Anaesthetic management using remimazolam in a patient with Charcot-Marie-Tooth disease complicated by sarcoidosis

Charcot-Marie-Tooth (CMT) disease is a progressive hereditary neuropathy that complicates anaesthesia because of neuromuscular-blocker sensitivity. Remimazolam, an ultra-short-acting benzodiazepine, has not yet been reported in CMT. We anaesthetised a woman in her 70s with CMT type 1A and cardiac sarcoidosis for femoral nail fixation under total intravenous anaesthesia with remimazolam and remifentanil. Rocuronium 0.6 mg/kg was given 3 min after induction. Mean arterial pressure ranged from -40% to +5% of baseline and was corrected with 6 mg ephedrine. Anaesthetic depth (bispectral index) and neuromuscular block (train-of-four) were continuously monitored. The infusion was stopped at closure; she was extubated 10 min later and received flumazenil 0.5 mg to ensure full consciousness. No postoperative complications occurred. Remimazolam achieved acceptable haemodynamic control and rapid emergence in this first reported CMT case, suggesting it may be a valuable option for patients with neuromuscular disease and cardiac impairment.