Atypical arrhythmic complications in familial hypokalemic periodic paralysis

Journal of Cardiovascular Medicine

Volumn 10, Issue 1, 2009, Pages 68-71

  Maffè, Stefano ^a,c   Signorotti, Fabiana ^a   Perucca, Antonello ^a   Bielli, Massimo ^a   Hladnik, Uros ^b   Ragazzoni, Elena ^a   Maduli, Elisabetta ^a   Paffoni, Paola ^a   Dellavesa, Pierfranco ^a   Paino, Anna Maria ^a   Zenone, Franco ^a   Parravicini, Umberto ^a   Pardo, Nicolò Franchetti ^a   Cucchi, Lorenzo ^a   Zanetta, Marco ^a  

^a Borgomanero Hospital   (Italy)

^b Baschirotto Institute for Rare Diseases   (Italy)

^c NONE   (Italy)

Author keywords

Genetic mutation; Hypokalemia; Paralysis; Sinus arrest

Indexed keywords

ANTIHISTAMINIC AGENT; ATROPINE; CARBOHYDRATE; CORTISONE; POTASSIUM;

ADULT; ARTICLE; ARTIFICIAL HEART PACEMAKER; BREATHING MUSCLE; CARBOHYDRATE INTAKE; CASE REPORT; CIRCADIAN RHYTHM; ELECTROCARDIOGRAM; EXERCISE; GENE; GENE MUTATION; GENETIC ANALYSIS; HEART ARRHYTHMIA; HEART RATE; HEART RIGHT BUNDLE BRANCH BLOCK; HUMAN; HYPOKALEMIA; INSECT STING; LABORATORY TEST; LIMB WEAKNESS; MALE; MUSCLE CRAMP; PERIODIC PARALYSIS; PHYSICAL EXAMINATION; QT PROLONGATION; QUADRIPLEGIA; SCN4A GENE; SINUS ARREST; SINUS ARRHYTHMIA; SINUS BRADYCARDIA; STRENGTH; SYNCOPE; TENDON REFLEX;

ADULT; BRADYCARDIA; CARDIAC PACING, ARTIFICIAL; DNA MUTATIONAL ANALYSIS; ELECTROCARDIOGRAPHY; HEART RATE; HUMANS; MALE; MUTATION; PACEMAKER, ARTIFICIAL; PARALYSIS, HYPERKALEMIC PERIODIC; POTASSIUM COMPOUNDS; SINUS ARREST, CARDIAC; SODIUM CHANNELS; SYNCOPE; TREATMENT OUTCOME;

EID: 67650881934     PISSN: 15582027     EISSN: None     Source Type: Journal    
DOI: 10.2459/JCM.0b013e3283189564     Document Type: Article

References (16)

1. Ahlawat SK, Sadchev A. Classic disease revisited: hypokalemic paralysis. Postgrad Med J 1999; 75:193-197.
2. Lapie P, Lory P, Fontaine B. Hypokalemic periodic paralysis: an autosomal dominant muscle disorder caused by mutations in a voltage-gated calcium channel. Neuromuscul Disord 1997; 7:234-240.
3. 2+ ions in excitation-contraction coupling of skeletal muscle fibres. Biochim Biophys Acta 1995; 1241:59-116.
4. Jurkat-Rott K, Mitrovic N, Hang C, Kouzmekine A, Iaizzo P, Herzog J, et al. Voltage-sensor sodium channel mutations cause hypokalemic periodic paralysis type 2 by enhanced inactivation and reduced current. Proc Natl Acad Sci U S A 2000; 97:9549-9554.
5. Kuzmenkin A, Muncan V, Jurkat-Rott K, Hang C, Lerche H, Lehmann-Horn F, Mitrovic N. Enhanced inactivation and pH sensitivity of Na+ channel mutations causing hypokalemic periodic paralysis type II. Brain 2002; 125:835-843.
6. Struyk AF, Scoggan KA, Bulman DE, Cannon SC. The human skeletal muscle Na channel mutation R669H associated with hypokalemic periodic paralysis enhances slow inactivation. J Neurosci 2000; 20:8610-8617.
7. Wang Q, Shen J, Splawski I, Atkinson D, Li Z, Robinson JL, et al. SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome. Cell 1995; 80:805-811.
8. Schott JJ, Alshinawi C, Kyndt F, Probst V, Hoorntje TM, Hulsbeek M, et al. Cardiac conduction defects associate with mutations in SCN5A. Nat Genet 1999; 23:20-21.
9. Wang DW, Viswanathan PC, Balser JR, George AL, Benson DW. Clinical, genetic, and biophysical characterization of SCN5A mutations associated with atrioventricular conduction block. Circulation 2002; 105:341-346.
10. Pereon Y, Lande G, Demolombe S, Tich SNT, Sternberg D, Le Marec H, David A. Paramyotonia congenita with an SCN4A mutation affecting cardiac repolarization. Neurology 2003; 60:340-342.
11. Webster A, BradyW,Morris F.Recognising signs of danger: EKG changes resulting from an abnormal serum potassium concentration. Emerg Med J 2002; 19:74-77.
12. Kung AW. Thyrotoxic periodic paralysis: a diagnostic challenge. J Clin Endocrinol Metab 2006; 91:2490-2495.
13. Hsu YJ, Lin YF, Chau T, Liou JT, Kuo SW, Lin SH. Electrocardiographic manifestations in patients with thyrotoxic periodic paralysis. Am J Med Sci 2003; 326:128-132.
14. Kantola IM, Tarssanen LT. Familial hypokalaemic periodic paralysis in Finland. J Neurol Neurosurg Psychiatry 1992; 55:322-324.
15. Johnsen T. Family Periodic Paralysis with Hypokalaemia. Danish Medical Bulletin 1981; 28:1-27.
16. Moneret-Vautrin DA, de Chillou C, Codreanu A. Long QT syndrome in a patient with allergic rhinoconjunctivitis and auto-immune diabetes: focus on the choice of anti-H1 drugs. Allerg Immunol (Paris) 2006; 38:347-350.