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Periodic paralysis may have a familial (dominant inheritance) basis. The syndromes to be described are channelopathies that manifest as abnormal, often potassium-sensitive, muscle-membrane excitability and lead clinically to episodes of flaccid weakness or paralysis, sometimes in association with abnormalities of the plasma potassium level. Strength is initially normal between attacks, but progressive myopathic weakness may develop in up to one-third of patients as they age. Mutations in genes encoding three ion channels (CACNA1S, SCN4A, and KCNJ18) account for most cases. Hypokalemic periodic paralysis is characterized by attacks that tend to occur on awakening, after exercise, or after a heavy meal and may last for several days. Patients should avoid excessive exertion. A low-carbohydrate and low-salt diet may help prevent attacks. An ongoing attack may be aborted by potassium chloride given orally or by intravenous drip, provided the ECG can be monitored and kidney function is satisfactory. In young Asian men, it is commonly associated with hyperthyroidism and has been related to polymorphism in the CACNA1S gene; treatment of the endocrine disorder prevents recurrences. A nonselective beta-adrenergic blocker may prevent attacks until the endocrine abnormality has been treated. In hyperkalemic periodic paralysis, which is mostly associated with mutations in the SCN4A gene, attacks also tend to occur after exercise but usually last for less than 1 hour. They may be terminated by intravenous calcium gluconate (1–2 g) or by intravenous diuretics (furosemide, 20–40 mg), glucose, or glucose and insulin. Normokalemic periodic paralysis is similar clinically to the hyperkalemic variety, but the plasma potassium level remains normal during attacks. Several randomized trials support the use of dichlorphenamide (50–100 mg orally twice daily) for prevention of attacks in both hyperkalemic and hypokalemic periodic paralysis; acetazolamide (250–750 mg orally daily) is also effective. Chlorothiazide may also be used to prevent attacks in hyperkalemic periodic paralysis.

WHEN TO REFER

All patients should be referred.

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Sansone  VA  et al; Muscle Study Group. Randomized, placebo-controlled trials of dichlorphenamide in periodic paralysis. Neurology. 2016 Apr 12;86(15):1408–16.
[PubMed: 26865514]  

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