Kıztanır Hikmet, Bektaş Gonca, Yıldız Edibe Pembegül, Uzunhan Tuğçe Aksu, Tatlı Burak, Aydınlı Nur, Çalışkan Mine, Özmen Meral
Department of Pediatrics, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Department of Pediatric Neurology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Brain Dev. 2017 Mar;39(3):248-251. doi: 10.1016/j.braindev.2016.10.008. Epub 2016 Nov 4.
To determine the incidence and clinical relevance of neuronal autoantibodies in children with demyelinating syndromes.
We conducted a prospective study including 31 consecutive children with demyelinating syndromes. Four patients with N-Methyl-D-aspartate receptor (NMDAR) encephalitis, 32 patients with Guillain-Barre syndrome, 13 children with benign childhood epilepsy, and 28 healthy children were used as controls. Prior to initiating immunomodulatory therapy, serum samples were tested for antibodies against NMDAR, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) 1, AMPAR2, leucine-rich glioma-activated protein 1, contactin-associated protein 2, gamma-aminobutyric acid B receptors, paraneoplastic ma antigen 2 (PNMA2/Ta), Yo, Ri, Hu, CV2, amphiphysin, and aquaporin-4 by indirect immunofluorescence assays.
Three anti-neuronal antibodies were detected; NMDAR antibody in one with multiple sclerosis, PNMA2/Ta antibody in one with multiple sclerosis, and Yo antibody in one with clinically isolated syndrome. The positivity rate of neuronal autoantibodies in demyelinating syndrome was 10%. All seropositive patients were found to be negative for tumor screening. None of these patients exhibited symptoms of encephalitis.
Children with demyelinating syndromes without symptoms of encephalitis can be positive for anti-neuronal antibodies.
确定脱髓鞘综合征患儿中神经元自身抗体的发生率及临床相关性。
我们进行了一项前瞻性研究,纳入了31例连续的脱髓鞘综合征患儿。4例N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者、32例吉兰-巴雷综合征患者、13例儿童良性癫痫患者及28例健康儿童作为对照。在开始免疫调节治疗前,通过间接免疫荧光法检测血清样本中针对NMDAR、α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体(AMPAR)1、AMPAR2、富含亮氨酸的胶质瘤激活蛋白1、接触蛋白相关蛋白2、γ-氨基丁酸B受体、副肿瘤ma抗原2(PNMA2/Ta)、Yo、Ri、Hu、CV2、 amphiphysin和水通道蛋白4的抗体。
检测到3种抗神经元抗体;1例多发性硬化症患者检测到NMDAR抗体,1例多发性硬化症患者检测到PNMA2/Ta抗体,1例临床孤立综合征患者检测到Yo抗体。脱髓鞘综合征中神经元自身抗体的阳性率为10%。所有血清阳性患者的肿瘤筛查均为阴性。这些患者均未出现脑炎症状。
无脑炎症状的脱髓鞘综合征患儿抗神经元抗体可能呈阳性。