Sequential antibodies to potassium channels and glutamic acid decarboxylase in neuromyotonia

• Published in: Neurology Vol. 64; no. 7; pp. 1290 - 1293
• Main Authors: ANTOZZI, C, FRASSONI, C, MANTEGAZZA, R, VINCENT, A, REGONDI, M. C, ANDREETTA, F, BERNASCONI, P, CIANO, C, CHANG, T, CORNELIO, F, SPREAFICO, R
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 12-04-2005
• Subjects: Adult; Animals; Autoantibodies - immunology; Autoimmune Diseases of the Nervous System - immunology; Autoimmune Diseases of the Nervous System - physiopathology; Biological and medical sciences; Blinking - immunology; Brain Stem - immunology; Brain Stem - physiopathology; Cyclophosphamide - therapeutic use; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Disorders of higher nervous function. Focal brain diseases. Central vestibular syndrome and deafness. Brain stem syndromes; gamma-Aminobutyric Acid - biosynthesis; Glutamate Decarboxylase - immunology; Humans; Immunosorbent Techniques; Immunosuppressive Agents - therapeutic use; Isaacs Syndrome - immunology; Isaacs Syndrome - physiopathology; Kv1.2 Potassium Channel - immunology; Kv1.6 Potassium Channel - immunology; Male; Medical sciences; Nervous system (semeiology, syndromes); Neurology; Potassium Channels, Voltage-Gated - immunology; Rats; Reflex, Abnormal - immunology; Thymoma - complications; Thymoma - immunology; Thymoma - physiopathology; Thymus Neoplasms - complications; Thymus Neoplasms - immunology; Thymus Neoplasms - physiopathology; Treatment Outcome; Glutamic acid; Carbon-carbon lyases; Decarboxylase; Nervous system diseases; Enzyme; Carboxy-lyases; Lyases; Potassium
• ISSN: 0028-3878; 1526-632X
• DOI: 10.1212/01.WNL.0000156945.39471.2C

A patient with thymoma-associated neuromyotonia and voltage-gated potassium channel (Kv1.2 and Kv1.6) antibodies by immunoprecipitation and rat brain immunolabeling was treated successfully with immunoadsorption and cyclophosphamide. Curiously, glutamic acid decarboxylase antibodies, absent at onset, appeared later. Stiff-person syndrome was absent, but fast blink reflex recovery suggested enhanced brainstem excitability. The range of antibodies produced in thymoma-associated neuromyotonia is richer, and the timing of antibody appearance more complex, than previously suspected.