Hypokalemic Periodic Paralysis, Paralysis, Hyperkalemic Periodic, Paramyotonia Congenita
Conditions
Keywords
neurologic and psychiatric disorders, periodic paralysis, rare disease
Brief summary
OBJECTIVES: I. Assess the efficacy of dichlorphenamide in the treatment of episodic weakness attacks in patients with hyperkalemic periodic paralysis, paramyotonia congenita with periodic paralysis, and hypokalemic periodic paralysis.
Detailed description
PROTOCOL OUTLINE: This is a randomized, double-blind study. Patients are stratified by participating institution and diagnosis. The weekly attack rate is determined during an 8-week assessment prior to therapy initiation and at crossover. Patients are randomly assigned to oral dichlorphenamide (DCP) or placebo for 9 weeks and then cross to the alternate treatment. Patients on DCP at baseline continue on the same dose; those on acetazolamide (ACZ) at baseline receive a DCP dose equivalent to one fifth of the ACZ dose.
Interventions
Sponsors
Study design
Eligibility
Inclusion criteria
PROTOCOL ENTRY CRITERIA: --Disease Characteristics-- Hypokalemic periodic paralysis Typical clinical profile Normal serum thyroxine Hypokalemia during spontaneous or glucose-induced paralytic attack in subject or affected family member Periodic paralysis associated with sodium channel 17q alpha-subunit, e.g.: * Hyperkalemic periodic paralysis with or without myotonia * Paramyotonia congenita with periodic paralysis Distinct, regular episodes of weakness at least once a week and no more than 3 times a day No history of worsening symptoms with carbonic anhydrase inhibitor No history of life-threatening weakness episodes prior to treatment No atypical periodic paralysis without demonstrable 17q alpha-subunit defect --Prior/Concurrent Therapy-- No requirement for the following agents, unless for periodic paralysis: * Diuretics * Antiepileptics * Antiarrhythmics * Magnesium supplements * Steroids * Calcium supplements * Beta-blockers * Potassium supplements * Calcium channel blockers --Patient Characteristics-- Hepatic: No hepatic disease Renal: * No renal failure * No nephrolithiasis Cardiovascular: * No heart disease * No cardiac arrhythmia Pulmonary: No restrictive or obstructive lung disease Other: * No active thyroid disease * No pregnant women