Reisin R C, Cersósimo R, García Alvarez M, Massaro M, Fejerman N
Department of Neurology, Hospital Británico, Buenos Aires, Argentina.
Muscle Nerve. 1993 Dec;16(12):1310-6. doi: 10.1002/mus.880161207.
We identified 5 of 44 consecutive children (11%) with Guillain-Barré syndrome who had electrophysiologic evidence of severe reduction of the mean amplitude of the compound motor action potentials (mean CMAP amplitude < 10% of lower limit of normal). EMG studies revealed profuse fibrillation activity in distal and proximal muscles after 2 weeks of onset. We compared this group with 16 consecutive children with GBS prospectively evaluated over 1 year, all of whom presented a mean CMAP amplitude > 10% of lower limit of normal. Children in the first group were more likely to require assisted ventilation (60% vs. 6.2%) and were more frequently quadriplegic at the peak of their disability (80% vs. 18.7%). They also required longer periods to improve one functional grade (mean 63.6 days vs. 16.6 days) and to become ambulatory (mean 156 days vs. 17.6 days). Moreover, only the children in the first group had distal atrophy of four limbs after 1 year of follow-up. Severe reduction of the mean amplitude of the CMAPs in children with GBS identifies a subgroup of patients with axonal damage that produces more severe weakness and delayed recovery.
我们在44例连续性格林-巴利综合征患儿中识别出5例（11%），这些患儿有复合运动动作电位平均波幅严重降低的电生理证据（复合肌肉动作电位平均波幅<正常下限的10%）。肌电图研究显示，起病2周后，远端和近端肌肉出现大量纤颤活动。我们将该组患儿与16例连续性格林-巴利综合征患儿进行比较，这16例患儿是在1年时间里接受前瞻性评估的，他们的复合肌肉动作电位平均波幅均>正常下限的10%。第一组患儿更有可能需要辅助通气（60% 对6.2%），在残疾高峰时四肢瘫痪的情况更常见（80% 对18.7%）。他们还需要更长时间才能改善一个功能等级（平均63.6天对16.6天）和实现行走（平均156天对17.6天）。此外，随访1年后，只有第一组患儿出现四肢远端萎缩。格林-巴利综合征患儿中复合肌肉动作电位平均波幅严重降低，表明存在轴索性损伤的一组患儿，其肌无力更严重，恢复延迟。
