三神经参与的布尔巴开始的抗IgLON5疾病
Florent Cluse1, Marc Hermier2, Genevieve Demarquay2
1From the ALS Resource and Competence Center (F.C., J.S., A.P., E.B.), Pierre Wertheimer Hospital, Hospices Civils de Lyon; Electroneuromyography and Neuromuscular Diseases Unit (F.C.), Pierre Wertheimer Hospital, Hospices Civils de Lyon; Department of Neuroradiology (M.H.), Pierre Wertheimer Hospital, Hospices Civils de Lyon; Neurophysiology & Epilepsy Unit (G.D.), Neurological Hospital P. Wertheimer, Hospices Civils de Lyon; Reference Centre for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis (V.R., J.H.), Hospices Civils de Lyon, Neurological Hospital, Bron; and Centre de Compétences des Maladies Neuro Musculaires (M.M.), CHU Grenoble Alpes, Grenoble, France. florent.cluse@chu-lyon.fr.
抗IgLON5疾病可以模仿运动神经元疾病,表现为球膜症状和非典型的三角神经病变. 在这种情况下,对IgLON5自身抗体的测试对于诊断至关重要.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 神经生理学 神经生理学
背景情况:
- 抗IgLON5疾病 (IgLON5-D) 呈现的神经症状,可以模仿其他疾病,如肌缩侧面硬化症.
- 三神经病变是一种未被认可的特征,可能发生在IgLON5-D.中.
研究的目的:
- 描述两个非典型的三角神经病变的病例,这些病例发生在有抗IgLON5疾病的患者身上.
- 强调识别这些特征对于准确诊断的重要性.
主要方法:
- 临床评估,包括消化不良,,和声.
- 电生理学研究 (针头肌图,眼反射,激光唤起的潜能).
- 大脑MRI和IgLON5自身抗体的血清/中枢神经液检测.
主要成果:
- 两名患者均表现出腹筋症状和阳性IgLON5自身抗体.
- 核磁共振扫描显示三角神经扩大;电生理学证实三角神经病变和小纤维功能障碍在一个病人.
- 一位患者出现了面部疼痛,而另一位没有.
结论:
- 形三角神经病变和MRI异常在患有凸骨症状的患者中,需要对IgLON5自身抗体进行测试.
- 早期诊断IgLON5-D对于适当的管理至关重要.
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