Periodic Paralysis in Taiwan

CHEN, KWANG-MING

doi:10.1001/archneur.1965.00460260055007

Cited by 15 publications

(1 citation statement)

References 28 publications

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“…When patients become euthryoid, the attacks of weakness stop, and the CMAP exercise test normalizes (108). The prevalence of TPP is much higher in Asia (China, Korea, Japan) than in North America or Europe (38, 173). About 2% of this high-prevalence group will have episodes of weakness while hyperthyroid, compared to 0.1–0.2% in other populations.…”

Section: Potassium Channelopathies Of Skeletal Musclementioning

confidence: 99%

Channelopathies of Skeletal Muscle Excitability

Cannon

2015

Comprehensive Physiology

175

176

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Familial disorders of skeletal muscle excitability were initially described early in the last century and are now known to be caused by mutations of voltage-gated ion channels. The clinical manifestations are often striking, with an inability to relax after voluntary contraction (myotonia) or transient attacks of severe weakness (periodic paralysis). An essential feature of these disorders is fluctuation of symptoms that are strongly impacted by environmental triggers such as exercise, temperature, or serum K+ levels. These phenomena have intrigued physiologists for decades, and in the past 25 years the molecular lesions underlying these disorders have been identified and mechanistic studies are providing insights for therapeutic strategies of disease modification. These familial disorders of muscle fiber excitability are “channelopathies” caused by mutations of a chloride channel (ClC-1), sodium channel (NaV1.4), calcium channel (CaV1.1) and several potassium channels (Kir2.1, Kir2.6, Kir3.4). This review provides a synthesis of the mechanistic connections between functional defects of mutant ion channels, their impact on muscle excitability, how these changes cause clinical phenotypes, and approaches toward therapeutics.

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Section: Potassium Channelopathies Of Skeletal Musclementioning

confidence: 99%

Channelopathies of Skeletal Muscle Excitability

Cannon

2015

Comprehensive Physiology

175

176

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Gossypol and hypokalaemia

Wang

Yeung

1985

Contraception

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