokalemic periodic paralysis and <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>2 but not in <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>1. (v) Fatigue has a similar detrimental effect on all patient groups except for patients with hyperkalemic periodic paralysis in whom muscle weakness and myotonia more than fatigue affect <jats:styled-content style=\"fixed-case\">Q<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content> perception. (vi) Muscle symptoms considered in <jats:styled-content style=\"fixed-case\">INQ<\/jats:styled-content>oL correlate with physical symptoms assessed by <jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>\u201036 (<jats:styled-content style=\"fixed-case\">R<\/jats:styled-content> from \u22120.34 to \u22120.76).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>QoL perception in patients with <jats:styled-content style=\"fixed-case\">SMC<\/jats:styled-content> is similar to that of patients with <jats:styled-content style=\"fixed-case\">DM<\/jats:styled-content>s, chronic multisystem disabling conditions. Our results provide information to target treatment and health care of these patients. The sensitivity of <jats:styled-content style=\"fixed-case\">INQ<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content> to changes in <jats:styled-content style=\"fixed-case\">Q<\/jats:styled-content>o<jats:styled-content style=\"fixed-case\">L<\/jats:styled-content> in the <jats:styled-content style=\"fixed-case\">SMC<\/jats:styled-content> needs to be further explored in longitudinal studies.<\/jats:p><\/jats:sec>","DOI":"10.1111\/j.1468-1331.2012.03751.x","type":"journal-article","created":{"date-parts":[[2012,5,20]],"date-time":"2012-05-20T14:14:31Z","timestamp":1337523271000},"page":"1470-1476","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":64,"title":["Measuring quality of life impairment in skeletal muscle channelopathies"],"prefix":"10.1111","volume":"19","author":[{"given":"V. 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