Autoimmune encephalitis (AIE)

Cafalli, Claudia; Amorim, Eliane; Silva, Flavio; Alves, J.M.; Anhesini, Mauricio; Bernardo, Wanderley Marques

doi:10.1590/1806-9282.66.10.1327

Cited by 5 publications

(7 citation statements)

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“…The gender distribution and the proportion of AE subtypes tended to be consistent with those of previous reports (1,15). In this study, the incidence of disease onset peaked in patients aged 11-20 and 61-70 years, a finding that differed from prior reports, which showed that peak incidence of disease onset occurred in young and middle aged patients (3,14). The difference observed may be due to regional differences, including differing occurrence rates of AE subtypes in patient populations considered.…”

Section: Discussionsupporting

confidence: 90%

“…AE is thought to be associated with antibodies to neuronal cell-surface proteins such as N-methyl-D-aspartate receptor (NMDAR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (CASPR2), gammaaminobutyric-acid B receptor (GABABR), etc. Some differences have been observed with regard to the clinical manifestations and prognosis of patients with different subtypes of AE (2,3). Most AE patients respond well to immunotherapy.…”

Section: Introductionmentioning

confidence: 99%

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Characteristics and Prognosis of Autoimmune Encephalitis in the East of China: A Multi-Center Study

Qiao

Liu

et al. 2021

Front. Neurol.

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Objective: This study aimed to investigate epidemiological characteristics, clinical manifestations, and long-term outcomes of patients with autoimmune encephalitis (AE) in the east of China.Methods: From January 2015 to December 2019, 226 potential AE patients were recruited from five clinical centers, and a total of 185 patients who met the diagnostic criteria were included in the study. We retrospectively reviewed clinical features, auxiliary examinations, details of treatments, and outcomes of AE, and identified risk factors of poor prognosis. Modified Rankin Scale scores were used to evaluate neurological function, and scores of 3–6 indicated a poor-prognosis.Results: Patients with five main subtypes of AE were enrolled in the study, as follows: anti-NMDAR (79), anti-LGI1 (55), anti-CASPR2 (30), anti-GABABR (16), and anti-AMPAR (5). Among 185 patients, 58.38% (108/185) were male and 41.62% (77/185) were female. The median age at disease onset was 41 years (interquartile range, 17–62). The most common clinical manifestations of AE were seizures (146, 78.92%) and memory deficit (123, 66.49%). A total of 95 (51.35%) patients had abnormal brain magnetic resonance imaging results. Electroencephalographic findings were abnormal in 131 (70.81%) patients, and 168 (90.81%) and 26 (14.05%) patients were treated with first- and second-line immunotherapies, respectively. All surviving patients were followed-up for at least 1 year (range 12–36 months). Good clinical outcomes were achieved in 117 (63.24%), while 68 (36.76%) patients had a poor prognosis. Further, 33 (17.84%) patients relapsed and 10 (5.41%) died within 1 year post-discharge. Older patients tended to have a poorer prognosis, and the occurrence of mental behavioral disorders, movement disorders, disturbance of consciousness, central hypoventilation, and tumors were overrepresented in the poor-prognosis group.Conclusions: AE is a treatable disease, and most patients have a good prognosis. There are differences in the clinical manifestations of patients with different AE subtypes. Some with AE will relapse, and long-term follow-up is of great significance for further research.

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Section: Discussionsupporting

confidence: 90%

Section: Introductionmentioning

confidence: 99%

Characteristics and Prognosis of Autoimmune Encephalitis in the East of China: A Multi-Center Study

Qiao

Liu

et al. 2021

Front. Neurol.

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“…The included cases comprised 107 patients with anti-NMDAR encephalitis, 86 with anti-LGI1 encephalitis, 22 with anti-GABABR encephalitis, and 22 with miscellaneous subtypes of AE; these being anti-CASPR2 encephalitis (9 cases), anti-AMPAR encephalitis ( 4 ), anti-MOG encephalitis ( 6 ), anti-GAD 65 encephalitis ( 2 ), and anti-amphiphysin encephalitis ( 1 ). Patient recruitment is described in the flowchart in Figure 1 .…”

Section: Resultsmentioning

confidence: 99%

“…Autoimmune encephalitis (AE) is a severe inflammatory brain disorder mediated by autoimmune mechanisms and accounts for 10% to 20% of encephalitis cases ( 1 ). The main clinical features of AE are acute or subacute epilepsy, cognitive dysfunction, and mental abnormalities.…”

Section: Introductionmentioning

confidence: 99%

Thyroid Function and Low Free Triiodothyronine in Chinese Patients With Autoimmune Encephalitis

Qiao

Zhang

et al. 2022

Front. Immunol.

