Téma:  Case Studies

Acquired neuromyotonia – autoimmune channelopathy

Peter Špalek, Ivan Martinka, Ľubomír Lisý

Neuromyotonia is a rare disorder of spontaneous and continuous muscle fibre activity of peripheral nerve origin. There are two main forms of neuromyotonia – inherited and acquired. Most acquired cases of neuromyotonia have autoimmune origin with autoantibodies against voltage-gated potassium channels (VGKC) that are present on the presynaptic motor nerve terminals (autoimmune channelopathy). Loss of these VGKCs leads to prolonged depolarisation, prolonged opening of calcium channels and excess acetylcholine release. We report a 30-year-old male patient with 4 year-history of myokymia, muscle stiffness, muscle cramps, delayed relaxation after contraction (pseudomyotonia) and hyperhidrosis, brought on by exercise, later the complaints have been continuous. A spontaneous burst of single motor-unit activity and neuromyotonic discharges to 170 Hz were the most characteristic features found in electromyography studies. The diagnosis of acquired neuromyotonia was confirmed by evidence of serum antibodies against VGKCc (266 pmol/l). Plasma exchange and combined long-term immunosuppressive therapy (prednisone and cyclophosphamide) resulted in marked and sustained improvement.

Ročník 2008  Témy časopisu Neurology 3 / 2008

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