eMedicine Specialties > Neurology > Neuromuscular Diseases
Periodic Paralyses: Follow-up
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 |
| « Previous Page |
References
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].
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].
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].
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].
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].
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].
Assadi F. Diagnosis of hypokalemia: a problem-solving approach to clinical cases. Iran J Kidney Dis. Jul 2008;2(3):115-22. [Medline].
Streib EW. AAEE minimonograph #27: Differential diagnosis of myotonic syndromes. Muscle Nerve. Sep 1987;10(7):603-15. [Medline].
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].
Levitt JO. Practical aspects in the management of hypokalemic periodic paralysis. J Transl Med. Apr 21 2008;6:18. [Medline].
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].
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].
Griggs RC. Evaluation and Treatment of Myopathies. 1995. Philadelphia: FA Davis; 318-354.
Hoffman EP, Lehmann-Horn F, Rudel R. Overexcited or inactive: ion channels in muscle disease. Cell. Mar 10 1995;80(5):681-6. [Medline].
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].
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].
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].
McManis PG, Lambert EH, Daube JR. The exercise test in periodic paralysis. Muscle Nerve. Oct 1986;9(8):704-10. [Medline].
Meola G, Sansone V. Treatment in myotonia and periodic paralysis. Rev Neurol (Paris). May 2004;160(5 Pt 2):S55-69. [Medline].
Ptacek L. The familial periodic paralyses and nondystrophic myotonias. Am J Med. Jul 1998;105(1):58-70. [Medline].
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].
Ruff RL. Slow inactivation: slow but not dull. Neurology. Mar 4 2008;70(10):746-7. [Medline].
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].
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
Follow-up: Periodic Paralyses