El Hajj Aseel, Ruiz Anne, Gavoille Antoine, Couturier Justine, Giraudon Pascale, Benyahya Lakhdar, Malaise Lisa, Bigotte Maxime, Benetollo Claire, Amorim Gaetan, Roux Julia, Leroy Carole, Kogel Ann-Kathrin, Ayzenberg Ilya, Paul Friedemann, Ramanathan Sudarshini, Dale Russell C, Deiva Kumaran, Brilot Fabienne, Marignier Romain
Forgetting Team, CNRS, INSERM, Centre de Recherche en Neurosciences de Lyon CRNL U1028 UMR5292, Université Claude Bernard Lyon 1, Lyon, France.
CNRS, INSERM, Centre de Recherche en Neurosciences de Lyon CRNL U1028 UMR5292, Université Claude Bernard Lyon 1, Bron, France.
J Neurol Neurosurg Psychiatry. 2025 Jul 16;96(8):753-756. doi: 10.1136/jnnp-2024-335579.
Biomarkers for predicting myelin oligodendrocyte glycoprotein antibody (Ab)-associated disease (MOGAD) clinical course are still missing. Binding capacity to a mutant MOG protein variant (MOG-P42S; non-P42) was shown to correlate with an increased relapse risk in adult patients.The objective of our study was to assess the frequency of binding to the non-P42 MOG variant in a cohort of paediatric MOGAD and to investigate its association with specific clinical profiles and disease course.
We included children with MOG-Ab seropositive samples collected after their first demyelinating episode from five different centres. We performed live cell-based assays with native full-length MOG (MOG-FL) and mutant MOG-P42S and correlated the results with clinical data.
Of the 81 MOG-FL identified patients serum, 40 bound the non-P42 MOG. Non-P42 patients exhibited an earlier median age of onset (p=0.002). Phenotype distribution was different between groups (p=0.001), with non-P42 patients predominantly exhibiting acute disseminated encephalomyelitis phenotype. Notably, the non-P42 group was associated with a higher relapse rate (relative rate: 2.6 (95% CI 1.1 to 6.2), p=0.03), adjusted for clinical phenotype.
Non-P42 is a promising biomarker for predicting relapse in paediatric MOGAD patients.
预测髓鞘少突胶质细胞糖蛋白抗体（Ab）相关疾病（MOGAD）临床病程的生物标志物仍然缺乏。已证明与突变型MOG蛋白变体（MOG-P42S；非P42）的结合能力与成年患者复发风险增加相关。我们研究的目的是评估儿科MOGAD队列中与非P42 MOG变体结合的频率，并研究其与特定临床特征和疾病病程的关联。
我们纳入了来自五个不同中心的首次脱髓鞘发作后采集的MOG-Ab血清阳性样本的儿童。我们使用天然全长MOG（MOG-FL）和突变型MOG-P42S进行基于活细胞的检测，并将结果与临床数据相关联。
在81例经MOG-FL鉴定的患者血清中，40例与非P42 MOG结合。非P42患者的中位发病年龄更早（p=0.002）。两组间的表型分布不同（p=0.001），非P42患者主要表现为急性播散性脑脊髓炎表型。值得注意的是，经临床表型调整后，非P42组的复发率更高（相对率：2.6（95%CI 1.1至6.2），p=0.03）。
非P42是预测儿科MOGAD患者复发的一个有前景的生物标志物。
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