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抗 LGI1 边缘性脑炎伴同时性 NMDAR 抗体阳性的罕见病例。

A rare case of anti-LGI1 limbic encephalitis with concomitant positive NMDAR antibodies.

机构信息

Department of neurology, First Affiliated Hospital of Zhengzhou University, Zhengzhou City, 450052, Henan Province, China.

出版信息

BMC Neurol. 2020 Sep 7;20(1):336. doi: 10.1186/s12883-020-01918-7.

DOI:10.1186/s12883-020-01918-7
PMID:32894089
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7487823/
Abstract

BACKGROUND

N-methyl-D-aspartate receptor (NMDAR) and leucine-rich glioma-inactivated 1 (LGI1) antibodies define the most prevalently recognized autoimmune encephalitis syndromes, while the simultaneous occurrence of both conditions has hardly been published before.

CASE PRESENTATION

We report the case of a 67-year-old patient who presented with generalized tonic-clonic seizures (GTCS) followed by behavioral changes, psychosis, sleep disorders, decreased consciousness, and faciobrachial dystonic seizures. Ancillary findings included serum hyponatremia and imaging evidence of high-intensity lesions within bilateral medial temporal lobes on T2-weighted fluid attenuation inversion recovery. Both LGI1 and NMDAR antibodies were positive in serum and cerebral spinal fluid using transfected cell based assays. Despite prominent clinical features of anti-LGI1 limbic encephalitis (LGI1-LE), the patient also exhibited overlapping symptoms of anti-NMDAR encephalitis, like early-onset GTCS, which might be related to the concomitant positive NMDAR antibodies.

CONCLUSIONS

We report a rare case of LGI1-LE with overlapping symptoms and simultaneous positive NMDAR antibodies. It is necessary to evaluate the presence of NMDAR antibodies in certain LGI1-LE patients with unusual symptoms. However, caution should be exercised in interpreting the observation, given the fact of a single-case study.

摘要

背景

N-甲基-D-天冬氨酸受体(NMDAR)和富含亮氨酸胶质瘤失活 1 型(LGI1)抗体定义了最常见的自身免疫性脑炎综合征,而同时发生这两种情况的情况几乎没有报道过。

病例介绍

我们报告了一例 67 岁患者的病例,该患者出现全身强直阵挛性发作(GTCS),随后出现行为改变、精神病、睡眠障碍、意识下降和面臂肌张力障碍性发作。辅助检查包括血清低钠血症和双侧内侧颞叶 T2 加权液体衰减反转恢复高信号病变的影像学证据。使用转染细胞基础检测,血清和脑脊液中均检测到 LGI1 和 NMDAR 抗体阳性。尽管该患者具有明显的抗 LGI1 边缘性脑炎(LGI1-LE)的临床特征,但也表现出重叠的抗 NMDAR 脑炎症状,如早发性 GTCS,这可能与同时存在的阳性 NMDAR 抗体有关。

结论

我们报告了一例罕见的 LGI1-LE 合并重叠症状和同时存在阳性 NMDAR 抗体的病例。在某些具有不典型症状的 LGI1-LE 患者中,有必要评估 NMDAR 抗体的存在。然而,鉴于这是一个单一病例研究,在解释观察结果时应谨慎。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d16f/7487823/d1fec8f7c76b/12883_2020_1918_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d16f/7487823/b986f43b7ee7/12883_2020_1918_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d16f/7487823/7c6fb0ac8dae/12883_2020_1918_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d16f/7487823/d1fec8f7c76b/12883_2020_1918_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d16f/7487823/b986f43b7ee7/12883_2020_1918_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d16f/7487823/7c6fb0ac8dae/12883_2020_1918_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d16f/7487823/d1fec8f7c76b/12883_2020_1918_Fig3_HTML.jpg

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