Clinical Characteristics and Prognosis of Severe Anti-N-methyl-d-aspartate Receptor Encephalitis Patients

Zhang, Yan; Liu, Gang; Jiang, Mengdi; Chen, Weibi; He, Yanbo; Su, Yingying

doi:10.1007/s12028-018-0536-6

Cited by 50 publications

(43 citation statements)

References 32 publications

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“…Previous studies have indicated that the parietal aEEG bandwidth may separate patients with favorable and poor long-term outcomes in the early disease stages (24). In our study, the first available EEG findings that obtained before immunotherapy showed generalized slowing in 14/18 patients (77.7%), focal slowing in 2/18 patients (11.1%), and no abnormalities in 2/18 patients (11.1%), consistent with the findings of previous reports (25, 26). No patients exhibited abnormal background activity at the final follow-up.…”

Section: Discussionsupporting

confidence: 92%

Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis

Zhang

Zhou

et al. 2019

Front. Neurol.

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Objective: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common form of autoimmune encephalitis in pediatric patients. In this study, we aimed to investigate the clinical features and long-term outcomes of pediatric patients with anti-NMDAR encephalitis in China. Methods: We conducted a retrospective study of children (age range: 0–18 years) with anti-NMDAR encephalitis treated at Children's Hospital of Fudan University between July 2015 and November 2018. Demographic characteristics, clinical features, treatment, and outcomes were reviewed. Results: Thirty-four patients with anti-NMDAR encephalitis were enrolled (age range: 5 months to 14 years; median age: 7 years; female: 18). The median follow- up duration was 20 months (range: 6–39 months). Eighteen (52.9%) patients initially presented with seizures and 10 (29.4%) with abnormal (psychiatric) behaviors or cognitive dysfunction. Thirty (88.2%) patients exhibited more than two symptoms during the disease course. No neoplasms were detected. Twelve (35.2%) patients had abnormal cerebrospinal fluid (CSF) findings, including leukocytosis, and increased protein concentration. Eighteen (52.9%) patients exhibited normal brain MRI findings. Electroencephalography revealed abnormal background activity in 27 (79.4%) patients, and epileptiform discharges in 16 (47.0%) patients prior to immunotherapy. All patients received first-line immunotherapy, with 30 (88.2%) and four (11.8%) patients achieving good (Modified Rankin Scale [mRS] score of 0–2) and poor outcomes (mRS score of 3–5), respectively. Initial mRS scores differed significantly between the good and poor outcome groups. Fourteen out of 18 patients (77.7%) with seizures accepted anti-epileptic drug (AED) administration, and seizure freedom was achieved in 12 out of 14 (85.7%) patients at the last follow-up. Ten of these 12 (83.3%) patients withdrew from AED treatment within 1 year. Conclusions: Most patients achieved seizure freedom, so long-term use of AEDs may not be necessary for pediatric patients with anti-NMDAR encephalitis. Among our patients, 83.3% were sensitive to first-line immunotherapy and achieved good outcomes. Higher mRS scores before immunotherapy predicted poor outcomes, highlighting the need for a comprehensive assessment of patients with anti-NMDAR encephalitis.

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Section: Discussionsupporting

confidence: 92%

Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis

Zhang

Zhou

et al. 2019

Front. Neurol.

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“…Patients with anti-NMDAR encephalitis in our study demonstrated a lower incidence in women, with tumors, central hypoventilation, and a better prognosis, and this is consistent with the results of previous studies in China. 16 – 18 There was no sex difference, but a tendency to women was found. The prevalence of tumors was also lower in the Korean population than in the Western study populations.…”

Section: Discussionmentioning

confidence: 85%

Validation of the NEOS score in Chinese patients with anti-NMDAR encephalitis

Peng

Dai

Liu

et al. 2020

Neurol Neuroimmunol Neuroinflamm

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ObjectiveThe performance of anti-NMDAR Encephalitis One-Year Functional Status (NEOS) in predicting the 1-year functional status in Chinese patients with anti-NMDAR encephalitis is unknown.MethodsWe recruited patients with anti-NMDAR encephalitis from the Multicenter and Prospective Clinical Registry Study of Anti-NMDAR Encephalitis in Beijing Area. Patients were followed up for 1 year. We defined the poor functional status as a modified Rankin Scale score of more than 2 and good functional status as a modified Rankin Scale score of no more than 2. We performed a receiver-operator characteristic analysis to assess the discriminatory power of the NEOS score in predicting the 1-year functional status by using the area under the curve (AUC). Calibration was assessed by Pearson correlation coefficient and Hosmer-Lemeshow tests.ResultsAmong the 111 patients with anti-NMDAR encephalitis recruited from 364 potentially eligible participants, 87 (78.4%) had good functional status at 1 year, whereas the remaining 24 (21.6%) had poor functional status. The AUC of the NEOS score for 1-year poor functional status was 0.86 (95% CI 0.78–0.93, p < 0.001). The increased NEOS was associated with higher risk of 1-year poor functional status in patients with anti-NMDAR encephalitis.ConclusionsThe NEOS score is considered a reliable predictor of the risk of 1-year poor functional status in Chinese patients with anti-NMDAR encephalitis. This score could help to estimate the velocity of clinical improvement in advance.Clinicaltrial.gov identifierNCT02443350.Classification of evidenceThis study provides Class III evidence that in patients with anti-NMDAR encephalitis, the NEOS score predicts 1-year functional status.

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“…Although AAG is an autoimmune disorder that causes autonomic dysfunction responsive to IVIg, it was considered unlikely because it causes postganglionic autonomic failure, and nearly half of AAG cases are positive for anti-ganglionic ACh receptor antibody (6). In some types of encephalitis, such as anti-NMDAR encephalitis and anti-GAD encephalitis, autonomic dysfunction can be induced by the impairment of the subthalamus or brainstem (7)(8)(9). However, these antibodies were negative.…”

Section: Discussionmentioning

confidence: 99%

Anti-LGI1 Encephalitis Developing Immunoglobulin Responsive Orthostatic Hypotension after Remission

et al. 2021

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Anti-leucine-rich glioma-inactivated 1 (LGI1) antibody is associated with limbic encephalitis. We herein report a patient with anti-LGI1 encephalitis who developed severe orthostatic hypotension (OH) responsive to immunoglobulin therapy five years after developing symptoms of encephalitis. A 71-year-old man presented with amnesia caused by limbic encephalitis. The symptoms of encephalitis improved partially without any immunotherapy. Five years later, he developed severe OH, and anti-LGI1 antibody was positive. The catecholamine dynamics indicated that the central autonomic nervous system was the lesion of his OH. Intravenous immunoglobulin therapy improved the OH. This case suggests that anti-LGI1 antibody can be associated with severe OH.

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Clinical Characteristics and Prognosis of Severe Anti-N-methyl-d-aspartate Receptor Encephalitis Patients

Abstract: Most severe anti-NMDAR encephalitis patients will eventually achieve good long-term prognoses after receiving early, positive and unremitting combined immunotherapy and life support.

Cited by 50 publications

References 32 publications

Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis

Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis

Validation of the NEOS score in Chinese patients with anti-NMDAR encephalitis

Anti-LGI1 Encephalitis Developing Immunoglobulin Responsive Orthostatic Hypotension after Remission

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