Seizure characteristics, treatment, and outcome in autoimmune synaptic encephalitis: A long-term study

Zhang, Wuqiong; Wang, Xue; Shao, Na; Ma, Rui; Meng, Hongmei

doi:10.1016/j.yebeh.2018.10.038

Cited by 31 publications

(62 citation statements)

References 34 publications

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“…Another study demonstrated that 37.2% patients with anti-NMDAR, anti-LGI1, and anti-GABA B R encephalitis developed persistent seizures to different extents and the remaining experienced seizure remission during the follow-up. 18 However, this study did not provide the definition of persistent seizures and seizure remission, probably leading to the overestimation of epilepsy risk. 18 In addition, Casciato et al's study had reported a much higher percentage of 40% developed chronic epilepsy in limbic encephalitis with anti-voltage-gated potassium channel (LGI-1 and CASPR2), anti-NMDAR, anti-SOX1, anti-Ri, anti-Hu and anti-glutamic acid decarboxylase antibodies.…”

Section: Discussionmentioning

confidence: 90%

“…18 However, this study did not provide the definition of persistent seizures and seizure remission, probably leading to the overestimation of epilepsy risk. 18 In addition, Casciato et al's study had reported a much higher percentage of 40% developed chronic epilepsy in limbic encephalitis with anti-voltage-gated potassium channel (LGI-1 and CASPR2), anti-NMDAR, anti-SOX1, anti-Ri, anti-Hu and anti-glutamic acid decarboxylase antibodies. 10 This could be explained by different samples.…”

Section: Discussionmentioning

confidence: 90%

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Seizures and risk of epilepsy in anti‐NMDAR, anti‐LGI1, and anti‐GABA_BR encephalitis

Shen

Fang

Yang

et al. 2020

Ann Clin Transl Neurol

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Background Accumulating data have suggested seizures occur frequently in patients with neuronal surface antibody‐mediated autoimmune encephalitis. We aimed to evaluate seizure outcomes and potential factors associated with the development of epilepsy in patients with anti‐N‐methyl‐D‐aspartate receptor (NMDAR), anti‐leucine‐rich glioma‐inactivated 1 (LGI1), and anti‐gamma‐aminobutyric‐acid B receptor (GABABR) encephalitis. Methods Patients with anti‐NMDAR, anti‐LGI1, and anti‐GABABR encephalitis were prospectively recruited from 2014 to June 2019, with a median follow‐up period of 30.5 months (range 8–67 months). Seizure outcomes were assessed and risk factors of epilepsy were analyzed. Results A total of 119 patients with anti‐NMDAR, anti‐LGI1, and anti‐GABABR encephalitis were included, and 83 (69.7%) of them developed new‐onset seizures. By the end of follow‐up, 17 (21.3%) of 80 patients had seizure relapses after intermittent seizure remission or exhibited uncontrolled seizure episodes, contributing to epilepsy. Immunotherapy delay and interictal epileptic discharges (IEDs) were identified to be associated with the development of epilepsy in patients with anti‐NMDAR, anti‐LGI1, and anti‐GABABR encephalitis, particularly anti‐NMDAR encephalitis. Furthermore, multivariate logistic regression analysis demonstrated that immunotherapy delay was an independent predictor for epilepsy. Conclusion Our study suggested that immunotherapy delay and IEDs were associated with the development of epilepsy in patients with anti‐NMDAR, anti‐LGI1, and anti‐GABABR encephalitis. Early diagnosis and treatment were required, and particular consideration should be given to patients with these risk factors.

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Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 90%

Seizures and risk of epilepsy in anti‐NMDAR, anti‐LGI1, and anti‐GABA_BR encephalitis

Shen

Fang

Yang

et al. 2020

Ann Clin Transl Neurol

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“…This is consistent with previous reports. 4,5,13,19 Interestingly, we observed that a large number of EEG slow waves or intermittent spikes were detected at onset in 12 (12/19) relapsed patients. This may suggest the importance of EEG monitoring for disease recurrence.…”

Section: Discussionmentioning

confidence: 71%

“…In the last few decades, the eld of autoimmune epilepsy has received increasing attention with the discovery of different subtypes of neural autoantibodies. 5,[16][17][18][19] In the latest 2017 epilepsy classi cation, autoimmune epilepsy has been recognized as a distinct entity by the International League Against Epilepsy. 20 Most patients with autoimmune epilepsy present with seizures, cognitive decline, and behavioral or psychiatric dysfunction.…”

Section: Discussionmentioning

confidence: 99%

Long-term Outcomes of Patients with Anti–Leucine-Rich Glioma-Inactivated 1 Encephalitis

