title: [Anesthetic Management of a Patient with Facioscapulohumeral Muscular Dystrophy: Importance of Monitoring Neuromuscular Function at Multiple Sites].
abstract: A 39-year-old female with facioscapulohumeral muscular dystrophy (FSHD) was scheduled for thoracoscopic resection of an anterior mediastinal tumor. She had slowly progressive weakness and atrophy in the fascial and shoulder girdle muscles. General anesthesia was induced and maintained with propofol, remifentanil, and fentanyl combined with thoracic paravertebral block. Rocuronium-induced neuromuscular blockade was evaluated with acceleromyography at the corrugator supercilii, masseter, and adductor pollicis muscles. There was no reaction at the atrophic corrugator supercilii muscle in response to train-of-four (TOF) stimulation even before rocuronium administration. In contrast twitch responses at the masseter and adductor pollicis muscles to TOF stimulation could be evoked and the duration of action of rocuronium was found to be similar to that of the normal population. The perioperative course was uneventful. Neuromuscular monitoring sites should be carefully selected in FSHD patients because of possible inability to monitor neuromuscular function at the atrophic muscles.
authors.forename: Shuhei
authors.initials: S
authors.lastname: Matsui
authors.forename: Satoshi
authors.initials: S
authors.lastname: Tanaka
authors.forename: Kenkichi
authors.initials: K
authors.lastname: Kiyosawa
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Tanaka
authors.forename: Mikito
authors.initials: M
authors.lastname: Kawamata
mesh_terms: D000328:Adult; D000732:Androstanols / Q000494:pharmacology*; D000768:Anesthesia, General; D000777:Anesthetics / Q000494:pharmacology*; D005152:Facial Muscles; D005260:Female; D006801:Humans; D008479:Mediastinal Neoplasms / Q000150:complications / Q000601:surgery*; D018482:Muscle, Skeletal / Q000187:drug effects; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000150:complications*; D019148:Neuromuscular Blockade; D061886:Neuromuscular Monitoring; D000077123:Rocuronium
chemical_list: D000732:Androstanols; D000777:Anesthetics; D000077123:Rocuronium