Riassunto
La paralisi periodica ipopotassiemica (pp-IPO-k), la paralisi periodica iperpotassiemica (pp-IPER-k), la paramiotonia congenita (PMC) e la sindrome di Andersen-Tawil SAT) sono canalopatie ereditarie di tipo autosomico dominante caratterizzate dal ripetersi di fenomeni “critici” nel corso dei quali la potassiemia si discosta dal suo valore basale e il paziente presenta un disturbo motorio generalizzato o parcellare. In linea di massima, se la potassiemia scende, il disturbo motorio è di tipo deficitario (debolezza o paralisi); se aumenta, può essere di tipo deficitario e/o di tipo miotonico. Le variazioni della potassiemia possono essere anche minime e talvolta addirittura assenti. Ciò, secondo Mc Ardle [1], potrebbe dipendere anche dal fatto che il muscolo in crisi è tendenzialmente ipoperfuso e il suo liquido interstiziale subisce un relativo ristagno. Nei casi dubbi, l’induzione farmacologica con glucosio o con potassio consente di formulare la diagnosi di pp-IPO-k o, rispettivamente, di pp-IPER-k.
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Fiacchino, F. (2009). Paralisi periodiche e miopatia da deplezione potassica. In: Terapia delle malattie neurologiche. Springer, Milano. https://doi.org/10.1007/978-88-470-1120-5_19
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DOI: https://doi.org/10.1007/978-88-470-1120-5_19
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