Anti-LGI1-associated cognitive impairment: Presentation and long-term outcome
Anti-LGI1-associated cognitive impairment: Presentation and long-term outcome
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DOI：
10.1212/wnl.0000000000003009
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发表时间：
2016-08-23
期刊：
影响因子：
9.9
通讯作者：
Graus, Francesc
中科院分区：
文献类型：
--
作者：
Arino, Helena;Armangue, Thais;Graus, Francesc
目的：研究一系列LGI 1抗体（Ab）相关性认知功能减退患者的临床表现、长期预后和LGI 1 Ab的演变。方法：回顾性分析76例LGI 1 Ab相关性认知功能减退患者的临床资料。表现的综合征分为边缘系统脑炎（LE）、非LE或脑病（MRI正常且无CSF细胞增多）。复发的频率和临床结果进行了评估，在48例患者长期随访（中位数39个月，范围18-200）。结果：63例（83%）发展LE，3（4%）非LE，10（13%）脑病。所有患者均接受类固醇、IV免疫球蛋白（IG）或两者同时使用。在2年时，17例（35%; 95% CI 21%-49%）完全恢复，17例（35%）功能独立但未达到基线或无法重返工作岗位，11例（23%）因中度或重度认知缺陷需要帮助，3例（6%）死亡。不良结局的预测因素包括对初始免疫治疗无应答（比值比23.0，95%CI 2.4-215.6，p = 0.006）和13例患者（27%）发生的临床复发（比值比10.2，95%CI 1.0-100.1，p = 0.047）。在所有患者中，LGI 1 Ab均为IgG 4，通常在血清和CSF中均可检出（仅CSF，8%）。16例长期随访的患者中，4例血清抗体仍为阳性，3例完全恢复，无一例转IgG 1型。结论：13%的LGI 1抗体阳性患者发生认知功能障碍，但无脑炎诊断标准。免疫治疗后，只有35%的患者恢复到基线认知功能。在完全临床恢复后，血清LGI 1 Ab仍可检测到。
Objective:We investigated a series of patients with LGI1 antibody (Ab)-related cognitive deterioration to determine the clinical presentation, long-term outcome, and LGI1 Ab evolution.Methods:We retrospectively analyzed the clinical information of 76 patients with LGI1 Ab-related cognitive deterioration. Presenting syndromes were classified as limbic encephalitis (LE), non-LE, or encephalopathy (normal MRI and no CSF pleocytosis). Frequency of relapses and clinical outcome were assessed in 48 patients with prolonged follow-up (median 39 months, range 18-200).Results:Sixty-three patients (83%) developed LE, 3 (4%) non-LE, and 10 (13%) encephalopathy. All patients received steroids, IV immunoglobulins (Ig), or both. At 2 years, 17 (35%; 95% CI 21%-49%) fully recovered, 17 (35%) became functionally independent but not at baseline or were unable to return to work, 11 (23%) required assistance because of moderate or severe cognitive deficits, and 3 (6%) died. Predictors of bad outcome included no response to initial immunotherapy (odds ratio 23.0, 95% CI 2.4-215.6, p = 0.006) and clinical relapses (odds ratio 10.2, 95% CI 1.0-100.1, p = 0.047) that occurred in 13 patients (27%). In all patients, the LGI1 Abs were IgG4 and usually detectable in both serum and CSF (only CSF, 8%). Abs remained positive in serum of 4 of 16 patients with long-term follow-up; 3 of these 4 patients fully recovered and none showed class switch to IgG1.Conclusions:Up to 13% of patients with LGI1 Abs develop cognitive impairment without criteria of encephalitis. After immunotherapy, only 35% of patients return to their baseline cognitive function. Serum LGI1 Abs may remain detectable after full clinical recovery.