A case of Isaacs' syndrome with associated central nervous system findings

We report a 44-year-old female with Isaacs' syndrome, peripheral motor neuropathy, and features of central pontine myelinolysis (CPM). The patient presented with stiffness and muscle spasms accompanied by profound sweating. She also had bilateral Babinski signs. Electrodiagnostic abnormalities were characteristic of Isaacs' syndrome. Magnetic resonance imaging demonstrated features of CPM. She gained modest relief from baclofen, valproate, and diazepam. She improved dramatically following plasmapheresis and continued to recover on prednisone. She was weaned from steroids without relapse.