| Main clinical features
|
associated with a secundary reduced sarcolemma KATPs current
episodic weakness accompanied by low serum potassium levels, aborted by administration of potassium or by exercise and precipitated by insulin or glucose administration (oral intake of KCl is effective in blocking the symptomatic attacks)
hypokalemic periodic paralysis shows markedly reduced penetrance in females, although penetrance is 100% in males
in muscle fibers of patients, the basic defects are a reduced excitability and an increased sodium conductance, and these defects are aggravated by reduction of the extracellular potassium concentration |