Prolonged vecuronium neuromuscular blockade associated with Charcot Marie Tooth neuropathy

Abstract

Charcot Marie Tooth (CMT) disease comprises a group of disorders characterized by progressive distal muscle weakness and wasting. Review of the anaesthetic literature produced conflicting reports concerning the responses to neuromuscular blocking drugs in these patients. We describe a case in which vecuronium 0.11 mg kg-1 produced prolonged neuromuscular blockade lasting 115 min in a patient with the condition. Conduction velocity in the facial nerve is usually less affected than the ulnar or peroneal nerve in CMT patients. This nerve may be more useful in monitoring neuromuscular blockade, both in titrating the dose of neuromuscular blocking drug and ensuring adequate reversal at the end of a procedure. Recent advances in molecular biology have enabled identification of the underlying genetic abnormalities and pathophysiology of CMT. These advances are reviewed and implications of CMT for the anaesthetist discussed.