Nonparaneoplastic Anti-N-Methyl-d-Aspartate Receptor Encephalitis: A Case Series of Four Children

Raha, Sarbani; Gadgil, Pradnya; Sankhla, Charulata Savant; Udani, Vrajesh

doi:10.1016/j.pediatrneurol.2012.01.012

Cited by 15 publications

(9 citation statements)

References 12 publications

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“…The search identified 862 potentially relevant studies, and 201 were selected for data extraction. From these, 48 references met inclusion criteria, including 27 individual case reports and 21 case series (Figure 1 ) ( 4 , 10 – 12 , 14 , 15 , 17 – 57 ). Two prospective cohort studies were found ( 45 , 53 ).…”

Section: Resultsmentioning

confidence: 99%

“…There are two main variations of instability that require critical care management: a neurologic type with catatonic or hyperkinetic crises and one that manifests with autonomic and hemodynamic instability ( 10 , 27 , 50 ). Although 84% of children have hyperkinetic movements, the literature is lacking in effective therapies ( 8 ).…”

Section: Discussionmentioning

confidence: 99%

“…Valproic acid, gabapentin, and lithium have been used for mood dysregulation ( 25 , 28 , 31 , 72 ). Clonidine, trazodone, and benzodiazepines target sleep cycle dysregulation, and phenobarbital, trihexyphenidyl (Artane™), and opioids are used for extreme agitation ( 10 , 25 , 28 , 30 , 38 , 50 ). For catatonia, benzodiazepines or rarely ECTs have been reported ( 8 , 16 , 51 , 73 , 74 ).…”

Section: Discussionmentioning

confidence: 99%

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Pediatric Anti-N-Methyl-d-Aspartate Receptor Encephalitis: A Review with Pooled Analysis and Critical Care Emphasis

et al. 2017

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PurposeAnti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is being recognized with increasing frequency among children. Given the paucity of evidence to guide the critical care management of these complex patients, we provide a comprehensive review of the literature with pooled analysis of published case reports and case series.MethodsWe performed a comprehensive literature search using PubMed, Scopus, EMBASE, and Web of Science for relevant published studies. The literature search was conducted using the terms NMDA, anti-NMDA, Anti-N-methyl-d-aspartate, pediatric encephalitis, and anti-NMDAR and included articles published between 2005 and May 1, 2016.ResultsForty-eight references met inclusion criteria accounting for 373 cases. For first-line treatments, 335 (89.8%) received high-dose corticosteroids, 296 received intravenous immunoglobulin (79.3%), and 116 (31%) received therapeutic plasma exchange. In these, 187 children (50.1%) had a full recovery with only minor deficits, 174 patients (46.7%) had partial recovery with major deficits, and 12 children died. In addition, 14 patients were reported to require mechanical ventilation.ConclusionAnti-NMDA encephalitis is a formidable disease with great variation in clinical presentation and response to treatment. With early recognition of this second most common cause of pediatric encephalitis, a multidisciplinary approach by physicians may provide earlier access to first- and second-line therapies. Future studies are needed to examine the efficacy of these current therapeutic strategies on long-term morbidity.

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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Pediatric Anti-N-Methyl-d-Aspartate Receptor Encephalitis: A Review with Pooled Analysis and Critical Care Emphasis

et al. 2017

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“…[9,10] Psychiatric symptomatology and memory impairment is more prominent in adults, whereas abnormal behavior, movement disorders, and seizure are more frequently observed in children. [4,8,11,12] Movement disorders are a prominent feature of autoimmune encephalitis, particularly in anti-NMDAR encephalitis.…”

Section: Introductionmentioning

confidence: 99%

Variations of movement disorders in anti-N-methyl-D-aspartate receptor encephalitis

