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• W2014264145 abstract "Editor, Patients with myotonic dystrophy show myotonic responses to succinylcholine1 and neostigmine,2 and increased sensitivity to non-depolarising muscle relaxants.3 Sugammadex. a novel drug developed specifically for the rapid reversal of neuromuscular blockade induced by steroidal non-depolarising muscle relaxants, would be useful in patients with muscular disorders. Although the use of sugammadex was reported in a patient with myasthenia gravis,4 it has not been addressed in other disorders. A 24-year-old woman, 160 cm in height and weighing 75 kg, was scheduled for laparoscopic ovarian cystectomy. Her father and brother were previously diagnosed with myotonic dystrophy. At the age of 22, she was diagnosed by a neurologist as having myotonic dystrophy. Physical examination showed the typical facies and frontal baldness of myotonic dystrophy. Her sternocleidomastoid muscles were atrophic, and muscle strength in her hands, forearms and lower limbs was weak. Serum creatine phosphokinase was as high as 624 IU l−1 (normal range for women, 47–176 IU l−1). ECG and echocardiography were normal. She had myotonia in the hands and pain in the proximal upper limb muscles. She had a history of rhabdomyolysis after acetaminophen. Her physical status was classified as ASA 2. Anaesthesia was induced with intravenous propofol (2 mg kg−1) and remifentanil (0.5 μg kg−1 min−1) and maintained with intravenous infusions of propofol (4 mg kg−1 h−1) and remifentanil (0.2 μg kg−1 min−1). Ultrasound-guided bilateral transversus abdominis plane block was performed with 20 ml of 0.375% ropivacaine on each side. After induction of anaesthesia, but before administration of rocuronium for muscle relaxation, monitoring of neuromuscular activity was started using acceleromyography (TOF-Watch SX, Schering-Plough Ireland, Dublin, Ireland) at the adductor pollicis muscle. Repetitive train-of-four (TOF) stimulation was applied at the ulnar nerve at the wrist every 15 s until the end of anaesthesia, or at least until recovery of the TOF ratio to 0.9. To obtain complete muscular blockade, 0.3 mg kg−1 of rocuronium was required which was less than the normal dose of rocuronium for induction (0.6 mg kg−1). Tracheal intubation was performed and maintenance doses of rocuronium (0.1 mg kg−1) were administered as needed at the reappearance of the fourth twitch (T4) of the TOF. After surgery, with reappearance of T2 after the last dose of rocuronium, sugammadex (2 mg kg−1) was administered. The time to recovery of the TOF ratio to 0.9 from the start of sugammadex administration was within 2 min. Propofol and remifentanil were maintained until recovery of the TOF ratio to 0.9, and stopped before tracheal extubation. Complete recovery of neuromuscular function was assessed by the performance of a successful 5-s head lift, following which tracheal extubation was performed. No exacerbation of myotonia and no recurrence of muscle relaxation were observed peri-operatively. Myotonic dystrophy is an autosomal dominant, multi-system disorder, characterised by skeletal muscle weakness, myotonia, cardiac conduction abnormalities and precocious cataracts. Myotonic patients may have abnormal responses to specific pharmacological agents used in anaesthesia.5 Patients with myotonic dystrophy have increased sensitivity to non-depolarising muscle relaxants.3 Further, anti-cholinesterase drugs can precipitate myotonia or may result in inadequate antagonism of neuromuscular blockade.3 Hence, small doses of non-depolarising muscle relaxants should be administered to patients with myotonic dystrophy. The combination of rocuronium and sugammadex, however, can replace these previous anaesthetic management regimes in patients with muscle disorders.4 In the present study, our patient with myotonic dystrophy required 0.3 mg kg−1 of rocuronium for induction, and showed normal recovery of the TOF ratio to 0.9 after the administration of sugammadex (2 mg kg−1). Therefore, she had increased sensitivity to rocuronium, and showed normal responses to sugammadex.6,7 We suggest that sugammadex might be useful in patients with myotonic dystrophy." @default.
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• W2014264145 date "2011-02-01" @default.
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• W2014264145 title "The use of sugammadex in a patient with myotonic dystrophy" @default.
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• W2014264145 doi "https://doi.org/10.1097/eja.0b013e3283405b87" @default.
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