Efficacy and Safety of Rituximab in Chinese Children With Refractory Anti-NMDAR Encephalitis

Dou, Xiangjun; Li, Dongjing; Wang, Yan; Wang, Zhijing; Le, Yang; Ma, Nan; Wang, Dong; Li, Xia

doi:10.3389/fneur.2020.606923

Cited by 11 publications

(8 citation statements)

References 27 publications

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“…Antibodies against the N-methyl-d-aspartate receptor (NMDAR) are predominately of the IgG1 subclass (280). Patients with NMDAR encephalitis generally respond to rituximab, and in one case titers were undetectable post-treatment (152,239,(280)(281)(282). In an effort to enhance the effect of rituximab induction treatment, two non-randomized trials observed clinical benefit from repeated monthly dosing of rituximab in addition to induction dosing (283); and the addition of tocilizumab to rituximab (240).…”

Section: Nmdar Encephalitismentioning

confidence: 99%

Short- and Long-Lived Autoantibody-Secreting Cells in Autoimmune Neurological Disorders

2021

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As B cells differentiate into antibody-secreting cells (ASCs), short-lived plasmablasts (SLPBs) are produced by a primary extrafollicular response, followed by the generation of memory B cells and long-lived plasma cells (LLPCs) in germinal centers (GCs). Generation of IgG4 antibodies is T helper type 2 (Th2) and IL-4, -13, and -10-driven and can occur parallel to IgE, in response to chronic stimulation by allergens and helminths. Although IgG4 antibodies are non-crosslinking and have limited ability to mobilize complement and cellular cytotoxicity, when self-tolerance is lost, they can disrupt ligand-receptor binding and cause a wide range of autoimmune disorders including neurological autoimmunity. In myasthenia gravis with predominantly IgG4 autoantibodies against muscle-specific kinase (MuSK), it has been observed that one-time CD20+ B cell depletion with rituximab commonly leads to long-term remission and a marked reduction in autoantibody titer, pointing to a short-lived nature of autoantibody-secreting cells. This is also observed in other predominantly IgG4 autoantibody-mediated neurological disorders, such as chronic inflammatory demyelinating polyneuropathy and autoimmune encephalitis with autoantibodies against the Ranvier paranode and juxtaparanode, respectively, and extends beyond neurological autoimmunity as well. Although IgG1 autoantibody-mediated neurological disorders can also respond well to rituximab induction therapy in combination with an autoantibody titer drop, remission tends to be less long-lasting and cases where titers are refractory tend to occur more often than in IgG4 autoimmunity. Moreover, presence of GC-like structures in the thymus of myasthenic patients with predominantly IgG1 autoantibodies against the acetylcholine receptor and in ovarian teratomas of autoimmune encephalitis patients with predominantly IgG1 autoantibodies against the N‐methyl‐d‐aspartate receptor (NMDAR) confers increased the ability to generate LLPCs. Here, we review available information on the short-and long-lived nature of ASCs in IgG1 and IgG4 autoantibody-mediated neurological disorders and highlight common mechanisms as well as differences, all of which can inform therapeutic strategies and personalized medical approaches.

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Section: Nmdar Encephalitismentioning

confidence: 99%

Short- and Long-Lived Autoantibody-Secreting Cells in Autoimmune Neurological Disorders

2021

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“…Fortunately, their treatment principles are generally similar, consisting of HIMP or IVIg. Notably, RTX is more likely to be used to treat anti-NMDAR encephalitis with an acute onset ( Dalmau et al, 2011 ; Dou et al, 2020 ; Thaler et al, 2021 ), but only in remission periods for maintenance treatment in MOGAD. We recommend that patients with MOGAD should be tracked and simultaneously tested for MOG-Ab and other antibodies in the serum and CSF for the early detection of an overlapping syndrome.…”

Section: Discussionmentioning

confidence: 99%

Myelin Oligodendrocyte Glycoprotein Antibody Associated Cerebral Cortical Encephalitis: Case Reports and Review of Literature

Shu

Ding

Shang

et al. 2022

Front. Hum. Neurosci.

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Myelin oligodendrocyte glycoprotein antibody-associated disease is an immune-mediated demyelinating disease of the central nervous system that is present in both adults and children. The most common clinical manifestations are optic neuritis, myelitis, acute disseminated encephalomyelitis, and brainstem syndrome. Cerebral cortical encephalitis (CCE) is a rare clinical phenotype of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), which usually begins with seizures, headaches, and fever, and may be misdiagnosed as viral encephalitis in the early stages. Herein, we report two typical MOG antibody (MOG-Ab)-positive patients presenting with CCE, both of whom presented with headache, fever, seizures, and who recovered completely after immunotherapy. In addition, we performed a systematic review of the present literature from the perspectives of population characteristics, clinical symptoms, MRI abnormalities, treatments, and prognosis. Among the patients reported in 25 articles, 33 met our inclusion criteria, with the age of onset ranging from 4 to 52 years. Most of the patients had seizures, headache, fever, and unilateral cortical lesions on brain MRI. For acute CCE, 30 patients were treated with high-dose intravenous methylprednisolone, and the symptoms of most patients were completely relieved after immunotherapy. This study reported our experience and lessons learned in the diagnosis and treatment of MOG-Ab-positive CCE and provides a systematic review of the literature to analyse this rare clinical phenotype.

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“…There has been studies on anti-NMDAR encephalitis being refractory to 1st line treatment but responded well to 2nd line immune therapy with minimal side effects like in our case. 13 The neurological presentation of SARS-CoV2 in the pediatric age group is still surfacing. We need to brace ourselves up with newer presentations as we are still in the middle of this pandemic situation which seems to linger on for some time.…”

Section: Discussionmentioning

confidence: 99%

Post COVID anti-NMDAR Encephalitis in an adolescent girl

Alvi¹,

HaiderSultan²,

Sultan³

2022

PJNS

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Anti-NMDAR encephalitis is a neuro-inflammatory disorder caused by antibodies targeting neurons in the central nervous system. Certain viruses break immune tolerance of neuronal proteins and increase the permeability of blood brain barrier (BBB) to antibodies resulting in post infectious autoimmunity. We report a case about a 13 years old girl diagnosed as a case of anti-NMDAR encephalitis, who presented with psychiatric symptoms followed by abnormal movements and encephalopathy, with later development of autonomic instability with tachycardia and blood pressure at 98th centile. Her thyroid profile initially showed hyperthyroidism with negative anti-thyroid antibodies with subsequent normalization without anti-thyroid drug. Her SARS-CoV-2 IgM was positive despite being asymptomatic that had induced autoimmunity in central nervous system and caused transient thyroiditis. She poorly responded to first tier immune therapy (methylprednisolone pulse & plasmapheresis) but there was remarkable response to second tier immune therapy with Rituximab with prompt attainment of ambulation and self care.

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Efficacy and Safety of Rituximab in Chinese Children With Refractory Anti-NMDAR Encephalitis

Cited by 11 publications

References 27 publications

Short- and Long-Lived Autoantibody-Secreting Cells in Autoimmune Neurological Disorders

Short- and Long-Lived Autoantibody-Secreting Cells in Autoimmune Neurological Disorders

Myelin Oligodendrocyte Glycoprotein Antibody Associated Cerebral Cortical Encephalitis: Case Reports and Review of Literature

Post COVID anti-NMDAR Encephalitis in an adolescent girl

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