Kanmaz, SedaOzcan, MuhittinSimsek, ErdemSerin, Hepsen M.Aydogdu, IbrahimGokben, SarenurTekgul, Hasan2020-12-012020-12-0120201817-17451998-3948https://doi.org/10.4103/jpn.JPN_128_19https://hdl.handle.net/11454/62493Isaacs syndrome is rare disorder with peripheral nerve hyperexcitability syndromes with acquired neuromyotonia in childhood. We present a 13-year-old girl with muscle stiffness and neuromyotonia diagnosed Isaac syndrome with spontaneous discharge potentials on motor unit in electromyography and the diagnosis supported by the presence of antinuclear antibodies. A successful treatment was obtained using low-dose carbamazepine. Cause of Isaacs syndrome is unknown, generally thought to be an autoimmune etiology with voltage-gated potassium channelopathy; it sometimes occurs as a paraneoplastic syndrome. Early use of electromyography has critical role in the differential diagnosis with certain muscle disorders and peripheral nerve hyperexcitability syndromes.en10.4103/jpn.JPN_128_19info:eu-repo/semantics/openAccessIsaacs syndromeneuromyotoniaperipheral nerve hyperexcitabilitypseudomyotoniaArticle152153156WOS:0005499316000202-s2.0-85092000357Q3N/A