Qiao¹,

Wu²,

Liu³

et al. 2020

Preprint

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Background: This report aims to provide a detailed description of the clinical manifestation, immunotherapy, and long-term outcomes of 117 Chinese patients with anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis. Methods: We retrospectively selected 117 patients diagnosed with anti-LGI1 encephalitis from the databases of multiple clinical centers from September 2014 to December 2019. The clinical features, ancillary test results, and details of long-term outcomes were analyzed.Results: Among the 117 anti-LGI1 encephalitis patients, 81 (69%) were male and 36 (31%) were female; the median onset age was 51 years (range: 30-77 years). The median time from symptom onset until diagnosis was 8.7 weeks (range: 2-49 weeks). The main features evaluated in our cohort were seizures, cognitive impairment, and mental and behavioral abnormalities. One hundred and nine patients were treated with immunotherapy, After 3-5 days of treatment, the clinical symptoms were somewhat alleviated in all the patients, and their memory, mental ability, and behavior improved. The median follow-up time was 33 months (range: 6-59 months). A total of 19 (16%) patients experienced a relapse; the median duration from onset to the first relapse was 5 (0.3-27) months. There were no mortalities during the follow-up period.Conclusions: The outcome of patients with anti-LGI1 encephalitis is mostly favorable, although some patients continue to suffer from cognitive dysfunction. Early recognition is of great significance for the treatment of anti-LGI1 encephalitis. Prompt adequate immunotherapy has positive implications for the improvement of clinical symptoms of anti-LGI1 encephalitis. Long-term follow-up is important for the assessment of LGI1 antibody-mediated encephalitis.

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“…However, in children younger than 12 years old, the proportion of psychiatric symptoms at initial symptoms was nearly equal to that of seizures, but more common than that of movement disorder [10,15,21]. It remained unclear why there existed age differences on the initial symptoms of anti-NMDAR encephalitis.…”

Section: Discussionmentioning

confidence: 99%

Pediatric Anti-NMDAR Encephalitis in Southern China: Analysis of 107 Cases

Hou

et al. 2020

Preprint

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Background: Research on pediatric anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is limited. We studied clinical features of children with anti-NMDAR encephalitis in southern China.Methods: Pediatric anti-NMDAR encephalitis between 2014 and 2019 from one tertiary medical center was analyzed. The Modified Rankin Scale (mRS) score was adopted to evaluate outcomes.Results: 107 children (M/F=50/57; mean onset age=6.3 y) with anti-NMDAR encephalitis were involved. Boys significantly showed earlier onset age, higher ratio of fever, longer hospital day, more courses of steroid treatment, higher mRS score after treatment. 48.6% of patients had prodromal events with infectious events most common. The most common symptom at onset and during whole course was seizures and psychiatric symptoms respectively. Initial median white cell count of cerebral spinal fluid (CSF) was 22.0x106/L. 12.1% of patients had positive herpes simplex virus DNA of CSF. All patients had CSF positive NMDAR antibodies, although 86.9% of patients had serologically positive. 9.3% of patients had overlapped other neuronal antibodies. Electroencephalograph showed abnormalities with slow wave (95.3%) and epileptic activity (41.5%). Delta brush was revealed in 3.8% of patients. 36.4% of patients had lesions in brain MRI, with local lesions most common. No tumor was found in all. 84.9% of patients responded well to the first line therapy (steroid plus immunoglobulin), while 12 patients accepted second-line therapy (Rituximab) with 91.7% of effective rate. 12.1% of patients relapsed. 2 male patients died. The median length of hospital stay was 28 days. The mRS score was significantly improved after treatment. 51.0% of patients had a full recovery. 76.6% of patients had mild neurological disability (mRS≤2). Male, speech disorder, initial score of mRS and administration of second-line therapy were independent risk factors associated with the outcome.Conclusions: Of pediatric anti-NMDAR encephalitis in southern China: the mean onset age was about 6-year-old, earlier in boys; boys remained slower to recover and with a worse prognosis; the most common respective symptom at onset and during whole course was seizures and psychiatric symptoms; combination with tumor was rare; most patients respond well to the immunotherapy; relapse and fatality rate were relatively low; most patients had a good prognosis.

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Seizure characteristics, treatment, and outcome in autoimmune synaptic encephalitis: A long-term study

Cited by 31 publications

References 34 publications

Seizures and risk of epilepsy in anti‐NMDAR, anti‐LGI1, and anti‐GABA_BR encephalitis

Seizures and risk of epilepsy in anti‐NMDAR, anti‐LGI1, and anti‐GABA_BR encephalitis

Long-term Outcomes of Patients with Anti–Leucine-Rich Glioma-Inactivated 1 Encephalitis

Pediatric Anti-NMDAR Encephalitis in Southern China: Analysis of 107 Cases

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Seizure characteristics, treatment, and outcome in autoimmune synaptic encephalitis: A long-term study

Cited by 31 publications

References 34 publications

Seizures and risk of epilepsy in anti‐NMDAR, anti‐LGI1, and anti‐GABABR encephalitis

Seizures and risk of epilepsy in anti‐NMDAR, anti‐LGI1, and anti‐GABABR encephalitis

Long-term Outcomes of Patients with Anti–Leucine-Rich Glioma-Inactivated 1 Encephalitis

Pediatric Anti-NMDAR Encephalitis in Southern China: Analysis of 107 Cases

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Seizures and risk of epilepsy in anti‐NMDAR, anti‐LGI1, and anti‐GABA_BR encephalitis

Seizures and risk of epilepsy in anti‐NMDAR, anti‐LGI1, and anti‐GABA_BR encephalitis