From the Department of Neurology (S.S., N.K., T.I., Y.N., A.U., T.M.), Fujita Health University School of Medicine, Aichi; and Institute for Environmental and Gender Specific Medicine (H.M., C.I., K.I.), Juntendo University Graduate School of Medicine, Chiba, Japan.
Neurology. 2014 Jan 14;82(2):114-8. doi: 10.1212/WNL.0000000000000015. Epub 2013 Dec 6.
The aim of this study was to review 4 patients with encephalomyeloradiculoneuropathy (EMRN) and assess for autoantibodies against neutral glycolipids.
We studied the progression of clinical, radiologic, neurophysiologic, and CSF findings, as well as anti-neutral glycolipid antibodies in sera.
All patients developed acute or subacute motor weakness and impaired consciousness. Their CSF showed pleocytosis and high immunoglobulin G concentrations. MRI revealed lesions in the brain and spinal cord. Neurophysiologic examinations indicated dysfunction of the spinal cord, nerve roots, and peripheral nerves. Steroid pulsed immunotherapy and/or high dose of IV immunoglobulin replacement therapy resulted in clear and often dramatic clinical improvements. Reactivity to anti-neutral glycolipid antibodies was positive in all patients with acute EMRN but not in the recovery phase. Forty-seven age-matched patients with other neurologic disorders and 28 age-matched healthy volunteers tested negative for reactivity to anti-neutral glycolipid antibodies.
The resolution of radiologic and neurologic abnormalities and altered autoantibody titers against neutral glycolipids after immunotherapy suggest that EMRN is caused by an immune-mediated mechanism. These autoantibodies may be useful biomarkers for EMRN.
本研究旨在回顾 4 例脑脊髓神经根神经病（EMRN）患者，并评估其针对中性糖脂的自身抗体。
我们研究了临床、影像学、神经生理学和 CSF 发现的进展，以及血清中抗中性糖脂抗体。
所有患者均出现急性或亚急性运动无力和意识障碍。其 CSF 显示细胞增多和高免疫球蛋白 G 浓度。MRI 显示脑和脊髓的病变。神经生理学检查显示脊髓、神经根和周围神经功能障碍。皮质类固醇脉冲免疫疗法和/或大剂量静脉注射免疫球蛋白替代疗法导致明显且常为戏剧性的临床改善。所有急性 EMRN 患者的抗中性糖脂抗体反应均为阳性，但在恢复期则为阴性。47 名年龄匹配的其他神经疾病患者和 28 名年龄匹配的健康志愿者对中性糖脂抗体的反应均为阴性。
免疫治疗后放射学和神经异常的缓解以及自身抗体滴度的改变提示 EMRN 是由免疫介导的机制引起的。这些自身抗体可能是 EMRN 的有用生物标志物。
