Symptomatic treatment of children with anti‐NMDAR encephalitis

Mohammad, Shekeeb S.; Jones, Hannah; Hong, Martin; Nosadini, Margherita; Sharpe, Cynthia; Pillai, Supriya; Brilot, Fabienne; Dale, Russell C.

doi:10.1111/dmcn.12882

Cited by 66 publications

(43 citation statements)

References 26 publications

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“…However, given that most patients have neither preceding HSVE nor a teratoma and this idiopathic group have the highest relapse rate, the most common immunological triggers of this condition have clinical importance and await discovery. One such trigger may be genetic, perhaps a HLA predisposition, particularly given the known nonwhite racial bias of this condition …”

Section: The Nsab‐mediated Syndromes and Their Related Immunologymentioning

confidence: 99%

The clinical features, underlying immunology, and treatment of autoantibody‐mediated movement disorders

et al. 2018

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An increasing number of movement disorders are associated with autoantibodies. Many of these autoantibodies target the extracellular domain of neuronal surface proteins and associate with highly specific phenotypes, suggesting they have pathogenic potential. Below, we describe the phenotypes associated with some of these commoner autoantibody‐mediated movement disorders, and outline increasingly well‐established mechanisms of autoantibody pathogenicity which include antigen downregulation and complement fixation. Despite these advances, and the increasingly robust evidence for improved clinical outcomes with early escalation of immunotherapies, the underlying cellular immunology of these conditions has received little attention. Therefore, here, we outline the likely roles of T cells and B cells in the generation of autoantibodies, and reflect on how these may guide both current immunotherapy regimes and our future understanding of precision medicine in the field. In addition, we summarise potential mechanisms by which these peripherally‐driven immune responses may reach the central nervous system. We integrate this with the immunologically‐relevant clinical observations of preceding infections, tumours and human leucocyte antigen‐associations to provide an overview of the therapeutically‐relevant underlying adaptive immunology in the autoantibody‐mediated movement disorders. © 2018 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.

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Section: The Nsab‐mediated Syndromes and Their Related Immunologymentioning

confidence: 99%

The clinical features, underlying immunology, and treatment of autoantibody‐mediated movement disorders

et al. 2018

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“…Many of these psychiatric manifestations were in fact exhibited by our patient during the initial course of her illness. Use of antipsychotic medications to control agitation, psychosis, and/or movement disorder in patients has been associated with neuroleptic malignant syndrome and therefore should be included in the differential [9].…”

Section: Case Presentationmentioning

confidence: 99%

Progression from Acute Psychosis to Seizures and Autonomic Instability in an Adolescent with Ovarian Mass

Wen¹

2017

Clin Med Rev Case Rep

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Anti-N-Methyl-D-Aspartate Receptor Encephalitis (NMDARE) is a complex clinical entity that is both diagnostically and therapeutically challenging. A 17-year-old girl presented to the emergency room with worsening anxiety, confusion, and acute psychosis. While in an inpatient psychiatric unit, she developed progressive catatonia with decreased responsiveness. After transfer to the pediatric inpatient unit, she became hyperreflexic with seizure-like activity consisting of back arching, left arm extension, and right arm flexion refractory to lorazepam. Electroencephalogram (EEG) monitoring was started on the first hospital day and within twenty-four hours showed extreme delta brush pattern and abnormal background activity. Cerebrospinal fluid analysis demonstrated pleocytosis with a lymphocytic predominance. Imaging studies were notable for a left ovarian mass and non-specific sulcal hyperintensity on magnetic resonance imaging of the brain. The ovarian mass was subsequently resected, and tumor pathology was consistent with a grade II immature teratoma. Serum and CSF NMDAR IgG antibody titers wereboth elevated to 1:320, which combined with extreme delta brush pattern on EEG confirmed the diagnosis of paraneoplastic NMDARE. Further treatment for the patient's NMDARE after tumor removal included intravenous immune globulin, high dose pulse steroids, plasmapheresis, and rituximab. The patient's hospital course was complicated by episodes of autonomic instability. Prevention of dysautonomic crises, agitation, and rigidity were managed with benzodiazepines, benztropine, and diphenhydramine. Antipyretics were given for unexplained fevers while multiple antihypertensive agents were given for episodic hypertension. Over several weeks, the patient demonstrated gradual neurocognitive improvement to near-baseline levels and was discharged to a rehabilitation facility for further recovery. NMDARE is a major cause of disease with both psychiatric and neurologic features.Symptomatic treatment can be challenging requiring a balance of pharmacologic therapies. In the pediatric population, NMDARE is an important diagnosis to consider in applicable cases since it is a highly treatable disease with an overall good prognosis after adequate treatment.

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“…It is recommended that anti-psychotic therapy be used for a short period to avoid extrapyramidal side-effects. 17 A multidisciplinary approach including psychiatric support is very important. The recovery rate is considered to be excellent in children with anti-NMDAR encephalitis.…”

Section: Figure 1 Case 3: Initial Mr Images (Day 3 Of Admission): (A)mentioning

confidence: 99%

Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports

Bashiri¹,

Al-Rasheed

Hassan³

et al. 2016

Paediatrics and International Child Health

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Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently identified auto-immune disorder characterised by severe memory deficit, a decreased level of consciousness, seizures, autonomic dysfunction and movement disorders. Three girls with the disorder are reported; they were aged 4 years, 5 years and 10 months. The 10-month-old infant who is one of the youngest patients reported with anti-NMDAR encephalitis worldwide, had MRI features suggestive of herpes simplex encephalitis (known to trigger anti-NMDAR encephalitis), but CSF PCR for herpes simplex was negative. All the patients presented with seizures, behavioural change, regression of speech, dystonia and choreo-athetosis. Anti-NMDAR antibodies were detected in all patients' sera and cerebrospinal fluid (CSF). Intravenous immunoglobulin, corticosteroids and rituximab were administered at different intervals. Cases 1 and 2 made a full recovery, but case 3 has mild motor and speech delay. Patients who present with encephalopathy, seizures and movement disorders should be tested for anti-NMDAR antibodies in serum and CSF in addition to being screened for herpes simplex encephalitis.

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Symptomatic treatment of children with anti‐NMDAR encephalitis

Cited by 66 publications

References 26 publications

The clinical features, underlying immunology, and treatment of autoantibody‐mediated movement disorders

The clinical features, underlying immunology, and treatment of autoantibody‐mediated movement disorders

Progression from Acute Psychosis to Seizures and Autonomic Instability in an Adolescent with Ovarian Mass

Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports

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