PPNI Library

PPNI Library

PPNI Publishing Library contains a variety of reference materials for PPNI Forum Members and Researchers.


U.S. Department of Health & Human Services

National Institutes of Health

National Library of Medicine

National Center for Biotechnology Information



• Episodes of periodic paralysis typically involve generalized muscle weakness with hypotonia and areflexia but spare bulbar and respiratory muscles and cognition.
• Genetic testing is now the mainstay of diagnosing these syndromes in the majority of patients.
• Measurement of serum potassium levels during the attacks, recognition of precipitating factors, clinical or electrical myotonia, findings on electrodiagnostic tests, cardiac arrhythmia, and distinctive craniofacial and skeletal features help in distinguishing variant forms of inherited periodic paralysis.
• Serum potassium levels can remain within the normal range during the attacks of both hypokalemic periodic paralysis and hyperkalemic periodic paralysis.
• Even though acetazolamide is most commonly used as chronic therapy for hypokalemic periodic paralysis, hyperkalemic periodic paralysis, and related syndromes, there is no clear evidence that acetazolamide is effective from a randomized, double blind, placebo-controlled trial.
• In randomized, double-blind, placebo-controlled trials, dichlorphenamide appears to be promising in ameliorating symptoms in patients with hypokalemic and hyperkalemic periodic paralysis.
Clinical And Experimental Pediatrics