首页> 外文期刊>Journal of the Neurological Sciences: Official Bulletin of the World Federation of Neurology >Motor and sensory conduction failure in overlap of Guillain-Barre and Miller Fisher syndrome: two simultaneous cases.
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Motor and sensory conduction failure in overlap of Guillain-Barre and Miller Fisher syndrome: two simultaneous cases.

机译:格林-巴利综合征和米勒·费舍综合症重叠的运动和感觉传导衰竭:同时发生的两种情况。

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We report 2 patients diagnosed simultaneously with an overlap of Guillain-Barre syndrome (GBS) and Miller Fisher syndrome (MFS), who had anti-GT1a, anti-GQ1b, anti-GD1a and anti-GD1b antibodies. There was no identifiable specific preceding infection. Both patients presented with upper and lower limb paresthesias and severe weakness, bulbar and facial weakness, ophthalmoparesis and areflexia. In one, electrophysiology demonstrated multifocal conduction blocks (CBs) and mild motor conduction velocity slowing in intermediate segments and absent sensory nerve action potentials (SNAPs). The patient improved rapidly and fully recovered within 18 days from onset. CBs resolved, distal compound muscle action potential (CMAP) amplitudes increased and SNAPs normalized on subsequent testing. In the other patient, initial studies showed low/normal CMAPs, with absent SNAPs, without demyelinating features. This patient fully recovered within 21 days from onset. CMAPs markedly increased, SNAPs improved marginally. These 2 patients exhibited features indicative of the pathophysiological mechanism of conduction failure in motor and sensory fibers. This phenomenon relates to rapidly resolving CBs possibly induced by the transitory and limited attack of antiganglioside antibodies at the axolemma of the nodes of Ranvier not progressing to axonal degeneration. These cases widen the range of GBS subtypes in which reversible conduction failure has been described, to include overlap syndromes with MFS. The factors determining the electrophysiology, as well as the rate, degree and quality of recovery in GBS subtypes remain uncertain at the present time.
机译:我们报告2例同时被诊断患有格林巴利综合征(GBS)和米勒费舍尔综合征(MFS)重叠的患者,这些患者具有抗GT1a,抗GQ1b,抗GD1a和抗GD1b抗体。没有可识别的特定先前感染。两名患者均出现上肢和下肢感觉异常,严重无力,延髓和面部无力,眼轻瘫和无力。在一项研究中,电生理学证实了多灶性传导阻滞(CB)和中等程度的轻度运动传导速度减慢,并且缺乏感觉神经动作电位(SNAP)。病人起病后18天内迅速好转并完全康复。 CB解析,远端复合肌肉动作电位(CMAP)幅度增加,并且SNAP在后续测试中恢复正常。在另一位患者中,初步研究显示低/正常的CMAP,无SNAP,无脱髓鞘特征。该患者在发病后21天内完全康复。 CMAP显着增加,SNAP略有改善。这2例患者表现出指示运动和感觉纤维传导衰竭的病理生理机制的特征。这种现象与迅速解决CB有关,可能是由于抗神经节苷脂抗体在Ranvier结节的轴突处的短暂过渡和有限的攻击而诱发的,但并未进展为轴突变性。这些情况扩大了已描述可逆传导失败的GBS亚型的范围,以包括MFS重叠综合征。目前,决定电生理的因素以及GBS亚型恢复的速率,程度和质量仍不确定。

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