Yamakage M, Iwasaki H, Kawana S, Ishima T, Namiki A
Department of Anesthesiology, Sapporo Medical College.
Masui. 1991 Jan;40(1):105-8.
Facioscapulohumeral 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.
面肩肱型肌营养不良症（FSHMD）的特征是始于青春期的面部、胸肌和肩胛带肌肉的缓慢进行性萎缩。一名31岁的FSHMD男性患者，三角肌、前锯肌和胸大肌出现营养不良性改变，但手臂和腿部的远端肌肉未出现。他在恩氟烷-氧化亚氮麻醉下，使用6毫克维库溴铵进行了胸-肩胛固定手术。手术结束时，使用加速度换能器同时记录拇指和脚趾的四个成串刺激（TOF）反应。手臂的TOF刺激显示出明显的衰减现象（TOF；0.54），而腿部的TOF测试显示TOF比值完全恢复（TOF；1.0）。患者未显示残留神经肌肉阻滞的临床体征。显然，FSHMD患者手臂和腿部的神经肌肉阻滞程度存在差异。仅在手臂使用外周神经刺激器可能是评估FSHMD患者神经肌肉阻滞的不可靠指标。因此，对于FSHMD患者，应选择两个部位进行神经肌肉阻滞监测。
