Abstract

This article shows that acute episodes of limb weakness mimicking periodic paralysis that dramatically respond to acetazolamide may be due to homoplasmic mitochondrial DNA MT-ATP6/8 mutations. It is important for diagnosis because of the maternal inheritance of these diseases. Further, the findings illustrate mitochondrial effect on plasma membrane ion transport. See p. 1810 From editorialists Ruff & Cannon: “…increasing inward rectifier potassium channel current may be the mechanism of action of acetazolamide-induced improvement of symptoms in hypokalemic periodic paralysis.” See p. 1806 The authors recruited 54 adult patients with mitochondrial …

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