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Objective:To analyze the relationship between serum antibody titers, clinical characteristics, and prognosis in patients with encephalitis induced by anti leucine-rich glioma inactivated 1 (LGI1) antibody.Methods:Clinical data of 20 patients diagnosed with encephalitis in the Department of Neurology, First Hospital of Shanxi Medical University from February 2015 to February 2021 were collected and retrospectively analyzed. Patients were divided into 2 groups based on their serum anti LGI1 antibody titers, namely the high titer group (1∶100, 1∶320) and the low titer group (1∶10, 1∶32). The clinical characteristics, laboratory test results, and prognosis of the 2 groups of patients were compared. Relusts The age of the 20 patients with anti LGI1 antibody encephalitis ranged from 27 to 69 (53.5±11.2) years, with a male to female ratio of 1∶1. There were 9 patients in the low titer group and 11 patients in the high titer group. There was no statistically significant difference in the types and quantities of clinical symptoms between the 2 groups. Patients in the high titer group were more prone to abnormal lesions on cranial magnetic resonance imaging (10/11 vs 3/9, P=0.014). There was no statistically significant difference between the 2 groups in the presence or absence of cerebrospinal fluid anti LGI1 antibodies (9/11 vs 4/9, P=0.160). During the follow-up, it was found that 1/20 patient died of pulmonary embolism, 7/20 of patients were able to recover to their predisease state, and 9/20 of patients had residual memory impairment. In the high titer group, 3/11 of patients experienced recurrence, while there was no recurrence in the low titer group. There was no statistically significant difference in the neurological prognosis between the 2 groups at 3 months of discharge and follow-up (the number of patients whose modified Rankin Scale score≤2: 10/10 vs 8/9, P=0.474). Conclusions:Patients with high serum anti LGI1 antibody titers are more likely to develop intracerebral lesions. Higher antibody titers may be associated with a higher risk of disease recurrence. There was no significant correlation between serum antibody titers and neurological outcomes.
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To report a typical case of Morvan syndrome with positive anti-leucine rich glioma-inactivated 1(LGI1) and contactin-associated protein 2 (CASPR2) antibodies in serum and cerebrospinal fluid. A 39-years-old female initially presented weakness of extremeties. The main symptoms included paroxysmal limb pain, wheezing, itching, muscle twitching, epilepsy, hypomnesia, dysphoria, apathy, intractable insomnia, salivation and sweating. Tests of electrolytes found hypokalemia (2.7-3.1 mmol/L) and hyponatremia (130-136 mmol/L). Arterial blood gas analysis showed hypoxemia (oxygen saturation 50%-70%). Total thyroxine (TT4) was elevated to 207 nmol/L with positive thyroid peroxidase antibody (TPO-Ab) and thyroglobulin antibody (TG-Ab). LGI1and CASPR2 antibodies (CBA method) were positive in both serum and cerebrospinal fluid, and the remaining antibodies related to autoimmune encephalitis and paraneoplastic syndrome were negative. Head MRI was almost normal, while mild abnormalities were found in electroencephalogram. Electromyography showed slightly increased voltage of left quadriceps motor unit potential. After treated with corticosteroids, IVIG and mycophenolate mofetil, the patient completely improved. Cognitive function scores recovered from MoCA/MMSE (16/24) to MoCA/MMSE (26/29). Positivity of LGI1/CASPR2 antibodies both in serum/cerebrospinal fluid are rarely seen in patients with Morvan syndrome. Steroids and immunosuppressants are suggested for treatment as early as possible.
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Objective: To study the clinical characteristics of eucine-rich glioma-inactivated 1 protein (LGI1) antibody positive limbic encephalitis (LE), and to provide the reference for its diagnosis and streatment. Methods: The clinical data of a patient with anti-LGI1 antibody positive LE who presented the hypothalamus symptoms was analyzed, the literature review was performed, and the diagnosis and treatment methods of the LE patients were summarized. Results: The patient showed the following symptoms 2 months before hospitalisation, such as sexual dysfunction, overeating, taciturnity, daytime hyposia, intermittent sweating, fever without inducement, hyponatremia, intermittent memory loss and faciobrachial dystonic seizure (FBDS), and the above symptoms progressively exacerbated. After hospitalization, the Mini-Mental State Examination (MMSE) score was 22; the patient could' t finish the evaluation of Montreal Cognitive Assessment Scale (MoCA); the serum anti-LGI1 antibody was positive (1 100), and the cerebrospinal fluid anti-LGI1 antibody was positive (1:10). The MRI results showed T1 low signal, T2, Flair high signal, DWI high signal in bilateral hippocampus, amygdala and medial temporal lobe, especially in the right side. The patient was diagnosed as anti-LGIl antibody positive LE, whose symptoms relieved rapidly after administration of immunoglobulin and steroid therapy. After 6- monthfollow-up, the patient recovered completely. Conclusion: The positive results of serum and cerebrospinal fluid anti-LGIl antibody in the patients combined with the MRI results of head can confirm the diagnosis of anti-LGIl antibody positive LE. The hypothalamus symptoms, memory loss and FBDS are improved rapidly after immunoglobulin and steroid therapy. The hypothalamic injury possibly exist in the early-stage of anti-LGIl antibody positive LE.