et al. 2016

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Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis that presents with a wide variety of movement disorders. The purpose of our study is to review the manifestations and duration of movement disorders in different ages with NMDAR encephalitis.A retrospective cohort of 28 patients (20 females and 8 males) with positive cerebrospinal fluid (CSF) anti-NMDAR antibody in a 5-year period from major hospitals in Taiwan was enrolled. They were categorized into 3 age groups: 7 patients were ≤10 years, 14 patients were 10 to 18 years, and 7 patients were >18 years.Total 28 patients (20 females and 8 males) with age ranging from 8 months to 38 years were enrolled. Nearly all patients (n = 27/28, 96%) presented with at least 2 types of disorders, including orofacial–lingual dyskinesia (OFLD; n = 20), catatonia (n = 19), tremor (n = 11), bradykinesia (n = 11), dystonia (n = 11), choreoathethosis (n = 9), and ballism (n = 3). Only 1 patient below 10 years presented with isolated periodic choreoathethosis without other movement disorders. OFLD was common in all age groups. Choreoathetosis was most common in patients aged ≤10 years, while catatonia was most common in patients aged >10 years (P = 0.001 and 0.020, respectively). Bradykinesia was also more common in patients aged >10 years (P = 0.020). The clinical presentations of movement disorders were not significantly different in the age of 10 to 18 years and those >18 years. Neither patient ≤10 years old nor male patients had associated tumors. All patients’ movement disorders were improved after treatment, while female patients with tumors had worse short-term outcome (P = 0.014). Compared with other disorders, choreoathetosis persisted significantly longer in patients ≤10 years (P = 0.038), while OFLD and catatonia last longer in patients >10 years (P = 0.047 and 0.002, respectively).Our study shows that hyperkinetic movements such as choreoathetosis are more common and last longer in younger age groups, whereas hypokinetic movements such as catatonia and bradykinesia are more common and last longer in older age groups. Female patients with ovarian tumors had worse short-term outcome.

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“…Titulaer 2013). It was first characterized as a paraneoplastic syndrome in young women with ovarian teratomas, but has since been expanded to include children and men with or without tumors (Raha 2012). Between 37% and 65% of cases are now thought to occur in children.…”

Section: Discussionmentioning

confidence: 99%

Neuroleptic Malignant Syndrome in a Boy with NMDA Receptor Encephalitis

Berg

Byrne

Coffey

2015

Journal of Child and Adolescent Psychopharmacology

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Chief Complaint and Presenting ProblemM . is an 11-year-old African American boy who was referred to the psychiatry consult liaison service eight days after admission to an epilepsy monitoring unit for behavioral disruption and inability to sleep. History of Present IllnessParents reported that M. had been a normally developing fifth grader who did well in school. M. developed the sudden onset of seizures six weeks prior to admission; his family found him curled in a fetal position with irregular jerking movements. Parents transported him to the emergency department, and an EEG demonstrated absence seizures. M. was hospitalized for several days, treated with valproic acid, and then discharged to outpatient followup. During this admission, M. was evaluated by the psychiatry team for anxiety, as he had expressed fear that he was going to die as a result of his illness. He also endorsed auditory hallucinations of hearing voices, at one point telling him to have more seizures. Outpatient psychotherapy was recommended.In the following six weeks, M. had no identified seizures but experienced many new symptoms. M.'s attention, concentration, and academic performance declined dramatically, according to his teachers. He had multiple crying fits and mood swings each day. M. became increasingly somnolent, but had difficulty sleeping each night and began to slur his speech. Teachers recommended an Individualized Education Plan one month prior to referral due to his significant academic decline.One day prior to admission, M. presented to the emergency department with four absence seizures, and was loaded with valproic acid and discharged. On the day of admission, M. again presented to the emergency department with two brief absence seizures culminating in a generalized tonic-clonic seizure that lasted 30 minutes and required intravenous midazolam. M.'s serum valproic acid level was 126 (therapeutic range 50-100 ug/mL) and ammonia was 102 (normal range 11-35 uM/L). His presumptive diagnosis was hyperammonemia due to valproic acid toxicity, and he was admitted.Following admission, M. experienced episodes of dystonic posturing and nystagmus, only some of which were associated with epileptiform activity on long-term electroencephalogram (EEG) monitoring. His EEG consistently showed diffuse delta wave slowing. At the time of psychiatry consultation, M. was in full upper-extremity restraints due to agitation, and his verbal capacity had regressed to repeating the same word or phrase, often speaking in unintelligible sounds entirely. He followed commands inconsistently, and had not slept more than three hours a night in over a week. M. was given 0.3 mg of risperidone for agitation the night before psychiatry consultation. Psychiatry consultation was requested to further advise on medication management.After extensive assessment, psychiatry concluded that M. had suffered a grand mal seizure of unknown etiology and had recent cognitive decline consistent with delirium. In addition to continuing the extensive workup already underway, t...

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Nonparaneoplastic Anti-N-Methyl-d-Aspartate Receptor Encephalitis: A Case Series of Four Children

Cited by 15 publications

References 12 publications

Pediatric Anti-N-Methyl-d-Aspartate Receptor Encephalitis: A Review with Pooled Analysis and Critical Care Emphasis

Pediatric Anti-N-Methyl-d-Aspartate Receptor Encephalitis: A Review with Pooled Analysis and Critical Care Emphasis

Variations of movement disorders in anti-N-methyl-D-aspartate receptor encephalitis

Neuroleptic Malignant Syndrome in a Boy with NMDA Receptor Encephalitis

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