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Normokalemic periodic paralysis
Other Names for this Disease
- Normokalemic PP
- Periodic paralysis type 3
- Potassium-sensitive normokalemic periodic paralysis
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Normokalemic periodic paralysis (NormoKPP) is an inherited muscle disorder characterized by episodic attacks of muscle weakness. It was initially considered a distinct condition, but most physicians now consider it to be a variant form of hyperkalemic periodic paralysis. People with hyperkalemic periodic paralysis have increased potassium levels during an attack; people with the normokalemic form do not experience any change in their potassium levels during weakness, but most grow weak when given potassium. Studies have shown that both forms of the condition are caused by mutations in the SCN4A gene and it is inherited in an autosomal dominant manner. The goals of treatment are to relieve acute symptoms and prevent further attacks.
- Young-Wha Song et al. Normokalemic periodic paralysis is not a distinct disease. Muscle and Nerve. August 2012.
- S. Vicart et al. New mutations of SCN4A cause a potassium-sensitive normokalemic periodic paralysis. Neurology. December 2004.
- What is periodic paralysis?. Periodic Paralysis International. http://hkpp.org/. Accessed September 11, 2012.
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- The Merck Manuals Online Medical Library provides information on this condition. Click on the link to view the information.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. Click on the link to read information on this topic.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Normokalemic periodic paralysis. Click on the link to view a sample search on this topic.
- The The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Normokalemic periodic paralysis. Click on the link to go to OMIM and review these resources.