Hypokalemic periodic paralysis with unusual responses to acetazolamide and sympathomimetics

An atypical phenotype of hypokalemic periodic paralysis caused by a mutation in the sodium channel geneSCN4A

“…However, in some patients with either a specific calcium channel mutation or a sodium channel mutation, no effect and even worsening of the paralytic symptoms have been reported after acetazolamide treatment3, 11). Spironolactone and its derivatives have been used as alternatives to acetazolamide in those cases in which initial empirical therapy with acetazolamide has been ineffective12, 13). We initially prescribed acetazolamide to the patients described in this report; however, the episodes of muscle weakness did not improve in either frequency or severity following acetazolamide treatment.…”

Acetazolamide-induced Muscle Weakness in Hypokalemic Periodic Paralysis.

“…Previous reports describe that ACZ-induced weakness is associated with potassium loss, DM, a variant form of HypoPPor therapeutic effects of triamterene (3- DM,hyperinsulinemia, proximal renal tubular acidosis and benefits of triamterene. The first commonhallmark points out that ACZproduces mild to severe HypoPPattacks within three days after the initial administration of this drug in all patients (3)(4)(5), including our patient. After HypoPP patients receive the first treatment with ACZ,we should evaluate the degree and the frequency of their attacks cautiously.…”

“…Acetazolamide (ACL), a carbonic anhydrase inhibitor, is commonly used for the preventation of HypoPPattacks ( 1 , 2). However, several reports suggest that this agent exacerbates attacks of muscle weakness in patients with HypoPP (3)(4)(5) or paramyotonia congenita (6). The mechanism of these harmful effects remains unknown.Wereport a unique patient with HypoPP and diabetes mellitus (DM) with ACZ-induced paralytic attacks and proximal renal tubular acidosis.…”