Safety of nitrous oxide administration in patients with Charcot-Marie-Tooth disease

Nitrous oxide is routinely administered to children and adults with Charcot-Marie-Tooth disease (CMT) as an anaesthetic for procedures such as nerve conduction studies and maintenance for general anaesthesia. However it is listed as a 'moderate to significant' risk of potential toxicity and worsening neuropathy in people with CMT by the CMT Association (USA), CMT Association of Australia, CMT International (Canada) and CMT United Kingdom. We performed a systematic review focussing on the use of nitrous oxide in patients with CMT to help clarify its safety. This identified I I studies reporting 41 exposures to therapeutically inhaled nitrous oxide as maintenance for general anaesthesia with no reports of adverse effects or worsening of CMT neuropathy. In the absence of a single case in the literature reporting worsening neuropathy in CMT patients receiving nitrous oxide, this review provides good evidence that nitrous oxide should be considered a safe agent for use in children and adults with CMT. (C) 2007 Elsevier B.V. All rights reserved.