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Book

Hypokalemic Periodic Paralysis

Prabin Phuyal  1 , Beenish S. Bhutta  2 , Shivaraj Nagalli  3
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
.
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Book

Hypokalemic Periodic Paralysis

Prabin Phuyal et al.
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Excerpt

Hypokalemic periodic paralysis (hypoPP) is a rare disorder caused by skeletal muscle ion channel mutations, mainly affecting calcium or sodium channels. HypoPP is characterized by episodic severe muscle weakness, usually triggered by strenuous exercise or a high-carbohydrate diet. Patients with hypoPP experience a sudden onset of generalized or focal flaccid paralysis associated with low blood serum potassium levels (or hypokalemia), which can last for several hours before resolving spontaneously.

The majority of hypoPP cases are hereditary or familial. The familial form of hypoPP is a rare channelopathy resulting from mutations in either the calcium or sodium ion channels, predominantly affecting skeletal muscle cells. Acquired cases of hypoPP are also identified and associated with hyperthyroidism. The disease-causing mutation in hypoPP, specifically in the CACNA1S gene, was identified by Jurkat-Rott et al in 1994.

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Conflict of interest statement

Disclosure: Prabin Phuyal declares no relevant financial relationships with ineligible companies.

Disclosure: Beenish Bhutta declares no relevant financial relationships with ineligible companies.

Disclosure: Shivaraj Nagalli declares no relevant financial relationships with ineligible companies.

References

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    1. Jurkat-Rott K, Weber MA, Fauler M, Guo XH, Holzherr BD, Paczulla A, Nordsborg N, Joechle W, Lehmann-Horn F. K+-dependent paradoxical membrane depolarization and Na+ overload, major and reversible contributors to weakness by ion channel leaks. Proc Natl Acad Sci U S A. 2009 Mar 10;106(10):4036-41. - PMC - PubMed
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