Anesth Pain Med.  2024 Jan;19(1):54-61. 10.17085/apm.23111.

Neuromuscular monitoring of a patient with Charcot-Marie-Tooth disease; which monitoring technique is adequate? - A case report and literature review -

• Kim SU
• Kim S
• Jung KT

• KMID: 2552857
• DOI: http://doi.org/10.17085/apm.23111

Abstract

Background
Charcot-Marie-Tooth disease (CMTD) is a hereditary polyneuropathy associated with a life-threatening risk of pulmonary complications. Case: A 61-year-old male with CMTD for 40 years was admitted for the drainage of an abscess in his left ankle. Total intravenous anesthesia was administered, and an electromyography device was attached to the hand for neuromuscular monitoring; however, the response was not measured. Kinemyography and acceleromyography devices were attached to both hands, and responses were obtained. After neuromuscular blockade (NMB) with rocuronium 0.6 mg/kg, the train-of-four (TOF) response on kinemyography was normally measured, but the post-tetanic count on acceleromyography consistently showed 0 during anesthesia. Sugammadex 200 mg was injected to reverse the NMB. After 5 min, the TOF ratios for kinemyography and acceleromyography exceeded 90%. The patient recovered without any complications.
Conclusions
For CMTD patients, acceleromyography or kinemyography is superior to electromyography, and sugammadex can be used to reverse NMB successfully.

Keyword

Anesthesia; Charcot-Marie-Tooth disease; Neuromuscular blockade; Neuromuscular monitoring; Myography; Sugammadex

Figure

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  Fig. 1. The patient exhibited weakness and atrophy of the peroneal muscle (A) with a pes cavus foot deformity (B), which are a characteristic feature of Charcot-Marie-Tooth disease.

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  Fig. 2. Schematic overview of neuromuscular monitoring and blockade of the patient. EMG: electromyography, AMG: acceleromyography, KMG: kinemyography, TOF: train-of-four, PTC: post-tetanic count.

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