SummaryAcute systemic weakness is a common complaint in the emergency department and has a wide diVerential diagnosis that includes neurologic, metabolic, and infectious aetiologies (box 1). Acute hypokalaemic paralysis, a clinical syndrome characterised by acute systemic weakness and low serum potassium, is a rare but treatable cause of acute weakness. Therefore, it is imperative for physicians, particularly those working in acute care settings, to be aware of this condition. Hypokalaemia is caused by a variety of disorders (box 2),while acute weakness has been reported with some but not all the causes of hypokalaemia (box 3).The most prominent clinical features of hypokalaemia or potassium depletion are neuromuscular, although other systems, such as cardiovascular and gastrointestinal, may also be aVected. Some patients complain of muscular weakness, especially of the lower extremities, while marked and generalised weakness of skeletal muscles is common with more severe potassium depletion. Very severe hypokalaemia may lead to virtually total paralysis including respiratory, bulbar and cranial musculature. Deaths from respiratory failure and arrhythmia have been reported. On physical examination, in addition to decreased motor power, the patient may demonstrate decreased or absent tendon reflexes. The sensations and level of consciousness are generally unaVected.The cardinal laboratory manifestation is a serum potassium of less than 3.5 mmol/l during an attack, although it is usually much lower. Abnormalities in electrocardiogram (ECG) are common. The typical changes include flattening and inversion of T waves, appearance of U waves and ST segment sagging. ECG changes are, however, not well correlated with the severity of the disturbances in potassium metabolism. Symptomatology results from the increased ratio between intra-and extracellular potassium concentrations, which modifies membrane polarization and thereby alters the function of excitable tissues such as nerve and muscle. Diagnosis of hypokalaemic paralysis should be considered in any patient presenting with a sudden onset, areflexic, pure motor weakness involving one or more limbs, without alteration in the level of consciousness or sphincter function, and laboratory evidence of hypokalaemia.Hypokalaemic paralysis results from either alteration in transcellular distribution of potassium or actual potassium depletion from renal or extrarenal losses. Most cases are due to a transcellular shift of potassium and the diVerential diagnosis includes familial periodic paralysis, thyrotoxic periodic paralysis, and barium poisoning.
Familial periodic paralysis (FPP)All primary periodic paralyses have some features in common; they are all treatable and muscular weakness is reversible. Diagnosis is based upon patient history and confirmed by evaluation of serum potassium during attacks and by evaluating the response of muscle power to provocative testing with glucose, insulin, potassium, and cold.Familial hypokalaemic periodic paralysis occurs as an autosomal do...