Antibody-Mediated Autoimmune Encephalitis in Childhood

Brenton, J. Nicholas; Goodkin, Howard P.

doi:10.1016/j.pediatrneurol.2016.04.004

Cited by 69 publications

(52 citation statements)

References 86 publications

(162 reference statements)

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“…Eighteen of the 34 included patients (52.9%) initially presented with seizures, while 10 (29.4%) presented with abnormal (psychiatric) behaviors or cognitive dysfunction. We concluded that seizures and abnormal (psychiatric) behaviors and cognitive dysfunction are the most common symptoms of pediatric anti-NMDAR encephalitis, in agreement with previous findings (13–16).…”

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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“…Commonly used first line immune-therapies include high-dose corticosteroids, IVIG and plasmapheresis. Second-line therapies include rituximab and cyclophosphamide [14,24]. Within only hours of the plasma exchange there was a marked improvement in our patient's behaviour and social interaction.…”

Section: Discussionmentioning

confidence: 89%

Anti-NMDA receptor encephalitis in a toddler

Khundakji

Masri

Khuri‐Bulos

2018

International Journal of Pediatrics and Adolescent Medicine

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Anti N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder and is considered to be one of the most common causes of encephalitis in children. Despite the fact that around half of all reported cases are of children, the number of studies that report infants and toddlers is very small. Furthermore, reports on children from the Middle East particularly are extremely rare.We report a 21-month-old Jordanian female toddler with NMDAR encephalitis, who initially presented with behavioral changes and some autistic features. She presented a diagnostic challenge due to a concurrent urinary tract infection and gastroenteritis. Multiple investigations were conducted and she was treated with methylprednisolone and intravenous immunoglobulin (IVIg) empirically as well as plasma exchange and rituximab once the diagnosis was confirmed. Her condition improved gradually. We discuss her clinical picture and the diagnostic challenges within this age group; we also review the current related literature.

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“…The protocols described by experts, largely based on small series and case reports, use a combination of first-line therapies (steroids plus IVIg or plasmapheresis) eventually escalating to rituximab or cyclophosphamide. 3,5,13 The question of chronic immunosuppression and the role of steroid-sparing agents is a sensitive one when dealing with children, considering the potential influence of long-term therapy on fertility and the risk of future cancer. 5 There are not enough data to estimate a relapse rate or a tumor association in this subtype of autoimmune encephalitis but overall, it remains a treatable disease with good response to early immunotherapy and functional outcome.…”

Section: Management and Prognosismentioning

confidence: 99%

Child Neurology: Treatable bilateral striatal lesions related to anti-dopamine 2 receptor autoimmunity

Marques-Matos¹,

Melo

Sampaio

et al. 2018

Neurology

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The symptoms of autoimmune encephalitis with antibodies directed against neuronal cell surface antigens are generally reversible by removing the antibodies or the antibody-producing B cells. 1 Although phenotypically pleomorphic, autoimmune encephalitis usually presents as a rapidly progressive encephalopathy but may present with neurologic and psychiatric manifestations without an inflammatory CSF profile 2 or abnormalities on brain MRI. Antibodies directed against dopamine 2 receptor (D2R) have been described in 14 children 3,4 presenting with encephalitis and prominent movement features. While it is debated whether D2R antibodies are pathogenic or bystanders in human disease, 5,6 the response to immunotherapy reported for most patients 7 helps to build the case in favor of their pathogenicity. Clinical case A 16-month-old healthy boy was admitted to the emergency department due to sudden onset of altered mental status and abnormal posturing of his limbs. He showed marked irritability, poor eye contact, sustained flexion of his left limbs, and extension of his right limbs alternating with mixed choreic-dystonic movements of the 4 limbs and mandible. Neurologic examination revealed considerable axial hypotonia, with the patient being unable to sit unassisted, and the patient ceased to communicate verbally, despite having prior typical language development. A recent history of vomiting for the last 2 days was elicited but there was no evidence of fever and bloodwork including ammonia levels had normal results.

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Antibody-Mediated Autoimmune Encephalitis in Childhood

Cited by 69 publications

References 86 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

Anti-NMDA receptor encephalitis in a toddler

Child Neurology: Treatable bilateral striatal lesions related to anti-dopamine 2 receptor autoimmunity

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