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Periodic Paralysis—Hyperkalemic/Hypokalemic
Published in Charles Theisler, Adjuvant Medical Care, 2023
Hypokalemic periodic paralysis is the most common form of periodic paralysis. It affects males more often than females. Attacks can be periodic or occur daily and can last from hours to days. The low level of potassium in the blood during episodes of weakness results from potassium moving from the blood into muscle cells in an abnormal way.
Diseases of Muscle and the Neuromuscular Junction
Published in John W. Scadding, Nicholas A. Losseff, Clinical Neurology, 2011
Chris Turner, Anthony Schapira
The frequency and severity of attacks usually diminish with advancing years. Respiratory and bulbar muscles are only occasionally involved. Myotonia usually does not occur except occasionally to affect the eyelids and muscle hypertrophy is very rare. Some patients over 40 begin to develop fixed weakness which progresses slowly over years. Cardiac involvement is very uncommon. Secondary forms of hypokalaemic periodic paralysis occur and are usually the result of chronic potassium depletion, such as severe diarrhoea, or thyrotoxicosis. The latter is most frequently seen in Japanese or Chinese people and is rare outside East Asia. The paralytic episodes may occasionally occur before the diagnosis of thyrotoxicosis.
Improving genetic diagnostics of skeletal muscle channelopathies
Published in Expert Review of Molecular Diagnostics, 2020
Vinojini Vivekanandam, Roope Männikkö, Emma Matthews, Michael G. Hanna
Hypokalaemic periodic paralysis (HypoPP) patients present with attacks of paralysis associated with low serum potassium. Attacks often last longer, hours to days, and are triggered by carbohydrate rich food and exercise [13]. The majority (up to 88%) are caused by mutations in the CACNA1S gene encoding the muscle voltage-sensing calcium channel, CaV1.1, with the rest caused by SCN4A mutations [11]. Mutations in both genes cluster in the regions encoding the channel S4 voltage-sensing domains and cause a ‘gating pore leak current’ – an aberrant leak current – leading to paralysis [14].
Thyrotoxic periodic paralysis: a presentation of hyperthyroidism increasing in frequency around the world
Published in Journal of Endocrinology, Metabolism and Diabetes of South Africa, 2023
To date in Western countries, autosomal dominant hypokalaemic periodic paralysis is the more common presentation of the disease, affecting approximately 1 in 100 000 patients.2 Mutations in voltage-gated sodium, potassium and calcium channels in the skeletal muscle membrane result in the clinical presentation of periodic paralysis with initial clinical presentations typically occurring in the first and second decades of life.3