Revisão Narrativa
Hypokalemic Periodic Paralysis: A Review of Pathophysiology, Clinical Features, and Treatment
Conteúdo principal do artigo
Sérgio Ferreira Cristina
Victor Espadinha
Resumo
A paralisia periódica hipocaliemica é uma canalopatia muscular esquelética genética rara caracterizada por ataques recorrentes de tetraparesia, associados a hipocaliemia. Mutações nos canais de sódio de cálcio do muscúlo esquelético são responsáveis pela fisiopatologia desta entidade e cada mutação aparente ter diferentes implicações tanto no fenótipo como na resposta ao tratamento. Os doentes com paralisia periódica hipocaliemica com fraqueza muscular e hipocaliemia recorrentes, frequentemente após exercício ou refeições copiosas, mas são assintomáticos entre os ataques. Apesar da administração de potássio ser o pilar do tratamento agudo, os inibidores da anidrase carbónica desempenham um papel significativo no tratamento profilático. Neste artigo, tivemos como objetivo rever a fisiopatologia, as características clínicas e as opções de tratamento da paralisia periódica hipocaliemica.
Detalhes do artigo
Este trabalho encontra-se publicado com a Creative Commons Atribuição-NãoComercial 4.0.
Referências
Jandhyala SN, Madireddi J, Belle J, Rau NR, Shetty R. Hypokalaemic Periodic Paralysis- A Prospective Study of the Underlying Etiologies. J Clin Diagn Res 2015;9(9):OC17-9. doi: 10.7860/JCDR/2015/13237.6529
Miller TM, Dias da Silva MR, Miller HA, Kwiecinski H, Mendell JR, Tawil R, et al. Correlating phenotype and genotype in the periodic paralyses. Neurology 2004;63(9):1647-55. doi: 10.1212/01.wnl.0000143383.91137.00
Wi JK, Lee HJ, Kim EY, Cho JH, Chin SO, Rhee SY, et al. Etiology of hypokalemic paralysis in Korea: data from a single center. Electrolyte Blood Press 2012;10(1):18-25. doi: 10.5049/EBP.2012.10.1.18
Horga A, Raja Rayan DL, Matthews E, Sud R, Fialho D, Durran SC, et al. Prevalence study of genetically defined skeletal muscle channelopathies in England. Neurology 2013;80(16):1472-5. doi: 10.1212/WNL.0b013e31828cf8d0
Statland JM, Fontaine B, Hanna MG, Johnson NE, Kissel JT, Sansone VA, et al. Review of the Diagnosis and Treatment of Periodic Paralysis. Muscle Nerve 2018;57(4):522-530. doi: 10.1002/mus.26009
Li J, Moten S, Rauf AA. The role of nephrologists in management of hypokalemic periodic paralysis: a case report. J Med Case Rep 2022;16(1):65. doi: 10.1186/s13256-022-03283-0
Ke Q, Luo B, Qi M, Du Y, Wu W. Gender differences in penetrance and phenotype in hypokalemic periodic paralysis. Muscle Nerve 2013;47(1):41-5. doi: 10.1002/mus.23460
Tricarico D, Camerino DC. Recent advances in the pathogenesis and drug action in periodic paralyses and related channelopathies. Front Pharmacol 2011;2:8. doi: 10.3389/fphar.2011.00008
Kubota T, Wu F, Vicart S, Nakaza M, Sternberg D, Watanabe D, et al. Hypokalaemic periodic paralysis with a charge-retaining substitution in the voltage sensor. Brain Commun 2020;2(2):fcaa103. doi: 10.1093/braincomms/fcaa103
Levitt JO. Practical aspects in the management of hypokalemic periodic paralysis. J Transl Med 2008;6:18. doi: 10.1186/1479-5876-6-18
Ruff RL. Insulin acts in hypokalemic periodic paralysis by reducing inward rectifier K+ current. Neurology 1999;53(7):1556-63. doi: 10.1212/wnl.53.7.1556
Tricarico D, Capriulo R, Conte Camerino D. Insulin modulation of ATP-sensitive K+ channel of rat skeletal muscle is impaired in the hypokalaemic state. Pflugers Arch 1999;437(2):235-40. doi: 10.1007/s004240050774
Welland NL, Hæstad H, Fossmo HL, Giltvedt K, Ørstavik K, Nordstrøm M. The Role of Nutrition and Physical Activity as Trigger Factors of Paralytic Attacks in Primary Periodic Paralysis. J Neuromuscul Dis 2021;8(4):457-468. doi: 10.3233/JND-200604
Palmer BF, Clegg DJ. Physiology and Pathophysiology of Potassium Homeostasis: Core Curriculum 2019. Am J Kidney Dis 2019;74(5):682-695. doi: 10.1053/j.ajkd.2019.03.427
Sansone VA, Ricci C, Montanari M, Apolone G, Rose M, Meola G, et al. Measuring quality of life impairment in skeletal muscle channelopathies. Eur J Neurol 2012;19(11):1470-6. doi: 10.1111/j.1468-1331.2012.03751.x
Matthews E, Hanna MG. Muscle channelopathies: does the predicted channel gating pore offer new treatment insights for hypokalaemic periodic paralysis? J Physiol 2010;588(Pt 11):1879-86. doi: 10.1113/jphysiol.2009.186627
Tengan CH, Antunes AC, Gabbai AA, Manzano GM. The exercise test as a monitor of disease status in hypokalaemic periodic paralysis. J Neurol Neurosurg Psychiatry 2004;75(3):497-9. doi: 10.1136/jnnp.2003.013870
Griggs RC, Resnick J, Engel WK. Intravenous treatment of hypokalemic periodic paralysis. Arch Neurol 1983;40(9):539-40. doi: 10.1001/archneur.1983.04050080039005
Sansone VA, Burge J, McDermott MP, Smith PC, Herr B, Tawil R, et al. Randomized, placebo-controlled trials of dichlorphenamide in periodic paralysis. Neurology 2016;86(15):1408-1416. doi: 10.1212/WNL.0000000000002416
Matthews E, Portaro S, Ke Q, Sud R, Haworth A, Davis MB, et al. Acetazolamide efficacy in hypokalemic periodic paralysis and the predictive role of genotype. Neurology 2011;77(22):1960-4. doi: 10.1212/WNL.0b013e31823a0cb6
Sansone VA, Johnson NE, Hanna MG, Ciafaloni E, Statland JM, Shieh PB, et al. Long-term efficacy and safety of dichlorphenamide for treatment of primary periodic paralysis. Muscle Nerve 2021;64(3):342-346. doi: 10.1002/mus.27354
Desaphy JF, Altamura C, Vicart S, Fontaine B. Targeted Therapies for Skeletal Muscle Ion Channelopathies: Systematic Review and Steps Towards Precision Medicine. J Neuromuscul Dis 2021;8(3):357-381. doi: 10.3233/JND-200582
Dalakas MC, Engel WK. Treatment of "permanent" muscle weakness in familial Hypokalemic Periodic Paralysis. Muscle Nerve1983;6(3):182-6. doi: 10.1002/mus.880060303
