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Year : 2020  |  Volume : 15  |  Issue : 2  |  Page : 153-156

A rare case of peripheral nerve hyperexcitability in childhood: Isaacs syndrome

1 Department of Pediatrics, Division of Child Neurology, Ege University Medical Faculty, Izmir, Turkey
2 Department of Pediatrics, Ege University Medical Faculty, Izmir, Turkey
3 Department of Neurology, Ege University Medical Faculty, Izmir, Turkey

Correspondence Address:
Prof. Hasan Tekgul
Division of Pediatric Neurology, Department of Pediatrics, Ege University Medical School, Izmir.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_128_19

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Isaacs 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.


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