Primary periodic paralyses

@article{Finsterer2008PrimaryPP,
  title={Primary periodic paralyses},
  author={Josef Finsterer},
  journal={Acta Neurologica Scandinavica},
  year={2008},
  volume={117}
}
  • J. Finsterer
  • Published 2008
  • Medicine
  • Acta Neurologica Scandinavica
Objective –  To review the current knowledge about primary periodic paralyses (PPs). 
Understanding the physiology of the asymptomatic diaphragm of the M1592V hyperkalemic periodic paralysis mouse
When muscles become paralyzed in crises of hyperkalemic periodic paralysis, patients do not stop breathing. Here is why.
Primary Periodic Paralyses: A Review of Etiologies and Their Pathogeneses
TLDR
Periodic paralyses are a group of disorders characterized by episodes of muscle paralyses that occur as a result of mutations in genes encoding subunits of muscle membrane channel proteins such as sodium, calcium, and potassium channels. Expand
A Case of Addison's Disease Accompanied by Painless Thyroiditis and Hyperkalemic Periodic Paralysis
고칼륨혈증성 주기성 마비는 매우 드물지만 애디슨병과 연관되어 나타날 수 있는 치명적 합병증이다. 애디슨병은 임상증상이 점진적으로 나타나고 비특이적이어서 진단이 늦어지는 경우가 많아 고칼륨혈증, 저나트륨혈증과 같은 전해질이상, 피부나 점막 등의 색소침착, 과거 결핵력 등을 면밀히 살피는 것이 중요하며 급성 부신기능저하가 발생한 경우에는 반드시 유발인자Expand
The primary periodic paralyses: diagnosis, pathogenesis and treatment.
Periodic paralyses (PPs) are rare inherited channelopathies that manifest as abnormal, often potassium (K)-sensitive, muscle membrane excitability leading to episodic flaccid paralysis. HypokalaemicExpand
Muscle and Myotonic Diseases
Although the history and clinical examination remain the most effective way of diagnosing the presence of myopathy, increasingly the clinician has to rely on an understanding of muscleExpand
Paralisia periódica hipocalêmica esporádica Sporadic hypokalemic periodic paralysis
TLDR
A case of primary hypokalemic periodic paralysis in a 25-year-old patient is reported, and the clinical and laboratory parameters were typical. Expand
Treatment of hypokalemic periodic paralysis with topiramate
TLDR
11‐year‐old twins with hypoPP who were treated with topiramate, an anti‐epileptic drug known to have carbonic anhydrase inhibitory properties, experienced a decrease in the severity of their attacks upon initiation of treatment. Expand
Case 1: Transient quadriplegia in a teenager.
A 16-year-old female, previously in good health, awakened to marked weakness in her lower extremities, which progressed to involve her arms and neck over the course of the day, except for her abilityExpand
Glucocorticoids may trigger attacks in several types of periodic paralysis
TLDR
It is hypothesized that glucocorticoids cause hypokalemia due to their stimulation of the Na(+)-K(+) ATPase mediated by insulin and amylin anddue to their side effect of insulin resistance resulting in hyperglycemia. Expand
Paralisia Periódica Hipocalêmica Tipo 1: Envolvimento dos Canais de Cálcio Dependentes de Voltagem
TLDR
Despite considerable progress, is still poorly understood pathophysiology of mutant genes in muscle channelopathies, such as periodic paralysis, making it necessary to direct efforts not only to detect new genes and new mutations that cause, but also for its pathophysiologic consequences. Expand
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References

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The exercise test in periodic paralysis
Of 21 patients with clinically definite hypokalemic, hyperkalemic, or normokalemic periodic paralysis, 15 (71%) had a greater than normal increase in compound muscle action potential amplitude duringExpand
Randomized trials of dichlorphenamide in the periodic paralyses
TLDR
DCP is effective in the prevention of episodic weakness in both HypoPP and PSPP, and significantly reduced attack rates relative to placebo in the Hypo PP subjects. Expand
Hyperkalaemic periodic paralysis and anaesthesia
TLDR
It is concluded from the experiences that with depletion of potassium before surgery, prevention of carbohydrate depletion, avoidance of potassium‐releasing anaesthetic drugs and maintenance of normothermia, patients with hyperkalaemic periodic paralysis can be anaesthetised without complications. Expand
[Hyperkalemic periodic paralysis].
TLDR
Muscle strength usually returns to normal between attacks, although many affected people continue to experience mild stiffness (myotonia), particularly in muscles of the face and hands. Expand
Progressive myopathy in hyperkalemic periodic paralysis.
TLDR
The authors' experience suggests that a progressive myopathy is as common in hyperkalemic periodic paralysis as it is in the hypokalemic disorder. Expand
Thyrotoxic periodic paralysis and anesthesia report of a case and literature review.
TLDR
A case of an Asian male with known TPP undergoing general anesthesia, a brief case series involving 5 patients, and a review of the literature are presented. Expand
Hypokalemic periodic paralysis associated with malignant hyperthermia
TLDR
The association of the two disorders in a patient for whom the most frequent mutations for hypokalemic periodic paralysis were not found suggests further genetic heterogeneity of this condition, the interest of this case residing in the known coupling between dihydropyridine and ryanodine receptors. Expand
Electrocardiographic Manifestations in Patients with Thyrotoxic Periodic Paralysis
TLDR
Relatively rapid heart rate, high QRS voltage, and first‐degree AV block are important clues suggesting TPP in patients who present with hypokalemia and paralysis. Expand
SCN4A-associated hypokalemic periodic paralysis merits a trial of acetazolamide
TLDR
Primary hypokalemic periodic paralysis (HypoPP) is characterized by episodes of transient flaccid paralysis in association with reduced serum potassium levels, and a proportion of affected individuals have slowly progressive permanent weakness. Expand
Subsequent morphological changes in periodic paralysis. A study of seven cases.
TLDR
Muscle biopsies were studied in patients with various types of periodic paralysis to suggest that histology may be normal early in the disease and degenerative changes are correlated with the presence of permanent myopathic weakness. Expand
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