Clinical features of anti-leucine-rich glioma-inactivated 1 encephalitis in northeast China

Zhao, Qian; Sun, Lichao; Zhao, Danyang; Chen, Yanyan; Li, Mengmeng; Lu, Yingxue; Li, Guangjian; Lin, Weihong

doi:10.1016/j.clineuro.2021.106542

Cited by 9 publications

(8 citation statements)

References 24 publications

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“…Considering some of the relapses may occur years after the first onset, the relapse rate might be underestimated. 12 The relapse rates of both types of autoimmune encephalitis did not show a significant difference between different immunotherapy strategies in our study, which does not agree with Zhao et al 29 Further trial studies are needed to evaluate the correlation between relapse and immunotherapy strategy. Recently, a study in anti-NMDAR encephalitis indicated that clinical relapses and remissions are correlated with antibody titers.…”

Section: Discussioncontrasting

confidence: 90%

Clinical characteristics, long-term functional outcomes and relapse of anti-LGI1/Caspr2 encephalitis: a prospective cohort study in Western China

Guo

Liu

Lin

et al. 2022

Ther Adv Neurol Disord

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Objective: To study the clinical characteristics of anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis and anti-contactin-associated protein-like 2 (Caspr2) encephalitis and to investigate factors associated with poor long-term neurological functional outcomes and relapse among patients in western China. Methods: In this single-center prospective cohort study, we consecutively enrolled patients with anti-LGI1 encephalitis and anti-Caspr2 encephalitis from April 2014 to February 2021. Patient outcomes were assessed using the modified Rankin scale. Predictors of long-term functional outcomes and relapse were analyzed. Results: Forty-four anti-LGI1 encephalitis patients [median age: 44 years, range: 18–82 years; females: 25 (56.8%)], 35 anti-Caspr2 encephalitis patients [median age: 43 years, range: 14–80 years; females: 19 (54.3%)], and 5 dual-positive patients [median age: 44 years, range: 36–58 years; females: 5 (100%)] were enrolled. Overall, 86.4% anti-LGI1 encephalitis patients and 80% anti-Caspr2 encephalitis had a favorable neurological functional outcome (mRS 0-2). Tumor occurrence and weight loss were associated with poor long-term functional outcomes in anti-LGI1 encephalitis, whereas in anti-Caspr2 encephalitis, predictors included behavioral disorder at acute phase, abnormalities in brain magnetic resonance imaging, higher modified Rankin scale scores at onset, poor response to the initial immunotherapy at 4 weeks, age at onset<30 years, and relapse ( p<0.05). Overall, 13.6% of anti-LGI1 encephalitis patients and 20% of anti-Caspr2 encephalitis patients had at least one relapse. Sleep disorder at the acute phase was the risk factor of relapse in anti-LGI1 encephalitis, while female, age at onset <30 years, and behavioral disorder at acute phase were the risk factors of relapse in anti-Caspr2 encephalitis (log rank p<0.05). Conclusion: The clinical characteristics such as age, gender, and tumor occurrence rates of anti-LGI1 encephalitis and anti-Caspr2 encephalitis in western China are different from those in the Western countries. Most patients in our study had favorable long-term functional outcomes. The relapse rates are still high in both types of encephalitis, which warrants caution.

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Section: Discussioncontrasting

confidence: 90%

Clinical characteristics, long-term functional outcomes and relapse of anti-LGI1/Caspr2 encephalitis: a prospective cohort study in Western China

Guo

Liu

Lin

et al. 2022

Ther Adv Neurol Disord

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“…Our results revealed that the mRS score at admission was positively correlated with hyponatremia, suggesting that hyponatremia is also related to the severity of the disease. One study demonstrated that the prognosis of anti-LGI1 encephalitis patients with hyponatremia is poor ( 38 ). In this study, CSF CHI3L1 levels were correlated with CSF lactate levels.…”

Section: Discussionmentioning

confidence: 99%

CHI3L1 in the CSF is a potential biomarker for anti-leucine-rich glioma inactivated 1 encephalitis

Wang

et al. 2023

Front. Immunol.

