Heterogenous treatment for anti-NMDAR encephalitis in children leads to different outcomes 6–12 months after diagnosis

Sakpichaisakul, Kullasate; Patibat, Lucksanai; Wechapinan, Thanin; Sri-udomkajrorn, Somjit; Apiwattanakul, Metha; Suwannachote, Sirorat

doi:10.1016/j.jneuroim.2018.09.007

Cited by 11 publications

(6 citation statements)

References 25 publications

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“…These results are in agreement with a recent meta-analysis that factors associated with good functional outcome include corticosteroids with IVIg [22]. One previous review from Asia with a small number of pediatric NMDAR encephalitis patients (n = 19) showed that those receiving IVIg treatment with or without steroids had greater improvement in mRS scores at 6 (p = 0.04) and 12 months (p = 0.03) [10]. These differences could be attributed to the small sample size, heterogeneity of subject age and different statistical count methods.…”

Section: Discussionsupporting

confidence: 90%

“…However, it remains unclear whether the three treatment options as initial treatment vary in terms of treatment response, the speed of recovery, long-term prognosis, further recurrence and safety profile in patients with NMDAR encephalitis. A recent systematic review from Asia with a small number of pediatric NMDAR encephalitis patients (n = 19) showed that those receiving IVIg treatment with or without steroids had greater improvement in modified Rankin Scale (mRS) scores at 6 (p = 0.04) and 12 months (p = 0.03) [10]. In addition, in studies of other neuroimmunological diseases, such as multiple sclerosis, neuromyelitis optica spectrum disease or chronic Guillain-Barré syndrome, IVMP or IVIg showed significantly different efficacy and tolerability [11][12][13].…”

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confidence: 99%

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Efficacy and tolerability of intravenous immunoglobulin versus intravenous methylprednisolone treatment in anti‐N‐methyl‐d‐aspartate receptor encephalitis

Gong

Luo

Liu

et al. 2022

Euro J of Neurology

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Encephalitis associated with antibodies against extracellular epitopes of neuronal cell surface or synaptic proteins, so-called neuronal surface antibody (NSAb) associated encephalitis, is a new category of neuroimmunological disease that occurs with focal or widespread involvement of the nervous system [1]. In encephalitis with NSAbs, the most frequently recognized autoantibodies are against N-methyld-aspartate receptor (NMDAR) [2], which account for more than 50% of all cases of autoimmune encephalitis and are associated with severe disease and economic burden [3]. The immunopathogenesis of NMDAR encephalitis has been proposed to include early cerebrospinal fluid (CSF) lymphocytosis; subsequent overrepresentation of B cells, plasma cells and dendritic cells;

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Section: Discussionsupporting

confidence: 90%

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confidence: 99%

Efficacy and tolerability of intravenous immunoglobulin versus intravenous methylprednisolone treatment in anti‐N‐methyl‐d‐aspartate receptor encephalitis

Gong

Luo

Liu

et al. 2022

Euro J of Neurology

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“…Although first-line immunotherapy (corticosteroids, IVIG, therapeutic apheresis) is widely used, limited and contrasting evidence exists regarding the best efficacy safety profile of different first-line treatment combinations (eTable 8 in the Supplement). We found that therapeutic apheresis alone (5.6-fold increased odds of good outcome) or first-line treatment options used in combination (2.7-fold increased odds with corticosteroids and IVIG; 2.8-fold increased odds with corticosteroids, IVIG, and therapeutic apheresis) were effective in NMDARE, providing support for a pragmatic approach to selection of first-line therapies guided by adverse effect profile and patient acceptability. Importantly, the only approach to first-line treatment associated with worse outcome was deferral of treatment: lack of immunotherapy within 30 days of disease onset, which occurred in 363 of 728 patients (49.9%) in the total literature review cohort, was associated with 2.7-fold increased odds of poor outcome, consistent with the findings of Titulaer et al and other studies …”

Section: Discussionmentioning

confidence: 66%

Use and Safety of Immunotherapeutic Management of N-Methyl-d-Aspartate Receptor Antibody Encephalitis

et al. 2021

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Key Points Question What are the most effective treatments for N -methyl- d -aspartate receptor (NMDAR) antibody encephalitis? Findings In this meta-analysis of individual patient data including 1550 cases, treatment factors at first event that were significantly associated with good functional outcome 12 months from disease onset included first-line treatment with therapeutic apheresis alone, corticosteroids in combination with intravenous immunoglobulin (IVIG), or corticosteroids in combination with IVIG and therapeutic apheresis, while lack of immunotherapy within 30 days of disease onset was significantly associated with poor outcome. Rituximab and long-term IVIG use were significantly associated with nonrelapsing disease course. Meaning Separate treatment factors are associated with functional outcomes and relapsing disease biology in those with NMDAR antibody encephalitis.

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“…Even patients with the most severe forms of anti-NMDAR encephalitis can eventually achieve good long-term outcomes after receiving early, positive, and unremitting combined immunotherapy and life support (25). Another study (40) involving 19 children with anti-NMDAR encephalitis in Thailand revealed that IVIG treatment, was associated with greater improvements in mRS scores. These findings underscore the benefits of IVIG treatment for this condition.…”

Section: Discussionmentioning

confidence: 99%

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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Heterogenous treatment for anti-NMDAR encephalitis in children leads to different outcomes 6–12 months after diagnosis

Cited by 11 publications

References 25 publications

Efficacy and tolerability of intravenous immunoglobulin versus intravenous methylprednisolone treatment in anti‐N‐methyl‐d‐aspartate receptor encephalitis

Efficacy and tolerability of intravenous immunoglobulin versus intravenous methylprednisolone treatment in anti‐N‐methyl‐d‐aspartate receptor encephalitis

Use and Safety of Immunotherapeutic Management of N-Methyl-d-Aspartate Receptor Antibody Encephalitis

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

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Heterogenous treatment for anti-NMDAR encephalitis in children leads to different outcomes 6–12 months after diagnosis

Cited by 11 publications

References 25 publications

Efficacy and tolerability of intravenous immunoglobulin versus intravenous methylprednisolone treatment in anti‐N‐methyl‐d‐aspartate receptor encephalitis

Efficacy and tolerability of intravenous immunoglobulin versus intravenous methylprednisolone treatment in anti‐N‐methyl‐d‐aspartate receptor encephalitis

Use and Safety of Immunotherapeutic Management of N-Methyl-d-Aspartate Receptor Antibody Encephalitis

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

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Heterogenous treatment for anti-NMDAR encephalitis in children leads to different outcomes 6–12 months after diagnosis