Procainamide therapy, physical performance and energy expenditure in the Schwartz-Jampel syndrome.
The effects of procainamide administration were assessed in a 5-yr-old boy with Schwartz-Jampel syndrome (chondrodystrophic myotonia). Without procainamide the resting metabolic rate was found to be significantly higher than in an age-matched control group. With a serum level of 3.8 mg l-1 procainamide a reduction of the resting metabolic rate of 22% was observed, and times needed to climb stairs and to re-open eyes after forceful contraction (blepharospasm) were significantly reduced.
Spaans
F
F
Department of Clinical Neurophysiology, University of Limburg, Maastricht, The Netherlands.
Wagenmakers
A
A
Saris
W
W
Reekers
A
A
Theunissen
P
P
Cremers
H
H
D002675:Child, Preschool; D004734:Energy Metabolism / Q000187:drug effects*; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009043:Motor Activity / Q000187:drug effects; D009132:Muscles / Q000187:drug effects / Q000503:physiopathology*; D010009:Osteochondrodysplasias / Q000188:drug therapy / Q000503:physiopathology*; D011342:Procainamide / Q000097:blood / Q000627:therapeutic use*
D011342:Procainamide