Dataset Information

Clinical Features and Gut Microbial Alterations in Anti-leucine-rich Glioma-Inactivated 1 Encephalitis-A Pilot Study.

ABSTRACT: Anti-leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis is a rare autoimmune encephalitis (AE). We investigated the clinical features and gut microbial alterations of anti-LGI1 encephalitis. Fifteen patients newly diagnosed with anti-LGI1 encephalitis were recruited in the study prior to the administration of immunotherapy. The control group contains 25 well-matched healthy controls (HCs). All participants were Han Chinese from South China. Their clinical data and fecal samples were collected. The diversity and composition of gut microbiota were analyzed by 16S ribosomal RNA (16S rRNA) gene sequencing. The results showed that anti-LGI1 encephalitis was characterized by cognitive impairment, faciobrachial dystonic seizures, hyponatremia, and psychiatric symptoms. Abnormal EEG and brain MRI were presented in 9 and 10 patients, respectively. Compared to HCs, the anti-LGI1 encephalitis patients exhibited a decreased microbial diversity and an altered overall composition of gut microbiome. At the phylum level, anti-LGI1 encephalitis patients exhibited a higher abundance of Proteobacteria and a lower abundance of Firmicutes. The alterations in the phylum level were associated with autoimmune and inflammatory disorders. At the genus level, there was an increase in Sphingomonas, Anaerofustis, Succinvibrio, Clostridium, and SMB53 (genera related to movement disorders, psychiatric diseases, and with proinflammatory effects). However, the Faecalibacterium, Roseburia, Lachnospira, Ruminococcus, and Blautia [genera with ability to produce short-chain fatty acids (SCFAs)] were obviously reduced in the patient group. Our results suggest that anti-LGI1 encephalitis is characterized by special clinical features and is accompanied by alterations in specific gut microbiota. For the limited sample size and non-applicability to other populations, further studies are warranted to explore the relationships between gut microbiota and anti-LGI1 encephalitis.

SUBMITTER: Ma X

PROVIDER: S-EPMC7655127 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Clinical Features and Gut Microbial Alterations in Anti-leucine-rich Glioma-Inactivated 1 Encephalitis-A Pilot Study.

Ma Xueying X Ma Lili L Wang Zhanhang Z Liu Yingying Y Long Ling L Ma Xiaomeng X Chen Hao H Chen Zhaoyu Z Lin Xiuli X Si Lei L Chen Xiaohong X

Frontiers in neurology 20201027

Anti-leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis is a rare autoimmune encephalitis (AE). We investigated the clinical features and gut microbial alterations of anti-LGI1 encephalitis. Fifteen patients newly diagnosed with anti-LGI1 encephalitis were recruited in the study prior to the administration of immunotherapy. The control group contains 25 well-matched healthy controls (HCs). All participants were Han Chinese from South China. Their clinical data and fecal samples were coll ...[more]

PMID: 33193049

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Project description:Background: Clinical presentations and treatment programs about anti-leucine-rich glioma inactivated 1 (LGI1) encephalitis still remain incompletely understood. Objective: This study analyzed the clinical features and therapeutic effects of anti-LGI1 encephalitis. Methods: PubMed, EMBASE, and the Cochrane Library were searched to identify published English and Chinese articles until April 2021. Data were extracted, analyzed, and recorded in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Results: A total of 80 publications detailing 485 subjects matched our inclusion criteria. Short-term memory loss (75.22%), faciobrachial dystonic seizures (FBDS) (52.53%), other seizures excluding FBDS (68.48%), psychiatric symptoms (57.67%), and sleep disturbances (34.30%) were the most frequently described symptoms in anti-LGI1 encephalitis. Hyponatremia (54.90%) was the most common hematologic examination change. The risk of incidence rate of malignant tumors was higher than in healthy people. The positive rate of anti-LGI1 in serum (99.79%) was higher than CSF (77.38%). Steroids (93.02%), IVIG (87.50%), and combined use (96.67%) all had a high remission rate in the initial visit. A total of 35 of 215 cases relapsed, of which 6/35 (17.14%) did not use first-line treatment, and 21 (60.00%) did not maintain long-term treatment. Plasma exchange (PE) could be combined in severe patients, immunosuppressant could be used for refractory patients or for recurrence and using an anti-epileptic drug to control seizures may benefit cognition. Conclusions: Short-term memory loss, FBDS, psychiatric symptoms, and hyponatremia were key features in identifying anti-LGI1 encephalitis. Serum and CSF antibody tests should be considered in diagnosis criteria. Steroids with IVIG should be recommended, PE was combined for use in severe patients, immunosuppressant therapy might improve outcomes if recurrence or progression occurred, and control seizures might benefit cognition. The useful ways to reduce relapse rate were early identification, clear diagnosis, rapid treatment, and maintaining long-term treatment. The follow-up advice was suggested according to the research of paraneoplastic syndrome, and concern about tumors was vital as well.

