• M Yamakage, H Iwasaki, S Kawana, T Ishima, and A Namiki.
• Department of Anesthesiology, Sapporo Medical College.
• Masui. 1991 Jan 1; 40 (1): 105-8.

AbstractFacioscapulohumeral muscle dystrophy (FSHMD) is characterized by slowly progressive wasting of facial, pectoral and shoulder-girdle muscles that begins in adolescence. A 31 year-old man with FSHMD had dystrophic changes in the deltoid, anterior serratus and pectoralis major muscles but not in the distal muscle of his arms and legs. He underwent an operation for thoraco-scapula fixation under enflurane-nitrous oxide anesthesia with vecuronium 6 mg. At the end of the surgical procedure, the train-of-four (TOF) responses of a thumb and a toe, as measured by using an acceleration transducer, were recorded simultaneously. TOF stimulation in an arm demonstrated an apparent fade phenomenon (TOF; 0.54), while a TOF test in the leg showed complete recovery of the TOF ratio (TOF; 1.0). The patient revealed no clinical signs of residual neuromuscular blockade. It was clear that there was a difference in the degree of neuromuscular block between the arm and the leg in a FSHMD patient. Use of the peripheral nerve stimulator only in the arm may be an unreliable guide to assess neuromuscular block in FSHMD patients. Therefore, two sites should be chosen for monitoring neuromuscular blockade in a FSHMD patient.

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