Abstract

Neuromyotonia is a neuromuscular hyperexitability disorder characterized by muscle stiffness caused by continuous muscle fiber activity. It is an immune mediated disorder with elevated antibody level against presynaptic, voltage gated potassium channels, either as isolation or as a paraneoplastic process. Symptoms usually include muscle twitching during rest (myokymia), cramps, peudomyotonia (delayed relaxation), increased sweating, and sometimes motor weakness. In this case report, we present a seventy-two years old man who presented with pain in both thighs for one month. It gradually became worse to involve feet and chest. His brain CT scan showed features of brain atrophy. EMG showed fasciculation along neuromyotonic discharges with characteristic wave in frequency and amplitude typical of Isaacs’ syndrome. Potassium channel antibodies were very high. Diagnosis of Isaacs’ syndrome was made. He was followed up for two months with treatment by three-day course of methyl prednisolone followed by oral steroid and methotrexate with much improvement. This is the first case of Isaacs’ syndrome in Kurdistan.

Highlights

  • Isaacs’ syndrome is a neuromuscular disorder characterized by muscle stiffness caused by continuous muscle

  • The first case of Isaacs’ syndrome was diagnosed in 1961 when two men presented with persistent, generalized muscle stiffness, in addition to electromyography which showed spontaneous, rapid discharges of motor-unit potentials [4]

  • Radioimmunoassay of radioisotope labeled alpha-dendrotoxin-VGKCs solubilized from rabbit brain was the first method to identify antibodies to Voltage-Gated Potassium Channels (VGKC)

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Summary

Introduction

Isaacs’ syndrome is a neuromuscular disorder characterized by muscle stiffness caused by continuous muscle. How to cite this paper: Saeed, A.A., Mohammed, Z.A. and Saeed, R.R. (2016) Case Report: A 72 Years Old Man with Isaacs’ Syndrome: A Rare Entity with Different Outcomes. Voltage-gated potassium channels (VGKCs) are the targets [2]. Hyper excitability of the peripheral nerve can be induced by antibodies suppressing voltage-gated outward K(+) currents [3]. The first case of Isaacs’ syndrome ( known as acquired neuromyotonia) was diagnosed in 1961 when two men presented with persistent, generalized muscle stiffness, in addition to electromyography which showed spontaneous, rapid discharges of motor-unit potentials [4]

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