Conduction block in acute motor axonal neuropathy
Conduction block in acute motor axonal neuropathy
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DOI：
10.1093/brain/awq260
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发表时间：
2010-10-01
期刊：
影响因子：
14.5
通讯作者：
Yuki, Nobuhiro
中科院分区：
文献类型：
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作者：
Kokubun, Norito;Nishibayashi, Momoka;Yuki, Nobuhiro
格林-巴利综合征分为两个主要亚型，急性炎性脱髓鞘性多发性神经病和急性运动轴索神经病。急性运动轴索神经病的特征性电生理学特征是远端复合肌肉动作电位的振幅降低或缺失，表明轴索变性。相反，尸检结果显示急性运动轴索神经病的早期淋巴结变化可能导致运动神经传导阻滞。由于急性运动性轴索神经病中传导阻滞的存在尚未得到充分认识，我们回顾了传导阻滞发生的频率以及传导阻滞逆转或随后发生轴索变性的频率。根据Ho的诊断标准，对18例急性运动轴索神经病患者进行了电诊断，并对其正中神经和尺神经进行了重复的运动神经传导检查。根据美国电诊断医学协会的共识标准，检查这些神经的前臂段和尺神经的肘关节段，以评估传导阻滞。18例急性运动轴索神经病患者中有12例（67%）有明确（n = 7）或很可能（n = 5）传导阻滞。1例患者（6%）在两条神经的前臂段检测到明确的传导阻滞，5例患者（28%）检测到可能的传导阻滞。7名患者（39%）的手肘段尺神经存在明确的传导阻滞，2名患者（11%）可能存在传导阻滞。传导阻滞是可逆的12例中的7例，其次是轴突变性6。所有传导阻滞在三周内消失或开始消退，没有髓鞘再生的电生理证据。1例患者同时出现可逆性传导阻滞和传导阻滞伴轴突变性。有传导阻滞（n = 12）和无传导阻滞（n = 6）的患者以及有可逆性传导阻滞（n = 7）和无可逆性传导阻滞（n = 5）的患者的临床特征和抗神经节苷脂抗体谱相似，表明这两种情况形成连续体;从可逆性传导衰竭到轴突变性的病理生理学谱，可能是由抗体攻击郎维氏结轴膜处的神经节苷脂介导的，表明可逆传导阻滞和传导阻滞后轴突变性和无传导阻滞的轴突变性构成急性运动轴突神经病中的连续电生理条件。
Guillain-Barre syndrome is divided into two major subtypes, acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy. The characteristic electrophysiological features of acute motor axonal neuropathy are reduced amplitude or absence of distal compound muscle action potentials indicating axonal degeneration. In contrast, autopsy study results show early nodal changes in acute motor axonal neuropathy that may produce motor nerve conduction block. Because the presence of conduction block in acute motor axonal neuropathy has yet to be fully recognized, we reviewed how often conduction block occurred and how frequently it either reversed or was followed by axonal degeneration. Based on Ho's criteria, acute motor axonal neuropathy was electrodiagnosed in 18 patients, and repeated motor nerve conduction studies were carried out on their median and ulnar nerves. Forearm segments of these nerves and the across-elbow segments of the ulnar nerve were examined to evaluate conduction block based on the consensus criteria of the American Association of Electrodiagnostic Medicine. Twelve (67%) of the 18 patients with acute motor axonal neuropathy had definite (n = 7) or probable (n = 5) conduction blocks. Definite conduction block was detected for one patient (6%) in the forearm segments of both nerves and probable conduction block was detected for five patients (28%). Definite conduction block was present across the elbow segment of the ulnar nerve in seven patients (39%) and probable conduction block in two patients (11%). Conduction block was reversible in seven of 12 patients and was followed by axonal degeneration in six. All conduction blocks had disappeared or begun to resolve within three weeks with no electrophysiological evidence of remyelination. One patient showed both reversible conduction block and conduction block followed by axonal degeneration. Clinical features and anti-ganglioside antibody profiles were similar in the patients with (n = 12) and without (n = 6) conduction block as well as in those with (n = 7) and without (n = 5) reversible conduction block, indicating that both conditions form a continuum; a pathophysiological spectrum ranging from reversible conduction failure to axonal degeneration, possibly mediated by antibody attack on gangliosides at the axolemma of the nodes of Ranvier, indicating that reversible conduction block and conduction block followed by axonal degeneration and axonal degeneration without conduction block constitute continuous electrophysiological conditions in acute motor axonal neuropathy.