Refractory familial hypokalaemic periodic paralysis leading to cardiovascular compromise.

Dominic O Awuah, Murtaza S Hussain, Anoosha Ponnapalli, Smit Sunil Deliwala, Elfateh M Seedahmed, Ghassan Bachuwa

BMJ case reports 2022 Feb 07

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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. © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.

Citation

Dominic O Awuah, Murtaza S Hussain, Anoosha Ponnapalli, Smit Sunil Deliwala, Elfateh M Seedahmed, Ghassan Bachuwa. Refractory familial hypokalaemic periodic paralysis leading to cardiovascular compromise. BMJ case reports. 2022 Feb 07;15(2)