Isaacs syndrome: a slow potassium channelopathy caused by autoantibodies?

Isaacs syndrome is the most well-known autoimmune peripheral nerve hyperexcitability (PNH) syndrome (Ahmed and Simmons, 2015). Clinically, patients with Isaacs syndrome are characterized by visible myokymia at rest and by doublet, triplet, or multiplet single unit discharges in needle electromyography (nEMG) (Newsom-Davis and Mills, 1993). Antibodies against voltage-gated potassium channels (VGKC), which suppress the function of fast potassium channels (Kv1), have been detected in some patients with the syndrome (approximately 38%–50%).

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