Refractory familial hypokalaemic periodic paralysis leading to cardiovascular compromise

Abstract

Familial hypokalaemic periodic paralysis (FHPP) is a rare neuromuscular disorder that is classified under periodic paralysis (PP), which is characterised by episodes of muscle weakness. Common triggers include intense exercise, fasting or consumption of carbohydrate-rich meals. Hypokalaemic PP has an incidence of 1 in 100 000; despite the temporal association, cardiac manifestations are exceedingly rare. We present a case of FHPP, a channelopathy presenting with severe refractory hypokalaemia. The challenges with our patient were maintaining potassium levels within normal ranges and initiating a close follow-up plan. Due to the lack of clinical guidance in our case, many aspects of care, including surveillance, medications and genetic testing, remain unaddressed. Medical management includes aggressive correction with supplements, potassium-sparing diuretics and carbonic anhydrase inhibitors. Severe cases of dysrhythmias, especially ventricular fibrillation, require electrophysiology evaluation and possible implantation of a defibrillator to prevent sudden cardiac death.

Acute flaccid paralysis in Indonesian adult due to suspected familial hypokalemia paralysis: A rare case

“…Most cases of HPP are caused by heredity or familiarity, better known as familial hypokalemic periodic paralysis (FHPP). Gene mutations cause FHPP in calcium or sodium ion channels [ 5 ]. FHPP is often only identified when a patient's acute flaccid paralysis (AFP) occurs.…”