Response to atracurium and mivacurium in a patient with Charcot-Marie-Tooth disease

Abstract

Purpose: We studied the neuromuscular effects of both atracurium and mivacurium in a patient with Charcot-Marie-Tooth disease (CMTD) during nitrous oxide-oxygen-alfentanil-propofol anaesthesia. Neuromuscular blockade was monitored electromyographically. Train-of-four stimulation (2 Hz @ 20 sec intervals) was delivered to the ulnar nerve throughout the period of observation.

Clinical features: A 17-yr-old man with the diagnosis of CMTD was presented twice for two different orthopaedic surgical procedures. The CMTD had been diagnosed since childhood. Neurological examination revealed distal wasting of the upper and lower limbs, generalised absence of reflexes and decreased sensation in a stocking distribution. In both anaesthetics, induction was carried out with alfentanil and propofol, and anaesthesia was maintained with nitrous oxide in oxygen, alfentanil and propofol infusion. The patient demonstrated a normal response to both atracurium and mivacurium. Onset time and the maximum block attained after atracurium and mivacurium were 240 and 210 sec, and 97% and 99% inhibition of T1 (the first twitch of TOF stimulation), respectively. Recovery of T1 to 10% of the control value occurred 30 and 11.5 min after the administration of atracurium and mivacurium, respectively. The patient made uneventful recoveries after both anaesthetics.

Conclusion: There was no evidence of prolonged response to atracurium and mivacurium in our patient with CMTD.