| CASE REPORT
|Year : 2020 | Volume
| Issue : 2 | Page : 153-156
A rare case of peripheral nerve hyperexcitability in childhood: Isaacs syndrome
Seda Kanmaz1, Muhittin Ozcan2, Erdem Simsek1, Hepsen M Serin1, Ibrahim Aydogdu3, Sarenur Gokben1, Hasan Tekgul1
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
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.
Prof. Hasan Tekgul
Division of Pediatric Neurology, Department of Pediatrics, Ege University Medical School, Izmir.
Source of Support: None, Conflict of Interest: None
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