National Pediatric Myoclonus Center and National Pediatric Neuroinflammation Organization, Inc., Orlando, FL, USA.
National Pediatric Myoclonus Center and National Pediatric Neuroinflammation Organization, Inc., Orlando, FL, USA.
J Neuroimmunol. 2018 Aug 15;321:150-156. doi: 10.1016/j.jneuroim.2018.04.007. Epub 2018 Apr 12.
In 208 children with opsoclonus-myoclonus syndrome (OMS), CSF IgG oligoclonal bands (OCB) and 22 immunomarkers in CSF and 21 in serum/blood were measured. In 36 untreated OMS, 58% were OCB(+), whereas 55% of treated OMS were OCB(-). OCB positivity or negativity did not alter concentrations or frequencies of immunomarkers. The phenotypes of OCB(+) and OCB(-) patients were not distinctive. CSF B cells were expanded in untreated OMS regardless of OCB positivity. These data reveal a much higher frequency of OCB positivity in untreated OMS than previously realized and a disconnect between intrathecal OCB and inflammatory mediator production.
在 208 例发作性眼球运动性肌阵挛-小脑共济失调综合征(OMS)患儿中,检测了脑脊液 IgG 寡克隆带(OCB)和 22 种脑脊液免疫标志物及 21 种血清/血液免疫标志物。在 36 例未经治疗的 OMS 患儿中,58%呈 OCB(+),而 55%经治疗的 OMS 患儿呈 OCB(-)。OCB 阳性或阴性并不改变免疫标志物的浓度或频率。OCB(+)和 OCB(-)患者的表型没有明显区别。未经治疗的 OMS 患儿的脑脊液 B 细胞均有扩增,而不论 OCB 是否阳性。这些数据显示未经治疗的 OMS 患儿 OCB 阳性率较之前认识的更高,且鞘内 OCB 与炎症介质产生之间存在脱节。