The first pediatric anti-lactosylceramide antibody-positive encephalomyeloradiculoneuropathy.
The first pediatric anti-lactosylceramide antibody-positive encephalomyeloradiculoneuropathy.
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DOI：
10.1016/j.heliyon.2022.e10198
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发表时间：
2022-08
期刊：
影响因子：
4
通讯作者：
Abe, Yuichi
中科院分区：
文献类型：
--
作者：
Sasaki, Akiko;Hayakawa, Itaru;Mutoh, Tatsuro;Abe, Yuichi
关键词：
抗乳糖神经酰胺（LacCer）抗体是一种参与脑脊髓神经根病（EMRN）发病机制的抗中性糖脂抗体。它会对中枢和外周神经造成急性和亚急性损伤。然而，到目前为止，还没有抗LacCer抗体阳性EMRN的儿科病例报告。一名12岁女孩出现脑膜炎症状。她随后出现意识障碍和弛缓性四肢瘫痪，并因呼吸衰竭接受机械通气。MRI显示大脑白色物质、基底节、延髓以及脊髓前角C2至Th 1和Th 11至L1水平的病变。神经传导研究显示运动神经轴突神经病变。经类固醇冲击治疗、大剂量免疫球蛋白治疗及血浆置换后，病灶逐渐消退，神经系统症状稳定改善。在发病后6个月，神经系统后遗症最小。虽然血清抗水通道蛋白4和抗髓鞘少突胶质细胞糖蛋白抗体均为阴性，但她在血清和脑脊液中均显示抗乳糖神经酰胺抗体阳性，表明这些抗体可能参与了这种疾病的发病机制。第一例抗LacCer抗体阳性EMRN的儿科病例显示出与成人相同疾病相似的特征。抗中性糖脂抗体应在儿童表现出广泛的神经系统症状，涉及中枢和外周神经。抗中性糖脂抗体;抗乳糖神经酰胺抗体;脑脊髓根神经病;中枢和外周联合脱髓鞘
The anti-lactosylceramide (LacCer) antibody is an anti-neutral glycolipid antibody that is involved in the pathogenesis of encephalomyeloradiculoneuropathy (EMRN). It causes acute and subacute injuries to both the central and peripheral nerves. However, no pediatric cases of anti-LacCer antibody-positive EMRN have been reported so far. A 12-year-old girl presented with signs of meningitis. She subsequently showed disturbance of consciousness and flaccid tetraplegia and was placed on mechanical ventilation due to respiratory failure. MRI showed lesions in the cerebral white matter, basal ganglia, medulla oblongata, as well as the anterior horn of the spinal cord at the C2 to Th1 and Th11 to L1 levels. Nerve-conduction studies showed axonal neuropathy of the motor nerves. After steroid pulse therapy, high-dose immunoglobulin therapy, and plasma exchange, the lesions gradually regressed, and the neurological symptoms improved steadily. The neurological sequelae were minimal at 6 months after disease onset. Although serum anti-aquaporin 4 and anti-myelin oligodendrocyte glycoprotein antibodies were negative, she showed positive anti-lactosylceramide antibody in both serum and cerebrospinal fluid, indicating that these antibodies may be involved in the pathogenesis of this disease. The first pediatric case of anti-LacCer antibody-positive EMRN showed similar features to the same disease in adults. Anti-neutral glycolipid antibodies should be measured in children presenting with a wide range of neurological symptoms involving both central and peripheral nerves. Anti-neutral glycolipid antibodies; Anti-lactosylceramide antibody; Encephalomyeloradiculoneuropathy; Combined central and peripheral demyelination
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影响因子：
5.5
作者：
Sato, Tadashi;Iwabuchi, Kazuhisa;Ogawa, Hideoki
通讯作者：
Ogawa, Hideoki
影响因子：
82.9
作者：
通讯作者：
--
DOI：
10.5692/clinicalneurol.cn-001219
发表时间：
2019-01-30
期刊：
Rinsho shinkeigaku = Clinical neurology
影响因子：
--
作者：
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通讯作者：
Mutoh, Tatsuro
影响因子：
4.6
作者：
Iwabuchi K;Nakayama H;Oizumi A;Suga Y;Ogawa H;Takamori K
通讯作者：
Takamori K
影响因子：
3.4
作者：
Harada, Masaya;Miura, Shiroh;Taniwaki, Takayuki
通讯作者：
Taniwaki, Takayuki