Management of a 41-year-old man with hypokalemic periodic paralysis required intravenous potassium for a level of 1.9 mmol/L, followed by acetazolamide and dietary changes to prevent recurrence.
Case Report (n=1)
This case highlights the diagnostic and therapeutic challenges of managing hypokalemic periodic paralysis, emphasizing the need for individualized potassium replacement and preventive care with carbonic anhydrase inhibitors.
Abstract Introduction Hypokalemic periodic paralysis (HPP) is a rare autosomal dominant channelopathy characterized by transient episodes of muscle weakness or paralysis due to intracellular potassium shift and resultant hypokalemia. Attacks are often triggered by rest after exercise, high-carbohydrate meals, or stress. Although typically reversible with potassium repletion, recurrent episodes and variable responses to therapy make management challenging. We present a case of genetically confirmed HPP that illustrates diagnostic and therapeutic difficulties in long-term care. Case Report A 41-year-old man with a known family history of HPP presented with progressive, symmetric weakness of both lower and upper extremities over several hours despite taking over-the-counter potassium supplements. On arrival, his potassium was critically low at 1.9 mmol/L. He was admitted to the intensive care unit for cardiac monitoring and treated with intravenous and oral potassium, resulting in gradual recovery of strength and normalization of serum potassium. Despite careful repletion and counseling, he re-presented two months later with recurrent paralysis following strenuous physical activity and a carbohydrate-rich meal. Repeat labs confirmed hypokalemia (2.2 mmol/L) with otherwise normal electrolytes and thyroid function. Genetic testing identified a pathogenic CACNA1S mutation consistent with familial HPP. Long-term management included initiation of acetazolamide, dietary modification emphasizing low-glycemic carbohydrate intake, and structured outpatient monitoring to minimize recurrence. Discussion HPP arises from mutations in voltage-gated calcium or sodium channel genes (CACNA1S or SCN4A), leading to aberrant membrane excitability and intracellular potassium sequestration. The condition typically manifests in adolescence or early adulthood, with frequency and severity of attacks often declining with age. Acute management centers on cautious potassium repletion to restore muscle function while avoiding rebound hyperkalemia. Preventive therapy with carbonic anhydrase inhibitors such as acetazolamide or dichlorphenamide reduces attack frequency by stabilizing membrane excitability.This case underscores the unpredictable nature of potassium fluctuations even with close monitoring and adherence. Variability in absorption, dietary intake, and genetic phenotype can complicate potassium regulation, emphasizing the need for individualized replacement strategies. Broader awareness of HPP among clinicians is critical, as early recognition and tailored preventive care can significantly reduce morbidity and hospitalizations. This abstract is funded by: None
Govil et al. (Fri,) conducted a case report in Hypokalemic periodic paralysis (n=1). Potassium repletion, acetazolamide, and dietary modification was evaluated. Management of a 41-year-old man with hypokalemic periodic paralysis required intravenous potassium for a level of 1.9 mmol/L, followed by acetazolamide and dietary changes to prevent recurrence.