A rare case of peripheral nerve hyperexcitability in childhood: Isaacs syndrome
Articles > Article · 2020
Authors: Seda KANMAZ, Muhittin Ozcan, ERDEM ŞİMŞEK, Hepsen Mine SERİN, İbrahim AYDOĞDU, SARENUR GÖKBEN, Hasan TEKGÜL
JOURNAL OF PEDIATRIC NEUROSCIENCES, Journal Indexed in SCI Expanded, International, 2020, vol 15, pp 153-153
Summary
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.