Bien C G, Bien C I
Epilepsy Center Bethel, Krankenhaus Mara, Maraweg 17-21, 33617 Bielefeld, Germany.
Laboratory Krone, Bad Salzuflen, Germany.
Neurol Res Pract. 2020 Jan 3;2:4. doi: 10.1186/s42466-019-0047-8. eCollection 2020.
Autoimmune encephalitides with neural and glial antibodies have become an attractive field in neurology because the antibodies are syndrome-specific, explain the pathogenesis, indicate the likelihood of an underlying tumor, and often predict a good response to immunotherapy. The relevance and the management of antibody-associated encephalitides in the pediatric age group are to be discussed.
Subacutely evolving, complex neuropsychiatric conditions that are otherwise unexplained should raise the suspicion of autoimmune encephalitis. Determination of autoantibodies is the key diagnostic step. It is recommended to study cerebrospinal fluid and serum in parallel to yield highest diagnostic sensitivity and specificity. The most frequently found antibodies are those against the N-methyl-D-asparate receptor, an antigen on the neural cell surface. The second most frequent antibody is directed against glutamic acid decarboxylase 65 kDa, an intracellular protein, often found in chronic conditions with questionable inflammatory activity. Immunotherapy is the mainstay of treatment in autoimmune encephalitides. Steroids, apheresis and intravenous immunoglobulin are first-line interventions. Rituximab or cyclophosphamide are given as second-line treatments. Patients with surface antibodies usually respond well to immunotherapy whereas cases with antibodies against intracellular antigens most often do not.
With few exceptions, the experience in adult patients with autoimmune encephalitides can be applied to patients in the pediatric age range.
伴有神经和胶质细胞抗体的自身免疫性脑炎已成为神经病学中一个引人关注的领域，因为这些抗体具有综合征特异性，可解释发病机制，提示潜在肿瘤的可能性，并且常常预示对免疫治疗有良好反应。本文将讨论儿童年龄组中抗体相关性脑炎的相关性及管理。
以亚急性方式进展且病因不明的复杂神经精神状况应引起对自身免疫性脑炎的怀疑。自身抗体的检测是关键的诊断步骤。建议同时检测脑脊液和血清，以获得最高的诊断敏感性和特异性。最常发现的抗体是针对神经细胞表面抗原N-甲基-D-天冬氨酸受体的抗体。第二常见的抗体是针对谷氨酸脱羧酶65 kDa的抗体，这是一种细胞内蛋白，常见于炎症活性存疑的慢性疾病中。免疫治疗是自身免疫性脑炎的主要治疗方法。类固醇、血液成分单采和静脉注射免疫球蛋白是一线干预措施。利妥昔单抗或环磷酰胺用作二线治疗。有表面抗体的患者通常对免疫治疗反应良好，而有细胞内抗原抗体的病例大多反应不佳。
除少数例外情况外，成人自身免疫性脑炎的经验可应用于儿童年龄组的患者。
