Berciano José, Orizaola Pedro, Gallardo Elena, Pelayo-Negro Ana L, Sánchez-Juan Pascual, Infante Jon, Sedano María J
Service of Neurology, University Hospital "Marqués de Valdecilla (IDIVAL)", University of Cantabria, "Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)", Santander, Spain.
Service of Clinical Neurophysiology, University Hospital "Marqués de Valdecilla (IDIVAL)", Santander, Spain.
Clin Neurophysiol Pract. 2019 Nov 30;5:1-9. doi: 10.1016/j.cnp.2019.11.003. eCollection 2020.
Using recent optimized electrodiagnostic criteria sets, we primarily aimed at verifying the accuracy of the initial electrophysiological test in very early Guillain-Barré syndrome (VEGBS), ≤4 days of onset, compared with the results of serial electrophysiology. Our secondary objective was to correlate early electrophysiological results with sonographic nerve changes.
This is a retrospective study based on consecutive VEGBS patients admitted to the hospital. Each patient had serial nerve conduction studies (NCS) in at least 4 nerves. Initial NCS were done within 4 days after onset, and serial ones from the second week onwards. Electrophysiological recordings of each case were re-evaluated, GBS subtype being established accordingly. Nerve ultrasonography was almost always performed within 2 weeks after onset.
Fifteen adult VEGBS patients were identified with a mean age of 57.8 years. At first NCS, VEGBS sub-typing was only possible in 3 (20%) cases that showed an axonal pattern, the remaining patterns being mixed (combining axonal and demyelinating features) in 6 (40%), equivocal in 5 (33.3%), and normal in 1 (6.7%). Upon serial NCS, 7 (46.7%) cases were categorized as acute demyelinating polyneuropathy, 7 (46.7%) as axonal GBS, and 1 (6.6%) as unclassified syndrome. Antiganglioside reactivity was detected in 5 out of the 7 axonal cases. Nerve US showed that lesions mainly involved the ventral rami of scanned cervical nerves.
Serial electrophysiological evaluation is necessary for accurate VEGBS subtype classification. Ultrasonography helps delineate the topography of nerve changes.
We provide new VEGBS pathophysiological insights into nerve conduction alterations within the first 4 days of the clinical course.
运用近期优化的电诊断标准集,我们的主要目的是验证在发病≤4天的极早期吉兰-巴雷综合征(VEGBS)中,初始电生理检查结果与系列电生理检查结果相比的准确性。我们的次要目的是将早期电生理结果与超声检查的神经变化相关联。
这是一项基于连续收治入院的VEGBS患者的回顾性研究。每位患者至少对4条神经进行了系列神经传导研究(NCS)。初始NCS在发病后4天内进行,系列NCS从第二周开始。对每个病例的电生理记录进行重新评估,并据此确定GBS亚型。几乎所有患者在发病后2周内进行了神经超声检查。
共确定了15例成年VEGBS患者,平均年龄为57.8岁。在首次NCS时,仅3例(20%)表现为轴索性模式的病例能够进行VEGBS亚型分类,其余模式中,6例(40%)为混合型(兼具轴索和脱髓鞘特征),5例(33.3%)不明确,1例(6.7%)正常。在系列NCS检查后,7例(46.7%)被归类为急性脱髓鞘性多发性神经病,7例(46.7%)为轴索性GBS,1例(6.6%)为未分类综合征。7例轴索性病例中有5例检测到抗神经节苷脂反应性。神经超声显示病变主要累及扫描的颈神经前支。
系列电生理评估对于准确的VEGBS亚型分类是必要的。超声检查有助于描绘神经变化的部位。
我们为临床病程前4天内神经传导改变提供了关于VEGBS病理生理学的新见解。