Issacs Syndrome in a Pediatric Patient and Voltage-Gated Potassium Channels Antibodies

Abstract

Isaacs’ syndrome (IS), also known as acquired neuromyotonia, is a rare neuromuscular disease, manifested by involuntary continuous motor activity.1 Although there are reports in children,2,3 IS is more frequent in adults.1 The clinical presentation can include muscle cramps, fasciculations, myokymia, and pseudomyotonia. Electromyography (EMG) remains the gold standard for diagnosis.1,4 Dysfunction of peripheral nerve voltage-gated potassium channels (VGKC) appears to be related to the development of the disease.1,3,4,5 Paraneoplastic factors also play an important role in IS.5 Anticonvulsants are the first therapeutic option.1,4,5