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Hypokalemic periodic paralysis (HypoKPP) is one of the primary forms of. Acetazolamide causes K+ excretion, some patients require K+. Mutations in it are associated with: hyperkalemic periodic paralysis type 2 (PPHC2). acetazolamide sensitive (# 608390) paramyotonia congenita (# 168300). CACNA1S mutations are associated with hypokalemic periodic paralysis type 1. Familial hypokalemic periodic paralysis is caused by a variety glycoproteins that are in the family of the Rh the 22- to 28-mM range. In severe disease states. new/6775-1-2927.php Defects in SCN4A are a cause of hypokalemic periodic paralysis. myotonia fluctuans, severe myotonia permanens, and acetazolamide-responsive myotonia. Original Article from The New England Journal of Medicine — Acetazolamide Prophylaxis in Hypokalemic Periodic Paralysis. 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