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ObjectiveAnti-leucine-rich glioma inactivated 1(LGI1) encephalitis is one rare autoimmune encephalitis which is accompanied by inflammatory responses. (Anti-leucine-rich glioma inactivated 1 (anti-LGI1) encephalitis is an autoimmune disease mediated by inflammatory responses.)This study aimed to investigate the Chitinase 3-like 1(CHI3L1) in anti-LGI1encephalitis patients and evaluate its association with modified Rankin Scale (mRS) score in anti-LGI1 encephalitis at admission and 6 months follow-up.(This study looked into the relationship between Chitinase 3-like 1 (CHI3L1) and the modified Ranking Scale (mRS) score in anti-LGI1 encephalitis patients at admission and 6 months later.)MethodsThirty-five patients with anti-LGI1 encephalitis and 22 patients with non-inflammatory neurological disease were enrolled in this study. (We enrolled 35 patients with anti-LGI1 encephalitis and 22 patients with non-inflammatory neurological disease.)Cerebrospinal fluid (CSF) and serum levels of CHI3L1 were measured by enzyme-linked immunosorbent assay. (We quantified CHI3L1 in the serum and cerebrospinal fluid (CSF) by performing an enzyme-linked immunosorbent assay.)Patients were evaluated for mRS score at admission and at 6 months follow-up.(We recorded the mRS score of the patients at admission and 6 months later.)ResultsCHI3L1 levels in CSF and serum were highly elevated in patients with anti-LGI1 encephalitis at admission compared those with the controls.(At admission, patients with anti-LGI1 encephalitis had elevated CHI3L1 levels in the CSF and serum.) Additionally, patients presenting with cognitive impairment had significantly higher CSF CHI3L1 levels and mRS scores than those without cognitive impairment symptoms. Patients presenting with only faciobrachial dystonic seizures at admission had lower CSF CHI3L1 levels than those with other symptoms. Finally, CSF CHI3L1 levels were positively correlated with CSF lactate levels.ConclusionCHI3L1 level in CSF is correlated with the severity and prognosis of anti-LGI1 encephalitis. (CSF CHI3L1 levels are correlated with the severity and prognosis of anti-LGI1 encephalitis.)

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“…It has been reported that patients with detectable LGI1 antibodies in cerebrospinal fluid have more frequently have inflammatory factors in cerebrospinal fluid, hyponatremia, and MRI abnormalities. 6,8,21,22 Thus may indicate that the immune response is stronger and more likely to lead to structural damage and permanent functional defects. 6,8,21,22 In addition, it has been reported that an elevated LGI1-IgG CSF index can predict adverse neurological outcomes.…”

Section: Discussionmentioning

confidence: 99%

“…7,16,17 Therefore, early identification and timely management of patients with risk factors related to poor outcomes are important. The relationships among age, 6,[17][18][19] cognitive impairment, 6,17,20,21 FBDS frequency, 6,17,21 cerebrospinal fluid (CSF) LGI1 antibody (Ab), 6,21,22 and poor outcome have also been investigated. However, these findings are inconsistent.…”

Section: Introductionmentioning

confidence: 99%

“…17,18 In addition, the findings of Thompson's study indicated that the risk of poor outcomes increased fivefold in patients with cognitive impairment, 17 but the findings of Dong et al's studies suggested that cognitive impairment had no effect on poor outcome. 6,20,21 Given the current controversial results, we conducted a systematic review and meta-analysis to clarify the effects of various factors on disease outcome. Furthermore, although immunotherapy is widely used, its strategies are mostly formulated by experienced doctors, especially for second-line treatment and maintenance treatment.…”

Section: Introductionmentioning

confidence: 99%

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Efficacy of immunotherapy and prognosis in anti‐LGI1 encephalitis patients: A meta‐analysis

Kong

Gong

et al. 2023

Ann Clin Transl Neurol

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ObjectiveTo assess the efficacy and safety of immunotherapy for LGI1 antibody encephalitis, and consider the predictors of poor outcomes following immunotherapy.MethodsWe searched PubMed and Embase for articles reporting the immunotherapy data of anti‐LGI1 encephalitis patients. The proportions of patients with poor outcomes (modified Rankin Scale [mRS] score > 2) at 3 months, 12 months, and the last follow‐up, as well as the odds ratio [OR] of predictors were pooled.ResultsThe review included 162 articles with 1066 patients. The proportion of patients with poor functional outcomes was 21% at 3 months, 14% at 12 months, and 14% at the last follow‐up after receiving immunotherapy. The proportion of patients with reported relapse was 16.6%. The mean duration from onset to the first relapse was 15.6 months. Predictors significantly associated with poor outcomes were age (increase of 1 year), the presence of cognitive impairment, and CSF LGI1 antibody positive. We did not find a statistically significant association between the worst mRS score in the acute phase, the presence of faciobrachial dystonic seizures (FBDS), days from symptom onset to immunotherapy, second‐line treatment, maintenance immunotherapy, or follow‐up time and outcomes.InterpretationAlthough most patients respond to immunotherapy, a minority of patients still have poor outcomes. Advanced age, cognitive impairment, and CSF LGI1 antibody positive are associated with an increased risk of poor outcomes. However, due to the insufficiency of the data, these conclusions need to be interpreted with caution.

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Clinical features of anti-leucine-rich glioma-inactivated 1 encephalitis in northeast China

Cited by 9 publications

References 24 publications

Clinical characteristics, long-term functional outcomes and relapse of anti-LGI1/Caspr2 encephalitis: a prospective cohort study in Western China

Clinical characteristics, long-term functional outcomes and relapse of anti-LGI1/Caspr2 encephalitis: a prospective cohort study in Western China

CHI3L1 in the CSF is a potential biomarker for anti-leucine-rich glioma inactivated 1 encephalitis

Efficacy of immunotherapy and prognosis in anti‐LGI1 encephalitis patients: A meta‐analysis

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