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Objective To evaluate the efficacy and safety of mycophenolate mofetil (MMF) in antileucine-rich glioma-inactivated 1 (LGI1) encephalitis by analyzing the clinical and immunologic data of patients treated with MMF in this prospective cohort of anti-LGI1 encephalitis.Methods Patients treated with MMF for more than one year in Peking Union Medical College Hospital were included in this study.MMF was given at a dosage of 1.5-3.0 g/d in the induction period (two to four months) and 0.75-2.00 g/d in the maintenance period.All the patients were followed up regularly.Modified Rankin Scale (mRS) score evaluation,serum IgG and peripheral CD19-positive B cells,CD4-positive T cells and CD8-positive T cells testing were performed every two months.Results Fifteen patients were included in this study who received first-line immunotherapy combined with MMF.No other second-line therapy including rituximab was used.Thirteen patients responded well to MMF combined with first-line immunotherapy (a decrease in mRS score of more than 1).All 15 patients had a good outcome (i.e.,a mRS score of 0-2),including nine patients without residual symptoms (a mRS score of 0).After 12 months of MMF treatment,CD19-positive B cells were significantly decreased (median 320 (227,628) × 106/L vs 152 (105,223) × 106/L;Z=-2.028,P=0.043),while serum IgG (9.07 (6.70,11.32) g/L vs 8.35 (6.63,10.69) g/L,P=0.144)),CD4-positive T cells (1 136 (736,1 432) × 106/L vs 1 055 (802,1 072) × 106/L,P =0.866) and CD8-positive T cells (627 (413,784) × 106/L vs 568 (393,743) × 106/L,P =0.735) were not significantly changed.Three patients relapsed and were treated with additional cycle of first-line immunotherapy and increased dosage of MMF (induction dosage) resulting in remission.CD19-positive B cells were tested to be increased during the patients' relapse.No serious adverse event was noted in all these patients.Conclusions MMF is safe and effective as a long-term immunotherapy in patients with anti-LGI1 encephalitis.MMF can be used as an add-on therapy to first-line immunotherapy for autoimmune encephalitis.CD19-positive B cell count should be monitored and used as a parameter to individualize dosage of MMF.
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No abstract available.
Subject(s)
Glioma , Limbic Encephalitis , Potassium Channels, Voltage-Gated , SeizuresABSTRACT
The autoimmune encephalopathies are a group of conditions that are associated with autoantibodies against surface neuronal proteins, which are likely to mediate the disease. They are established as a frequent cause of encephalitis. Characteristic clinical features in individual patients often allow the specificity of the underlying antibody to be confidently predicted. Antibodies against the VGKC-complex, mainly LGI1(leucine-rich glioma-inactivated 1), CASPR2 (contactin-associated protein 2), and contactin-2, and NMDA (N-methyl, D-aspartate) -receptor are the most frequently established serological associations. In the minority of cases, an underlying tumour can be responsible. Early administration of immunotherapies, and tumour removal, where it is relevant, offer the greatest chance of improvement. Prolonged courses of immunotherapies may be required, and clinical improvements often correlate well with the antibody levels. In the present article, we have summarised recent developments in the clinical and laboratory findings within this rapidly expanding field.
As encefalopatias autoimunes constituem um grupo de condições associadas à presença, no soro, de anticorpos contra proteínas de superfície neuronais. Acredita-se que esses anticorpos sejam mediadores da ocorrência da doença, sendo reconhecidos atualmente como causas frequentes de encefalite. Apresentações clínicas características permitem, muitas vezes, predizer o grupo específico de anticorpos subjacentes. Anticorpos contra o complexo VGKF, especialmente LGI1 (leucine-rich glioma-inactivated1), CASPR2 (contactin-associated protein 2) e contactina-2, e contra o receptor NMDA(N-methyl, D-aspartate) são as associações sorológicas mais frequentemente estabelecidas. Na minoria dos casos, pode ser detectado um tumor subjacente. As maiores chances de melhora estão relacionadas à administração precoce de imunoterapia e à remoção do tumor, quando presente. A duração da imunoterapia pode se prolongada e a melhora se correlaciona, muitas vezes, com os níveis séricos de anticorpos. Neste artigo, estão resumidos os avanços recentes nos achados clínicos e laboratoriais neste campo que está em tão rápida expansão.