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BJA Correspondence

Anaesthetic management of a patient with consider that any patient with muscle disease is at increased
risk of MH.9 Pasha and Knowles have quoted the study by
Charcot–Marie – Tooth disease for staged
Antognini5 for an improbable relationship between MH and
diaphragmatic plication CMT syndrome. In that study, MH-inducing agents were admi-
Editor—With great interest, we have read the article by Pasha nistered in more than 100 surgical procedures, and no cases of
and Knowles.1 We found it to be a very interesting article as it MH were found. The authors mentioned the small sample size,
offers more information on these patients’ anaesthetic man- so they were unable to exclude a potential risk in these
agement. The authors describe two types of anaesthesia in patients. Furthermore, there is one case which describes MH
the same patient and used vecuronium in the first. Under an- in CMT syndrome.10 Therefore, we must be careful and avoid
aesthesia, they describe a possible residual effect with vecuro- anaesthetic agents that can cause MH as much as possible.
nium. As they mention in their article, there is considerable
dispute about the use of neuromuscular blocking agents in
these patients. No consensus has been reached on the man-
Declaration of interest

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agement of these patients. However, after considering the None declared.
potential prolonged neuromuscular block that may appear in
these patients, we wish to ask about the possibility of using M. del-Rio-Vellosillo*
rocuronium, given its greater selectivity with sugammadex.2 J. J. Garcia-Medina
Moreover, we would like to ask the authors if it was possible R. Martin-Gil-Parra
for them to monitor the neuromuscular response in the ad- Murcia, Spain
ductor pollicis or corrugator supercilii, and if so, what the E-mail:
results were. The literature describes some cases in which it
has been possible to monitor the neuromuscular response 1 Pasha TM, Knowles A. Anaesthetic management of a patient with
Charcot– Marie– Tooth disease for staged diaphragmatic plication.
with the adductor pollicis or corrugator supercilii in patients
Br J Anaesth 2013; 110: 1061–3
with Charcot –Marie –Tooth (CMT) syndrome and severe gener-
2 Naguib M, Brull SJ. Sugammadex: a novel selective relaxant binding
alized polyneuropathy.3 4 agent. Exp Rev Clin Pharmacol 2009; 2: 37–53
The authors do not describe if a neurological examination 3 Baraka AS. Vecuronium neuromuscular block in a patient with
was done before surgery, or any previous surgery and anaes- Charcot– Marie– Tooth syndrome. Anesth Analg 1997; 84: 927– 8
thesia in the patient. 4 Gálvez-Cañellas JL, Errando CL, Martı́nez-Torrente F, et al. Anaes-
CMTsyndrome is a peripheral polyneuropathy that produces thesia and orphan disease: difficult monitoring of neuromuscular
autonomic denervation and muscle atrophy until patients blockade in a patient with severe Charcot– Marie– Tooth disease
become immobilized, as in this case. Pasha and Knowles indi- type I. Eur J Anaesthesiol 2013; 30: 772– 5
cate that the use of succinylcholine in these patients could 5 Antognini JF. Anaesthesia for Charcot– Marie– Tooth disease: a
review of 86 cases. Can J Anaesth 1992; 39: 398– 400
be safe.5 A study by Martyn and Richtsfeld6 explained the phe-
6 Martyn JAJ, Richtsfeld M. Succinylcholine-induced hyperkalemia in
nomenon of proliferation of acetylcholine receptors on the
acquired pathologic states. Etiologic factors and molecular
muscle membrane in neuropathies, which can cause massive
mechanisms. Anesthesiology 2006; 104: 158– 69
release of potassium on exposure to succinylcholine. In add-
7 Hashimoto T, Morita M, Hamaguchi S, Kitajima T. Anesthetic man-
ition, prolonged immobilization may also increase the risk. agement for pancreaticoduodenectomy in a patient with Charcot–
Hence, we should exercise care, perhaps avoid succinylcholine, Marie–Tooth disease and liver cirrhosis. Masui 2009; 58: 1313–5
and consider rocuronium for rapid sequence induction in these 8 Kapur S, Kumar S, Eagland K. Anesthetic management of a parturi-
patients. ent with neurofibromatosis 1 and Charcot–Marie– Tooth disease.
We had a patient with CMT syndrome for an emergency Cae- J Clin Anesth 2007; 19: 405–6
sarean section in whom we undertook rapid sequence induc- 9 Mueller RA. How to identify malignant hyperthermia. Probl Anesth
tion with rocuronium. We reversed it with sugammadex and 1987; 1: 233– 44
saw no residual effect. In our case, there was no possibility of 10 Ducart A, Adnet P, Renaud B, Riou B, Krivosic-Horber R. Malignant
hyperthermia during sevoflurane administration. Anesth Analg
neuromuscular monitoring because it was an emergency Cae-
1995; 80: 609–11
sarean section and there was no monitor in our operating
theatre. After an extensive literature search, we found only doi:10.1093/bja/aet572
two studies where rocuronium was used without any residual
paralysis,4 7 although there was one report of prolonged
effect, possibly secondary to liver dysfunction.8
Finally, Pasha and Knowles1 considered the risk of malig-
Dexmedetomidine in peripheral and
nant hyperthermia (MH) in these patients, which is unfounded neuraxial block: a meta-analysis
because this is a neuropathy, not a myopathy. CMT syndrome is Editor—We read with interest the meta-analysis by Drs Abdal-
a peripheral demyelinating polyneuropathy that can evolve lah and Brull, which concluded that dexmedetomidine
progressively and severely to produce chronic muscular dys- prolongs both sensory and motor block after central and per-
trophy. As described in the literature, the relationship ipheral nerve blocks.1 We would like to highlight some of our
between CMT syndrome and MH is not clear; thus, one could concerns about the study.