Review of the Diagnosis and Treatment of Periodic Paralysis

Abstract

Periodic paralyses (PPs) are rare neuromuscular disorders caused by mutations in skeletal muscle sodium, calcium, and potassium channel genes. PPs include hypokalemic paralysis, hyperkalemic paralysis, and Andersen‐Tawil syndrome. Common features of PP include autosomal dominant inheritance, onset typically in the first or second decades, episodic attacks of flaccid weakness, which are often triggered by diet or rest after exercise. Diagnosis is based on the characteristic clinic presentation then confirmed by genetic testing. In the absence of an identified genetic mutation, documented low or high potassium levels during attacks or a decrement on long exercise testing support diagnosis. The treatment approach should include both management of acute attacks and prevention of attacks. Treatments include behavioral interventions directed at avoidance of triggers, modification of potassium levels, diuretics, and carbonic anhydrase inhibitors. Muscle Nerve 57: 522–530, 2018

Periodic Paralysis Syndromes: A T3 Thyrotoxicosis Etiology Presentation and Review of Literature

“…EKG findings may show changes that are associated with hypokalemia such as an increase in the amplitude of U waves, a decrease in the amplitude of the T wave, and depression of the ST segment. 4 The long term consequence of hypokalemic PP is myopathy that may begin after the age of 50. The severity of the myopathy varies with ion channel mutation.…”

Hypokalemic Periodic Paralysis: Reports of Two Cases and Brief Review

“…20 Potassium sparing diuretics e.g., spironolactone 25-100 mg/day, eplerenone 50-100 mg/day or triamterene 50-150 mg daily may be effective. 21 Acetazolamide (125-1000 mg daily in divided dose) 2,10,20,21 and dichlorphenamide (50 mg twice daily) 22,23 are the available carbonic anhydrase inhibitors. Our first patient apparently responded to spironolactone 100 mg/day, as evidenced by reduction of number of recurrences.…”

Periodic paralysis: what clinician needs to know?

“…Although the diseases are inherited, only one third of patients will have a family history. 19 High rate of acquired mutations may at least partly account for this observation. Presence of family history, previous recurrent episodes and abnormal serum potassium during the event would strongly suggest the diagnosis.…”

“…Men are commonly affected, often in 20-40 year age group, in contrast to primary hypoKPP which often manifests before 20 years. 19 Patient will be biochemically thyrotoxic but clinical features may vary from no symptoms to overt thyrotoxicosis. 20 Obese individuals are at increased risk due to hyperinsulinaemia.…”