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THERAPEUTIC MANAGEMENT OF PERIODIC PARALYSIS

General Overview of Management

 
The periodic paralyses are commonly managed through dietary manipulation designed to avoid fluctuations in serum potassium. This includes avoidance of excessive sodium and carbohydrate (sweets, sweetened beverages, pasta, starchy foods and similar foods) in HypoKPP, and avoidance of high potassium foods in HyperKPP. It is important to recognize that individuals exhibit significant variability in sensitivity to dietary components and other triggers. A dependable standard for all individuals has not been established. For example, in HypoKPP, some individuals report the inability to consume even very low levels of sodium without inducing weakness, while in a very few individuals salt has been shown to abort weakness. Management approaches include life-style changes to minimize sources of stress and exposure to other identified triggers. For improved control, individuals must work diligently to identify appropriate and effective management practices based on their own unique requirements.

In some hypokalemic cases, individuals require supplemental potassium, or the additional assistance of drugs. The most commonly used drugs, the carbonic anhydrase inhibitors, are reportedly beneficial in most forms of periodic paralysis. Acetazolamide, still the most commonly prescribed, has been reported as ineffective in as many as thirty percent of individuals trying it, but Acetazolamide has been the standard drug prescribed for periodic paralysis for over five decades, with a positive history of successful reduction of symptoms in the majority of patients. Dichlorphenamide is the second most commonly used drug and is frequently interchanged with Acetazolamide when or if Acetazolamide becomes less effective. Methazolamide is infrequently prescribed, but reported anecdotally by some patients to exhibit fewer side effects. The principle side effects of these drugs are the formation of kidney stones, and various sensations including transient tingling and numbness in the extremities. Clinical trials must be accomplished to demonstrate the effectiveness of these and other potentially effective therapies in the growing list of variants in the periodic paralyses.

Also See:

Practical aspects in the management of hypokalemic periodic paralysis: 4/21/2008, Jacob O Levitt.

Randomized trials of dichlorphenamide in the periodic paralyses. Working Group on Periodic Paralysi: January, 2000, Tawil R, McDermott MP, Brown R Jr, Shapiro BC, Ptacek LJ, McManis PG, Dalakas MC, Spector SA, Mendell JR, Hahn AF, Griggs RC.

Channelopathies: Moxley III RT..

Kidney stones, carbonic anhydrase inhibitors, and the ketogenic diet.

Acetazolamide-induced muscle weakness in hypokalemic periodic paralysis.

Sodium channel inactivation defects are associated with acetazolamide-exacerbated hypokalemic periodic paralysis. Bendahhou S, Cummins TR, Griggs RC, Fu YH, Ptacek LJ.

A case of potassium-sensitive periodic paralysis with cardiac dysrhythmia controlled with imipramine and acetazolamide: April, 2001, Endo T, Takeda A, Shiga Y, Fujimori J, Takahashi T, Tateyama M, Onodera H, Itoyama Y, Yoshimoto T.

Acetazolamide opens the muscular KCa2+ channel: a novel mechanism of action that may explain the therapeutic effect of the drug in hypokalemic periodic paralysis: September, 2000, Tricarico D, Barbieri M, Camerino DC.

Treatment of "permanent" muscle weakness in familial Hypokalemic Periodic Paralysis. Dalakas MC, Engel WK.

Treatment for periodic paralysis: from the Cochrane Library Sansone V, Meola G, Links T, Panzeri M, Rose MR

Periodic paralysis patients who exhibit cardiac dysrhythmias should be evaluated by an experienced clinician to insure effective follow-up and management of this potentially fatal status. In the case of Andersenís-Tawil Syndrome, with the involvement of multiple tissues and organ systems, and the established risk associated with long QT and other cardiac irregularities, Tawil and others have advised that the patient be followed by a team that includes a cardiologist and a neurologist to maintain close monitoring of the progress and effective management of the affected individual.

Other precautions are encouraged, especially when the use of anesthesia is being considered and during parturition (delivery).

Severe prognosis in a large family with hypokalemic periodic paralysis. Caciotti A, Morrone A, Domenici R, Donati MA, Zammarchi E.

Intravenous regional anesthesia in patients with hypokalemic periodic paralysis Ezri T, Dotan Z, Evron S.

Hypokalemic periodic paralysis associated with malignant hyperthermia. Rajabally YA, El Lahawi M.

Anesthetic management of familial hypokalemic periodic paralysis during parturition. Viscomi CM, Ptacek LJ, Dudley D.

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Section Last Modified:
12/29/2009 07:38 PT
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