title: Isaacs' syndrome in a patient with dermatomyositis: case report and review of the literature.
abstract: This is a case report of Isaacs' syndrome in dermatomyositis. The patient presented with proximal muscle weakness, rash, elevated muscle enzyme, myopathic electromyograph and typical muscle biopsy. Ultimately he developed typical symptoms of Isaacs' syndrome which is an autoimmune channelopathy from voltage gated potassium channel antibody (anti-VGKC) leading to dysfunction of axonal discharge at neuromuscular junctions. It shares some similar characteristics with dermatomyositis such as autoimmunity, its association with malignancy and the response to treatment.
authors.affiliation: Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Faculty of Medicine, Thammasat University, Rangsit, Thailand.
authors.forename: Ratchaya
authors.initials: R
authors.lastname: Lertnawapan
authors.affiliation: Division of Neurology, Department of Internal Medicine, Faculty of Medicine, Thammasat University, Rangsit, Thailand.
authors.forename: Kongkiat
authors.initials: K
authors.lastname: Kulkantrakorn
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood; D015551:Autoimmunity; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D003882:Dermatomyositis / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D004576:Electromyography; D006801:Humans; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D020386:Isaacs Syndrome / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000503:physiopathology; D008297:Male; D009043:Motor Activity; D053580:Muscle Strength; D009465:Neuromuscular Agents / Q000627:therapeutic use; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology; D020127:Recovery of Function; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D007166:Immunosuppressive Agents; D009465:Neuromuscular Agents; D024642:Potassium Channels, Voltage-Gated
keywords: Isaacs' syndrome; autoimmune channelopathy; dermatomyositis; myokymia; neuromyotonia