The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis

Ramanathan, Sudarshini; Al-Diwani, Adam; Waters, Patrick; Irani, Sarosh R.

doi:10.1007/s00415-019-09590-9

Cited by 55 publications

(69 citation statements)

References 131 publications

(207 reference statements)

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“…It has been reported in laboratory tests [10] that serum hyponatremia accounts for about 50%; patients with hyponatremia in this article account for 73.17%, with a minimum of 115.4 mmol/L, and hyponatremia is higher than previous reports. Report [11] shows that serum LGI1 antibody is easier to detect than CSF LGI1 antibody.…”

Section: Discussioncontrasting

confidence: 54%

Clinical Characteristics and Prognostic Analysis of Anti-LGI 1 Receptor Encephalitis in Northeast China

Zhao

et al. 2020

Preprint

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Abstract Background: To investigate the clinical characteristics and prognosis of Anti-LGI1 receptor encephalitis.Methods: This retrospective study enrolled forty -one patients with anti LGI1 receptor encephalitis diagnosed for the first time in the Department of Neurology, the first hospital of Jilin University from June 2015 to November 2019. Results: There are twenty-two males and nineteen females, with a median age of fifty-four years. The first symptom of male patients showed cognitive impairment in nine (40.91%) cases,facial muscle dystonia in six (27.27%) cases.Of the female patients,seven (36.84%)patients had facial-arm dystonia-like seizures,six (31.58%) patients had cognitive impairment.seven (18.42%) patients had abnormal tumor markers,but no tumor was found in all patients.Thirty patients (73.17%) had hyponatremia with a minimum of 115.4 mmol/L.Except for one patient,eighteen (45.00%) patients had normal cerebrospinal fluid indicators,twenty-two (55.00%) patients had abnormal cerebrospinal fluid indicators,Eleventh (50.00%) patients had elevated protein,and nine (40.91%) patients had elevated IgG,six (27.27%) patientys of white blood cells increased,and four (18.18%) patients of increased pressure. Except for four patients,twenty-two (59.46%) patients had abnormal signals of hippocampus and temporal lobe,seventeen (77.27%) of them had bilateral abnormal signals of hippocampus,five(22.73%)cases had unilateral abnormal signals of hippocampus;Eleventh(29.73%)had lacunar Cerebral infarction and ischemic focus;Two(5.41%)cases of frontal and insular abnormal signals;one(2.70%)case of temporal and occipital abnormal signals;one(2.70%) case of white matter lesions. Except for one patient,the EEG showed twenty-seven(67.50%)cases of slow waves and focal discharges,(six(22.22%) of which were left-temporal spiked slow waves,and five (18.52%) were of right-temporal spikes Slow wave,four (14.81%) cases with double temporal sharpened slow wave,six (22.22%) cases with frontotemporal sharpened slow wave, one (3.70%) case with frontal sharpened slow wave,one(3.70%)case with Three-phase waves; In addition,four(10.00%)cases of fast activity increased;three (7.50%) cases of background α generalization). Patients received first-line immunotherapy except six patients who gave up treatment. Conclusions: Anti-LGI1 receptor encephalitis is more common in middle-aged and older people. In male,the first manifestation is cognitive impairment, and the female is more common with the facial-arm dystonia-like seizures,and rarely can be manifested as discharge sensation, hallucinations and autonomic symptoms.lumbar puncture pressure increased more than decreased;protein was significantly higher than leukocytes;EEG showed slow waves and focal discharges were more common, and abnormal background and normal EEG can occur.MRI shows that hippocampus and temporal lobe abnormal signal have the highest proportion.There is no significant difference in the prognosis of hormones, globulin,or both.

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Section: Discussioncontrasting

confidence: 54%

Clinical Characteristics and Prognostic Analysis of Anti-LGI 1 Receptor Encephalitis in Northeast China

Zhao

et al. 2020

Preprint

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“…AE remains an under-recognized clinical syndrome but one where early and accurate detection is critical as prompt initiation of immunotherapy is closely associated with improved outcomes [12]. The treatment strategies and prognosis may be more complicated in patients with overlapping symptoms, and thus timely recognition of the potential concurrence of different antibodies is of great significance [4].…”

Section: Discussionmentioning

confidence: 99%

“…LGI1 antibodies define the most prevalently recognized autoimmune encephalitis (AE) syndromes, while the simultaneous occurrence of both conditions has hardly been published before [4]. We herein describe the case of a 67-year-old man with LGI1-LE presenting overlapping symptoms and simultaneous positive NMDAR antibodies.…”

Section: Introductionmentioning

confidence: 92%

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

Huang

Lian

et al. 2020

BMC Neurol

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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.

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“…With the reported incidence of 0.83/million/year (in Netherlands in 2015), anti-LGI1 encephalitis most frequently occurs in the elderly with a male predominance. Its main clinical features include faciobrachial dystonic seizures (FBDS), serum hyponatremia and other symptoms of ALE (4,5). Studies have established that around 90% of anti-LGI1 patients carry the human leukocyte antigen (HLA) DRB * 07:01 and DQA1 * 02:01 (6).…”

Section: Introductionmentioning

confidence: 99%

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

Wang

et al. 2020

Front. Neurol.

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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.

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The autoantibody-mediated encephalitides: from clinical observations to molecular pathogenesis

Cited by 55 publications

References 131 publications

Clinical Characteristics and Prognostic Analysis of Anti-LGI 1 Receptor Encephalitis in Northeast China

Clinical Characteristics and Prognostic Analysis of Anti-LGI 1 Receptor Encephalitis in Northeast China

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

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

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