eMedicine Specialties > Neurology > Neuromuscular Diseases

Periodic Paralyses: Follow-up

Author: Naganand Sripathi, MD, Director, Neuromuscular Clinic, Department of Neurology, Henry Ford Hospital
Contributor Information and Disclosures

Updated: Aug 26, 2009

Follow-up

Prognosis

  • Hyperkalemic periodic paralyses and paramyotonia congenita
    • When not associated with weakness, these usually do not interfere with ability to work.
    • Myotonia may require treatment.
    • Life expectancy is not known to be affected.
  • Hypokalemic periodic paralyses
    • Untreated patients may experience fixed proximal weakness, which may interfere with activities.
    • Several deaths have been reported, mostly related to aspiration pneumonia or inability to clear secretions.

Patient Education

  • Periodic Paralysis Resource Center (PPRC): This is the official Web site of the Periodic Paralysis Association.
  • Periodic Paralysis (PP): A Web site of the Muscular Dystrophy association. Articles from Quest magazine provide information regarding PP. The Research Digest link provides references on the causes and treatments.

Miscellaneous

Medicolegal Pitfalls

  • The major medicolegal risks are misdiagnosis or delayed diagnosis. However, PP can be notoriously difficult to diagnose, and typically patients have been evaluated by multiple physicians (including specialists) before the diagnosis is confirmed.
  • Adequate instruction/education should be provided to patients and their families or caregivers to ensure their safety when acute attacks occur.
 


More on Periodic Paralyses

Overview: Periodic Paralyses
Differential Diagnoses & Workup: Periodic Paralyses
Treatment & Medication: Periodic Paralyses
Follow-up: Periodic Paralyses
References

References

  1. Miller TM, Dias da Silva MR, Miller HA, et al. Correlating phenotype and genotype in the periodic paralyses. Neurology. Nov 9 2004;63(9):1647-55. [Medline].

  2. Venance SL, Cannon SC, Fialho D, et al. The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain. Jan 2006;129(Pt 1):8-17. [Medline].

  3. Matthews E, Labrum R, Sweeney MG, Sud R, Haworth A, Chinnery PF, et al. Voltage sensor charge loss accounts for most cases of hypokalemic periodic paralysis. Neurology. May 5 2009;72(18):1544-7. [Medline].

  4. Arzel-Hezode M, McGoey S, Sternberg D, Vicart S, Eymard B, Fontaine B. Glucocorticoids may trigger attacks in several types of periodic paralysis. Neuromuscul Disord. Mar 2009;19(3):217-9. [Medline].

  5. Donaldson MR, Yoon G, Fu YH, Ptacek LJ. Andersen-Tawil syndrome: a model of clinical variability, pleiotropy, and genetic heterogeneity. Ann Med. 2004;36 Suppl 1:92-7. [Medline].

  6. Dias Da Silva MR, Cerutti JM, Arnaldi LA, Maciel RM. A mutation in the KCNE3 potassium channel gene is associated with susceptibility to thyrotoxic hypokalemic periodic paralysis. J Clin Endocrinol Metab. Nov 2002;87(11):4881-4. [Medline].

  7. Assadi F. Diagnosis of hypokalemia: a problem-solving approach to clinical cases. Iran J Kidney Dis. Jul 2008;2(3):115-22. [Medline].

  8. Streib EW. AAEE minimonograph #27: Differential diagnosis of myotonic syndromes. Muscle Nerve. Sep 1987;10(7):603-15. [Medline].

  9. Fournier E, Arzel M, Sternberg D, et al. Electromyography guides toward subgroups of mutations in muscle channelopathies. Ann Neurol. Nov 2004;56(5):650-61. [Medline].

  10. Levitt JO. Practical aspects in the management of hypokalemic periodic paralysis. J Transl Med. Apr 21 2008;6:18. [Medline].

  11. Elbaz A, Vale-Santos J, Jurkat-Rott K. Hypokalemic periodic paralysis and the dihydropyridine receptor (CACNL1A3): genotype/phenotype correlations for two predominant mutations and evidence for the absence of a founder effect in 16 caucasian families. Am J Hum Genet. Feb 1995;56(2):374-80. [Medline].

  12. Engel AG, Lambert EH, Rosevear JW, Tauxe WN. Clinical and electromyographic studies in a patient with primary hypokalemic periodic paralysis. Am J Med. Apr 1965;38:626-40. [Medline].

  13. Griggs RC. Evaluation and Treatment of Myopathies. 1995. Philadelphia: FA Davis; 318-354.

  14. Hoffman EP, Lehmann-Horn F, Rudel R. Overexcited or inactive: ion channels in muscle disease. Cell. Mar 10 1995;80(5):681-6. [Medline].

  15. Junker J, Haverkamp W, Schulze-Bahr E, et al. Amiodarone and acetazolamide for the treatment of genetically confirmed severe Andersen syndrome. Neurology. Aug 13 2002;59(3):466. [Medline].

  16. Koch MC, Steinmeyer K, Lorenz C. The skeletal muscle chloride channel in dominant and recessive human myotonia. Science. Aug 7 1992;257(5071):797-800. [Medline].

  17. Lin SH, Lin YF, Chen DT, et al. Laboratory tests to determine the cause of hypokalemia and paralysis. Arch Intern Med. Jul 26 2004;164(14):1561-6. [Medline].

  18. McManis PG, Lambert EH, Daube JR. The exercise test in periodic paralysis. Muscle Nerve. Oct 1986;9(8):704-10. [Medline].

  19. Meola G, Sansone V. Treatment in myotonia and periodic paralysis. Rev Neurol (Paris). May 2004;160(5 Pt 2):S55-69. [Medline].

  20. Ptacek L. The familial periodic paralyses and nondystrophic myotonias. Am J Med. Jul 1998;105(1):58-70. [Medline].

  21. Ptacek LJ, Johnson KJ, Griggs RC. Genetics and physiology of the myotonic muscle disorders. N Engl J Med. Feb 18 1993;328(7):482-9. [Medline].

  22. Ruff RL. Slow inactivation: slow but not dull. Neurology. Mar 4 2008;70(10):746-7. [Medline].

  23. Tricarico D, Barbieri M, Mele A, et al. Carbonic anhydrase inhibitors are specific openers of skeletal muscle BK channelof K+-deficient rats. FASEB J. Apr 2004;18(6):760-1. [Medline].

  24. Zhang J, George AL, Griggs RC. Mutations in the human skeletal muscle chloride channel gene (CLCN1) associated with dominant and recessive myotonia congenita. Neurology. Oct 1996;47(4):993-8. [Medline].

Further Reading

Keywords

periodic paralysis, hypokalemia, hyperkalemia, myotonia, paramyotonia congenita, potassium-aggravated myotonia, voltage-sensitive ion channels, voltage-gated ion channels, channelopathy, calcium channels, sodium channels, chloride channels, thyrotoxicosis, periodic paralyses, PP

Contributor Information and Disclosures

Author

Naganand Sripathi, MD, Director, Neuromuscular Clinic, Department of Neurology, Henry Ford Hospital
Naganand Sripathi, MD is a member of the following medical societies: American Academy of Neurology, American Medical Association, Michigan State Medical Society, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Medical Editor

Paul E Barkhaus, MD, Professor, Department of Neurology, Medical College of Wisconsin; Director of Neuromuscular Diseases, Milwaukee Veterans Administration Medical Center
Paul E Barkhaus, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.