TY - T1的偏离规范儿童全脑灰质和深度增长MOGAD,女士,单相血清反应阴性的髓鞘脱失JF -神经学乔-神经病学SP - e425 LP - e437做- 10.1212 / WNL。首页0000000000207429六世- 101 - 4盟会Fadda AU -阿隆索Cardenas de la Parra盟盟茱莉亚O’mahony -帕特里克水域盟- e·安叶盟-阿米特酒吧或非盟-露丝安上盟Sridar Narayanan盟——道格拉斯·l·阿诺德AU - d·路易斯·柯林斯盟Brenda Banwell AU -加拿大儿科的脱髓鞘疾病网络Y1 - 2023/07/25 UR - //www.ez-admanager.com/content/101/4/e425.abstract N2 -背景和目标Pediatric-acquired脱髓鞘与抗体针对相关的中枢神经系统髓少突细胞糖蛋白(撤走;首页MOG antibody-associated疾病[MOGAD])发生单相或疾病复发和变量,但往往广泛T2损伤大脑。成熟期间MOGAD对大脑发育的影响是未知的。我们量化的影响小儿MOGAD对大脑的增长轨迹,而这与年龄的增长轨迹,sex-matched健康儿童和儿童多发性硬化症(MS,慢性复发疾病导致失败的正常的大脑发育和脑容量损失)和单相血清反应阴性的髓鞘脱失。方法我们在事件注册包括孩子攻击的前瞻性纵向加拿大儿科脱髓鞘疾病研究招募3最大的招生网站,接受研究的大脑核磁共振扫描,检测血清MOG-IgG。MOG-IgG被诊断出患有MOGAD儿童血清反应阳性的。每2017年麦当劳女士被诊断标准。单相血清反应阴性的髓鞘脱失确认儿童没有复发的临床和MRI证据MOG-IgG和aquaporin-4-IgG脱髓鞘和消极的结果。整个区域脑容量通过对称非线性计算注册模板。我们计算age-normalized sex-normalized z分数813年脑容量使用规范的数据集来自正常儿童的大脑核磁共振扫描,利用混合效应模型来评估潜在的偏差从大脑的增长轨迹。结果我们的46 MOGAD患儿的脑容量评估,与女士26日,51与单相血清反应阴性的脱髓鞘综合症。 Children with MOGAD exhibited delayed (p < 0.001) age-expected and sex-expected growth of thalamus, caudate, and globus pallidus, normalized for the whole brain volume. Divergence from expected growth was particularly pronounced in the first year postonset and was detected even in children with monophasic MOGAD. Thalamic volume abnormalities were less pronounced in children with MOGAD compared with those in children with MS.Discussion The onset of MOGAD during childhood adversely affects the expected trajectory of growth of deep gray matter structures, with accelerated changes in the months after an acute attack. Further studies are required to better determine the relative impact of monophasic vs relapsing MOGAD and whether relapsing MOGAD with attacks isolated to the optic nerves or spinal cord affects brain volume over time.ADEM=acute disseminated encephalomyelitis; ADS=acquired demyelinating syndromes; CAL=Children's Hospital in Calgary; CHOP=Children's Hospital of Philadelphia; CPDDS=Canadian Pediatric Demyelinating Disease Study; EDSS=Expanded Disability Status Scale; ETL=echo train length; HSC=Hospital for Sick Children; NIHPD=NIH Study of Normal Brain Development; ON=optic neuritis; PNC=Philadelphia Neurodevelopmental Cohort; POMS=pediatric-onset multiple sclerosis; TM=transverse myelitis ER -
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