PERIODIC FAMILIAL PARALYSIS WITH HYPOKALEMIA - HEMODYNAMIC AND METABOLIC STUDIES - FAVORABLE EFFECT OF ACETAZOLAMIDE

  • 1 January 1978
    • journal article
    • research article
    • Vol. 134  (11) , 655-672
Abstract
The same protocol was used 3 times to produce a paralytic episode in a typical case [human] of periodic familial paralysis with hypokalemia. This consisted of an effort with a perfusion of hypertonic glucose serum and insulin. The 1st test provoked at attack of hypokalemic tetraplegia. The 2nd test, 2 mo. after treatment with 500 mg daily of acetazolamide, produced no reaction. In the 3rd test, the metabolic acidosis caused by acetazolamide was reduced by the injection of sodium bicarbonate, and a stronger effect than in the 1st test was observed. This confirms the efficacy of acetazolamide as a preventive treatment for paralytic attacks; the most reasonable hypothesis being that it acts through the metabolic acidosis that it induces. Metabolic and hemodynamic studies were carried out during the provoked attacks. Cardiac output and O2 consumption are increased, while pulmonary capillary pressure and peripheral resistance are reduced. Diastolic pressure is lower when measured by an arm-cuff but shows no change when direct readings are taken in the blood vessels. These results suggest that there is an increase in cellular energy needs, or that the smooth muscle in the vessel walls is paralyzed. The 2 tetraplegic attacks in tests 1 and 3 were associated with a metabolic acidosis, which is explained by a simultaneous transfer, though in the opposite direction, of H+ and K+ ions; the intracellular pH was acid when there was not an attack, and this increased during paralysis.