To the Editor:
Hyperkalemic familial periodic paralysis (HFPP) is a rare inherited autosomal dominant disease that causes patients to experience episodes of flaccid weakness associated with increased serum potassium levels (1). Anesthesia can precipitate prolonged muscle weakness in these patients, particularly when succinylcholine is used (2,3).
A 42 year-old female patient reported multiple episodes of muscle weakness beginning at the age of 9. The diagnosis of HFPP was established on clinical and genetic bases and she was chronically given acetazolamide. She underwent percutaneous closure of an atrial septal defect responsible for transient ischemic stroke under general anesthesia. ECG, noninvasive blood pressure, pulse oxymetry, esophageal temperature, end-tidal CO2 and BIS (XP version) were monitored. A target-controlled propofol-remifentanil infusion was started and orotracheal intubation was easily performed (4,5). A transseptal Starflex Teflon device was properly positioned under transesophageal echocardiographic guidance in 40 min. The patient was able to tell her name and move on command 7 min after cessation of anesthetic delivery and discharged uneventfully. No change serum potassium levels were detected.
The use of target-controlled, rather than manually driven (6), anesthesia was particularly useful in this context by providing excellent intubation conditions without muscle relaxants and allowing early evaluation of postanesthetic muscular tone.
Jean-Pol Depoix, MD
Jean-Michel Julliard, MD
Pierre Aubry, MD
Jean-Marie Desmonts, MD
Alec Vahanian, MD
Jean Mantz, MD, PhD
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2. Weller JF, Elliott RA, Pronovost PJ. Spinal anesthesia for a patient with familial hyperkalemic periodic paralysis. Anesthesiology 2002;97:259–69.
3. Ashwood EM, Russel WJ, Burrow DD. Hyperkalemic periodic paralysis and anaesthesia. Anaesthesia 1992;47:579–84.
4. Marsh B, White M, Morton N, Kenny GNC. Pharmacokinetic model driven infusion of propofol in children. Br J Anaesth 1991;67:41–8.
5. Minto CF, Schnider TW, Egan TE, et al. Influence of age and gender on the pharmacokinetics and pharmacodynamics of remifentanil: I. Model development. Anesthesiology 1997;86:10–23.
6. Passot S, Servin F, Allary R, et al. Target-controlled versus manually controlled infusion of propofol for direct laryngoscopy and bronchoscopy. Anesth Analg 2002; 94:1212–6.