Pediatric Autoimmune Encephalitis.
Pediatric Autoimmune Encephalitis.
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DOI：
10.4103/jpn.jpn_185_16
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发表时间：
2017-04
影响因子：
0.5
通讯作者：
Falsaperla R
中科院分区：
文献类型：
--
作者：
Barbagallo M;Vitaliti G;Pavone P;Romano C;Lubrano R;Falsaperla R
自身免疫性（抗体介导的）脑炎 (AE) 正在成为儿童脑病的一个比以前认为的更常见的原因。自身免疫过程可能由感染、疫苗或隐匿性肿瘤引发。在后一种情况下，肿瘤神经自身抗体针对细胞内神经元抗原，但最近发现一组异质性脑炎综合征不具有潜在肿瘤，而是与针对神经元表面或突触抗原的自身抗体相关。神经精神症状在自身免疫性脑病中非常常见；因此，受影响的儿童最初可能会去看精神科医生。神经学特征包括运动障碍、癫痫发作、意识水平改变和认知衰退。通气不足和自主神经功能可能是一个方面。脑脊液中可能存在炎症表现，但相对非特异性。磁共振成像 (MRI) 也可能显示异常情况，为诊断提供线索，特别是在流体衰减反转恢复或 T2 加权图像方面。 AE 对免疫治疗反应良好，及时诊断和治疗非常有益。与未发现恶性肿瘤的患者相比，副肿瘤性脑炎患者更难治疗。在此，作者提供了有关最常见自身免疫性脑炎的临床表现、实验室和影像学结果、治疗和结果的最新文献数据。
Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists. Neurological features are movement disorders, seizures, altered conscious level, and cognitive regression. Hypoventilation and autonomic features may be an aspect. Inflammatory findings in the cerebrospinal fluid may be present but are relatively nonspecific. Magnetic resonance imaging (MRI) may also demonstrate abnormalities that provide clues for diagnosis, particularly on fluid-attenuated inversion recovery or T2-weighted images. AE is well responsive to immune therapy, with prompt diagnosis and treatment strongly beneficial. Patients with paraneoplastic encephalitis are more refractory to treatment compared to those in whom no malignancy is identified. Herein, the authors present an update of literature data on the clinical presentation, laboratory and imaging findings, therapy, and outcomes for the most common autoimmune encephalitides.