Sequential antibodies to potassium channels and glutamic acid decarboxylase in neuromyotonia.
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.
Antozzi
C
C
Neurology Department IV, Immunology and Muscular Pathology Unit, National Neurologic Institute Carlo Besta, Milan, Italy. antozzi@istituto-besta.it
Frassoni
C
C
Vincent
A
A
Regondi
M C
MC
Andreetta
F
F
Bernasconi
P
P
Ciano
C
C
Chang
T
T
Cornelio
F
F
Spreafico
R
R
Mantegazza
R
R
D000328:Adult; D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D020274:Autoimmune Diseases of the Nervous System / Q000276:immunology* / Q000503:physiopathology; D001767:Blinking / Q000276:immunology; D001933:Brain Stem / Q000276:immunology / Q000503:physiopathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D020386:Isaacs Syndrome / Q000276:immunology* / Q000503:physiopathology; D051663:Kv1.2 Potassium Channel / Q000276:immunology; D051667:Kv1.6 Potassium Channel / Q000276:immunology; D008297:Male; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D051381:Rats; D012021:Reflex, Abnormal / Q000276:immunology; D013945:Thymoma / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D013953:Thymus Neoplasms / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D016896:Treatment Outcome; D005680:gamma-Aminobutyric Acid / Q000096:biosynthesis
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D051663:Kv1.2 Potassium Channel; D051667:Kv1.6 Potassium Channel; D024642:Potassium Channels, Voltage-Gated; D005680:gamma-Aminobutyric Acid; D003520:Cyclophosphamide; D005968:Glutamate Decarboxylase