Course and outcome of a voltage-gated potassium channel antibody negative Morvan's syndrome.
Morvan's syndrome is a rare disease characterized by peripheral nerve hyperexcitability, associated with CNS and autonomic systems involvement. High serum voltage-gated potassium channel (VGKC) antibody titers have been reported, and, till now, Morvan's syndrome has been considered as a VGKC antibody associated disease. We describe a patient with Morvan's syndrome associated with myasthenia gravis and a thymoma in his previous history, with surprisingly undetectable levels of VGKC antibodies. The clinical course is similar to those cases of Morvan's syndrome with VGKC-Ab, except for the lack of response to plasma exchange, previously considered as the first choice treatment. Nevertheless, the good response to corticosteroids therapy and the association with myasthenia confirm an autoimmune origin of the disease.
Rinaldi
Carlo
C
Department of Neurological Sciences, Federico II University, Via Pansini, 5, 80131, Naples, Italy. rinaldi.ca@gmail.com
Russo
Cinzia Valeria
CV
Filla
Alessandro
A
De Michele
Giuseppe
G
Marano
Enrico
E
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D001323:Autoantibodies / Q000097:blood*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000097:blood / Q000150:complications* / Q000276:immunology; D020385:Myokymia / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000276:immunology*; D010951:Plasma Exchange; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D011241:Prednisone / Q000627:therapeutic use*; D019837:Ryanodine Receptor Calcium Release Channel / Q000276:immunology; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D024642:Potassium Channels, Voltage-Gated; D019837:Ryanodine Receptor Calcium Release Channel; D011241:Prednisone