Recurrent Hypokalemia leading to Flaccid Quadriparesis: A Renal or Connective Tissue Disorder

Abstract

Hypokalemic periodic paralysis (hypoKPP) is a clinical entity characterized by recurrent skeletal muscle paralysis due to a decrease in serum potassium levels; hypoKPP can have either a primary (familial) or a secondary cause. One of the secondary causes of hypoKPP is distal renal tubular acidosis (dRTA). Distal renal tubular acidosis (dRTA) is diagnosed when the urinary pH is greater than 5.3 and in the presence of hyperchloremic metabolic acidosis and hypokalemia, with one of the causes being primary Sjögren’s syndrome (pSS). PSS can have both glandular and extra glandular manifestations, with dryness of the eyes and mouth being the most common presenting symptoms. DRTA arising from pSS is very unusual, occurring in fewer than 2% of the cases of Sjogren’s syndrome (SS). Here, we report on a case of recurrent flaccid quadriparesis that appears to have been caused by distal RTA, resulting in hypokalemia; upon further investigation and clinical evaluation, the patient was diagnosed with pSS.