Abnormal Neurons in Teratomas in NMDAR Encephalitis
Abnormal Neurons in Teratomas in NMDAR Encephalitis
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DOI：
10.1001/jamaneurol.2014.488
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发表时间：
2014-06-01
期刊：
影响因子：
29
通讯作者：
Munoz, David G.
中科院分区：
文献类型：
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作者：
Day, Gregory S.;Laiq, Simin;Munoz, David G.
卵巢畸胎瘤常发生于N-甲基-D-天冬氨酸受体（NMDAR）脑炎患者，但NMDAR脑炎很少发生于卵巢畸胎瘤患者。了解为什么少数畸胎瘤患者出现自身免疫性脑炎可能会改善NMDAR脑炎和其他畸胎瘤相关自身免疫性疾病的管理。目的描述从NMDAR脑炎患者中切除的卵巢畸胎瘤内神经胶质细胞成分的独特组织。一项对照研究，比较了从1月1日至2011年1月30日连续累积的NMDAR脑炎病例中切除的卵巢畸胎瘤的病理特征，2009年和2013年12月15日，以及2012年6月1日至6月30日期间从对照组切除的卵巢畸胎瘤，研究对象：5例畸胎瘤相关NMDAR脑炎患者，血清或脑脊液中存在抗中枢神经系统（CNS）NMDAR自身抗体，38例对照（39例卵巢畸胎瘤）无神经系统症状或体征。使用直接显微镜检查福尔马林固定、石蜡包埋的卵巢畸胎瘤中CNS组织和炎性浸润的存在，主要结果和指标：卵巢畸胎瘤切除病例与对照组中的（发育不良）CNS神经元成分，结果5例NMDAR脑炎畸胎瘤中4例有中枢神经系统神经元成分，39例对照组中有20例（P =.36）。5例中有4例畸胎瘤中可见非典型神经元，而39例对照组中未见，这是鉴别畸胎瘤合并NMDAR脑炎的可靠依据（P
IMPORTANCE Ovarian teratomas are frequently described in patients with N-methyl-D-aspartate receptor (NMDAR) encephalitis, yet NMDAR encephalitis is rarely described in patients with ovarian teratomas. Understanding why a minority of patients with teratomas are seen with autoimmune encephalitis may improve the management of NMDAR encephalitis and other teratoma-associated autoimmune diseases.OBJECTIVE To characterize the unique organization of neuroglial elements within ovarian teratomas resected from patients with NMDAR encephalitis.DESIGN Case-control study comparing the pathological features of ovarian teratomas resected from consecutively accrued cases with NMDAR encephalitis between January 1, 2009, and December 15, 2013, and ovarian teratomas resected from controls between June 1, 2012, and June 30, 2013.SETTING Pathology tissue database at a tertiary academic care center.PARTICIPANTS Five cases with teratoma-associated NMDAR encephalitis and serum or cerebrospinal fluid autoantibodies against central nervous system (CNS) NMDAR and 38 controls (39 ovarian teratomas) without neurological symptoms or signs.EXPOSURES Formalin-fixed, paraffin-embedded ovarian teratomas were examined for the presence of CNS tissue and inflammatory infiltrates using direct microscopy, enhanced with standard histopathological and immunological stains.MAIN OUTCOMES AND MEASURES Frequency of detection of atypical (dysplastic) CNS neuronal elements in ovarian teratomas resected from cases vs controls, as well as characterization of the relationship between atypical neurons and immune infiltrates.RESULTS Central nervous system neuronal elements were detected in 4 of 5 teratomas resected from cases with NMDAR encephalitis and in 20 of 39 controls (P =.36). Atypical neurons were seen within teratomas resected from 4 of 5 cases but not in 39 controls, reliably distinguishing teratomas associated with NMDAR encephalitis (P