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Background and ObjectivesLow free triiodothyronine (FT3) is usually associated with worse functional outcome in critical illness; however, the information on thyroid dysfunction and autoimmune encephalitis (AE) is limited. This study aims to evaluate the clinical prognostic value of thyroid function and low-T3 syndrome in patients with multiple subtypes of AE. MethodsIn this retrospective study, we identified the hospital records of 319 candidate patients with AE admitted between January 2016 and December 2020. We then extracted the clinical features and outcomes. Modified Rankin scale (mRS) scores were used to evaluate the patients’ neurological function. The serum levels of FT3, free thyroxine (FT4), and thyroid-stimulating hormone (TSH) were measured upon admission. Normal thyroid stimulating hormone level with FT3 below the lower limit of the reference interval (2.63 nmol/L) was defined as low-T3 syndrome.ResultsA total of 237 AE cases remained after screening. Among these, 57.81% (137/237) were men and the average age at onset was 41 y (interquartile range, 12–61 y). We found that 83.54% (198/237) of the patients had a good prognosis, and 16.46% (39/237) had a poor prognosis. Abnormal thyroid function was observed in 30.80% of these patients, with a relatively greater prevalence in the group with a poor prognosis (p < 0.001). The serum FT3 levels in the poor-prognosis group were significantly lower than those in the good-prognosis group (p < 0.001). Low-T3 syndrome occurred in 15.19% of AE cases and was more frequent in patients with poor prognosis (p < 0.001).ConclusionsAbnormal thyroid function in AE is frequent, and serum FT3 levels in patients with poor prognosis are significantly lower than in those with good prognosis. Low-T3 syndrome could be a potential candidate for predicting the prognosis of AE following future research.

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“…Autoimmune encephalitis (AE) is an uncommon neurological disorder mediated by autoimmune mechanisms and is an important cause of rapidly progressive cognitive dysfunction, refractory epilepsy, and psychiatric abnormalities (1,2). While most patients have been shown to respond well to immunotherapy, some patients continue to have intractable seizures and varying degrees of cognitive impairment, which have a serious effect on their quality of life (3,4).…”

Section: Introductionmentioning

confidence: 99%

Coexistence of multiple anti-neuronal antibodies in autoimmune encephalitis in China: A multi-center study

et al. 2022

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BackgroundGiven that the combination of multiple antibodies in autoimmune encephalitis (AE) is rare and its clinical significance is unclear, this study aimed to investigate the clinical characteristics and significance of overlapping multiple anti-neuronal antibodies in patients with AE.MethodsWe conducted a retrospective analysis of the clinical characteristics, treatment, and prognostic details of 22 patients with multiple coexisting antibodies from multiple clinical centers in China.ResultsAmong the 276 patients who were AE antibody-positive, 22 (7.97%) had two or more antibodies. Among the 22 patients with coexisting AE-related antibodies, 14 patients (63.63%) were combined of cell surface and intracellular antibody, and the remaining 8 patients (36.36%) were detected to be cell surface antibody positive only. The main symptoms of the 22 patients in this cohort included fever, seizures, memory impairment, cognitive decline, and sleep disorders. Five (22.73%) patients had tumors, among whom four had small-cell lung cancers, and one had mediastinal tumors. A total of 20 patients were treated with steroids and intravenous immunoglobulin, and 18 showed varying degrees of symptomatic improvement after first-line immunotherapy. Three patients died of tumor progression or chemotherapy complications.ConclusionThe coexistence of multiple anti-neuronal antibodies in patients with AE may cause a superimposition and diversification of clinical manifestations. Combined paraneoplastic antibody positivity may be suggestive of an underlying malignancy.

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Autoimmune encephalitis (AIE)

Cited by 5 publications

References 1 publication

Characteristics and Prognosis of Autoimmune Encephalitis in the East of China: A Multi-Center Study

Characteristics and Prognosis of Autoimmune Encephalitis in the East of China: A Multi-Center Study

Thyroid Function and Low Free Triiodothyronine in Chinese Patients With Autoimmune Encephalitis

Coexistence of multiple anti-neuronal antibodies in autoimmune encephalitis in China: A multi-center study

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