Diagnosing autoimmune encephalitis in a real-world single-centre setting

Giordano, Antonino; Fazio, Raffaella; Gelibter, Stefano; Minicucci, Fabio; Vabanesi, Marco; Anzalone, Nicoletta; Magnani, Giuseppe; Filippi, Massimo; Martinelli, Vittorio

doi:10.1007/s00415-019-09607-3

Cited by 33 publications

(18 citation statements)

References 12 publications

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“…In recent years, the incidence of AE has been increasing. However, the etiology and pathogenesis of AE remains unclear (12)(13)(14). Given that AE is a serious but treatable disease, the ability to perform a timely and accurate assessment of disease prognosis is important for providing appropriate therapy and improving clinical outcomes of AE.…”

Section: Discussionmentioning

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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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: Discussionmentioning

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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“…Research on autoimmune diseases has broadened the spectrum of AE subtypes. [1][2][3] Some subtypes of AE have complex clinical manifestations that make diagnosis difficult. Leucine-rich glioma inactivated 1 (LGI1) is associated with voltage-gated potassium channels (VGKCs).…”

Section: Introductionmentioning

confidence: 99%

Clinical Features and Long-Term Outcomes of Anti-Leucine-Rich Glioma-Inactivated 1 Encephalitis: A Multi-Center Study

Qiao

Liu

et al. 2021

NDT

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Purpose: To describe the clinical manifestation, immunotherapy, and long-term outcomes of anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis. Patients and Methods: This study was a retrospective analysis of 117 patients with a diagnosis of anti-LGI1 encephalitis identified from the databases of multiple clinical centers between September 2014 and December 2019. The clinical features, ancillary test results, and details of long-term outcomes were evaluated. Results: Among the 117 patients with anti-LGI1 encephalitis, 69.2% (81/117) were male and 30.8% (36/117) were female. The median age of all patients at the onset of the disease was 57 years (interquartile range [IQR], 52-67). The median time from symptom onset to diagnosis was 8.7 weeks (IQR, 4.2-25). The main clinical features identified were seizures, cognitive impairment, and mental and behavioral abnormalities. Of the 117 patients, 109 were treated with immunotherapy. Symptoms including memory, mental ability, and behavior improved in all 109 patients after 3-5 days of treatment. The median time of follow-up for the treated patients was 33 months (IQR,. Of the treated patients, 16.2% (19/117) experienced a relapse, with a median delay of 5 months (IQR, 2.1-17) between onset and the first relapse. There were no mortalities over the follow-up period. Conclusion:The long-term outcome of patients with anti-LGI1 encephalitis was mostly favorable, although some patients continued to experience cognitive dysfunction. Early recognition is important for prompt initiation of immunotherapy that can improve clinical symptoms of anti-LGI1 encephalitis.

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“…Patients may develop a rapidly progressive encephalopathy syndrome, manifested by changes in mental state and a number of neurological symptoms including impaired memory and cognition, vision and speech problems, impaired coordination, movement disorders and seizures, and psychiatric symptoms including psychosis, euphoria, aggressiveness and compulsiveness, inappropriate sexual behavior, and panic attacks. Symptoms may progress to loss of consciousness or even coma 1,2 …”

Section: Introductionmentioning

confidence: 99%

Efficacy and safety of rituximab in autoimmune encephalitis: A meta‐analysis

et al. 2020

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Autoimmune encephalitis (AE) is a rare but debilitating neurological disease in which the body develops antibodies against its own neurons and causes inflammation of the brain. It affects all age groups but children and women are more commonly affected. Patients may develop a rapidly progressive encephalopathy syndrome, manifested by changes in mental state and a number of neurological symptoms including impaired memory and cognition, vision and speech problems, impaired coordination, movement disorders and seizures, and psychiatric symptoms including psychosis, euphoria, aggressiveness and compulsiveness, inappropriate sexual behavior, and panic attacks. Symptoms may progress to loss of consciousness or even coma. 1,2

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Diagnosing autoimmune encephalitis in a real-world single-centre setting

Cited by 33 publications

References 12 publications

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

Clinical Features and Long-Term Outcomes of Anti-Leucine-Rich Glioma-Inactivated 1 Encephalitis: A Multi-Center Study

Efficacy and safety of rituximab in autoimmune encephalitis: A meta‐analysis

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