Project description:PurposeBrain 18F-fluorodeoxyglucose positron emission tomography (FDG PET) is a sensitive technique for assisting in the diagnosis of patients with anti-leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis. However, the common pattern of this disorder assessed by FDG PET remains unknown. The present study aimed to explore the glucose metabolic patterns of this disorder based on PET voxel analysis.MethodsThis retrospective study enrolled 25 patients with anti-LGI1 encephalitis, who were admitted in Beijing Tiantan Hospital between September 2014 and July 2019. The glucose metabolic pattern was compared between the included patients and 44 age- and gender-matched healthy controls using Statistical Parametric Mapping. Then, the correlation between the metabolic pattern and scaled activities of daily living (ADLs) of the patients was assessed.ResultsThe median time from symptom onset to PET scans was 9 w (range:2-53w). The groupwise analysis revealed that patients with anti-LGI1 encephalitis had left hippocampal hypermetabolism and hypometabolism in almost all neocortical regions. The individual-level results showed most patients presented a decreased metabolism in neocortical regions, as well as an increase in metabolism in the hippocampus and basal ganglia. Furthermore, the metabolic gradient between hippocampus and neocortical regions was positively associated with the ADLs (frontal lobe, r=0.529, P=0.008; parietal lobe, r=0.474, P=0.019; occipital lobe, r=0.413, P=0.045; temporal lobe, r=0.490, P=0.015), respectively. In addition, the patients with facio-brachial dystonic seizures (FBDS) presented bilateral putamen hypermetabolism, when compared to patients without FBDS and healthy controls.ConclusionSubcortical hypermetabolism associated with cortical hypometabolism presented with a common metabolic pattern in patients with anti-LGI1 encephalitis in the present study. The resolution of the metabolic gradient of the hippocampal hypermetabolism and neocortical hypometabolism may bring about improved clinical neurologic disability.

Project description:Antibodies that immunoprecipitate (125)I-alpha-dendrotoxin-labelled voltage-gated potassium channels extracted from mammalian brain tissue have been identified in patients with neuromyotonia, Morvan's syndrome, limbic encephalitis and a few cases of adult-onset epilepsy. These conditions often improve following immunomodulatory therapies. However, the proportions of the different syndromes, the numbers with associated tumours and the relationships with potassium channel subunit antibody specificities have been unclear. We documented the clinical phenotype and tumour associations in 96 potassium channel antibody positive patients (titres >400 pM). Five had thymomas and one had an endometrial adenocarcinoma. To define the antibody specificities, we looked for binding of serum antibodies and their effects on potassium channel currents using human embryonic kidney cells expressing the potassium channel subunits. Surprisingly, only three of the patients had antibodies directed against the potassium channel subunits. By contrast, we found antibodies to three proteins that are complexed with (125)I-alpha-dendrotoxin-labelled potassium channels in brain extracts: (i) contactin-associated protein-2 that is localized at the juxtaparanodes in myelinated axons; (ii) leucine-rich, glioma inactivated 1 protein that is most strongly expressed in the hippocampus; and (iii) Tag-1/contactin-2 that associates with contactin-associated protein-2. Antibodies to Kv1 subunits were found in three sera, to contactin-associated protein-2 in 19 sera, to leucine-rich, glioma inactivated 1 protein in 55 sera and to contactin-2 in five sera, four of which were also positive for the other antibodies. The remaining 18 sera were negative for potassium channel subunits and associated proteins by the methods employed. Of the 19 patients with contactin-associated protein-antibody-2, 10 had neuromyotonia or Morvan's syndrome, compared with only 3 of the 55 leucine-rich, glioma inactivated 1 protein-antibody positive patients (P < 0.0001), who predominantly had limbic encephalitis. The responses to immunomodulatory therapies, defined by changes in modified Rankin scores, were good except in the patients with tumours, who all had contactin-associated-2 protein antibodies. This study confirms that the majority of patients with high potassium channel antibodies have limbic encephalitis without tumours. The identification of leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 as the major targets of potassium channel antibodies, and their associations with different clinical features, begins to explain the diversity of these syndromes; furthermore, detection of contactin-associated protein-2 antibodies should help identify the risk of an underlying tumour and a poor prognosis in future patients.

Dataset Information

Clinical Features and Gut Microbial Alterations in Anti-leucine-rich Glioma-Inactivated 1 Encephalitis-A Pilot Study.

Publications

Clinical Features and Gut Microbial Alterations in Anti-leucine-rich Glioma-Inactivated 1 Encephalitis-A Pilot Study.

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