[Familial periodic paralysis. Description of a family. Aspects of pathogenesis and treatment of the disease].
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BACKGROUND: Hypokalemic periodic paralysis with thyrotoxicosis has never been described in pregnancy or the puerperium. CASE: A 31-year-old Hispanic woman underwent three prostaglandin inductions for a second-trimester missed abortion. Her management was complicated by hyperthermia, nausea, vomiting, and diarrhea. She developed isolated proximal muscle paralysis and sensory loss on the first post-abortion day. Her serum potassium was 1.5 mEq/L. The serum free thyroxine index exceeded 25 and TSH was below 0.03 microIU/mL, leading to a diagnosis of thyrotoxic hypokalemic periodic paralysis. Oral and parenteral potassium repletion restored full neurologic function, and propylthiouracil treatment was initiated until thyroid ablation could be performed. CONCLUSION: Gastrointestinal potassium loss during prostaglandin-induced abortion may unmask previously undiagnosed periodic paralysis.
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A missense variant (R83H) of the gene (KCNE3) encoding a potassium channel-associated peptide, MinK-related peptide 2 (MiRP2), has been reported in periodic paralysis patients. In the current study, no difference in the frequency of the MiRP2-R83H variant between periodic paralysis patients and healthy individuals was found. Furthermore, there was no segregation of this gene variant with the disease. These observations weaken the proposal that MiRP2-R83H causes periodic paralysis.
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A Latin-American teenager was begun on a regimen of acetazolamide for a presumptive diagnosis of familial periodic paralysis. He presented 2 weeks later with near total body paralysis and was found to have florid manifestations of Graves' disease. Although thyrotoxic periodic paralysis occurs most frequently in Oriental males, it occasionally is found in Caucasians. Unlike familial, euthyroid, and periodic paralysis, thyrotoxic periodic paralysis may be worsened by administration of acetazolamide. Physical signs and laboratory evidence of hyperthyroidism must be sought in cases of periodic paralysis to differentiate the two diseases.
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