Epileptic phenotypes, electroclinical features and clinical characteristics in 17 children with anti-NMDAR encephalitis

Haberlandt, Edda; Ensslen, Matthias; Gruber-Sedlmayr, U; Plecko, Barbara; Brunner‐Krainz, Michaela; Schimmel, Mareike; Schubert‐Bast, Susanne; Neirich, U; Philippi, Heike; Kurleman, G; Tardieu, Marc; Wohlrab, Gabriele; Borggraefe, Ingo; Rostásy, Kevin

doi:10.1016/j.ejpn.2016.11.016

Cited by 36 publications

(22 citation statements)

References 15 publications

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“…Moreover, 54.9% (39/71) of the seizures were focal (Table 1). Most patients initially presented with behavioral changes and seizures, which aligned with previous findings [14,15]. In an observational cohort study, 87% of patients exhibited 4 or more categories of symptoms by the end of the first month [16].Our study investigated whether clinical symptoms were associated with sex and CSF antibody titers in patients with anti-NMDAR encephalitis.…”

Section: Discussionsupporting

confidence: 82%

Clinical features and influencing prognostic factors in patients with anti-NMDAR encephalitis: a cohort follow-up study in Chinese patients

Wang

Miao

Shi

et al. 2019

Preprint

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Objectives The clinical manifestations of patients with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis in East China and factors associated with prognosis were analyzed.Methods A retrospective study of 106 patients (58 females; 48 males) with anti-NMDAR encephalitis in East China was carried out from June 2015 to February 2019. Clinical features and factors influencing outcomes were reviewed.Results Behavioral changes were observed in 74.5% (79/106) of patients, and comprised the initial symptoms in 61.3% (65/106). Seizures were observed in 67% (71/106) of patients, and served as initial symptoms in 31.1% (33/106). A total of 54.9% (39/71) of seizures were focal seizures. More clinical symptoms were observed in female patients than in male patients ( P =0.000). Similarly, background activity (BA) with cerebrospinal fluid (CSF) antibody titers at the peak stage was more severe in female patients than in male patients ( P = 0.000). The Binary logistic regression and ROC curve analyses revealed the factors associated with poor outcomes included consciousness disturbance (OR: 4.907, 95% CI: 1.653-14.562, P =0.004; area: 65.4%, sensitivity: 44.2%, specificity: 86.5%, P =0.014 ), EEG BA (OR: 3.743, 95% CI: 1.766-7.932, P =0.001; area: 76.6%, sensitivity: 73%, specificity: 75%, P =0.000), number of symptoms (OR: 2.911, 95% CI: 1.811-4.679, P =0.000; area: 77.1%, sensitivity: 59.5%, specificity: 78.6%, P =0.000) and CSF antibody titer (OR: 31.778, 95% CI: 8.891-113.57, P =0.000; area: 83.9%, sensitivity: 89.2%, specificity: 78.6%, P =0.000).Conclusions EEG BA and number of symptoms were associated with CSF antibody titers. Consciousness disturbances, EEG BA, number of symptoms and CSF antibody titers served as predictors of poor outcomes.

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Section: Discussionsupporting

confidence: 82%

Clinical features and influencing prognostic factors in patients with anti-NMDAR encephalitis: a cohort follow-up study in Chinese patients

Wang

Miao

Shi

et al. 2019

Preprint

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“…An EEG may show the very distinctive ‘extreme delta brush’ pattern in up to a third of adults, but other studies have found substantially lower frequencies . Even higher rates of this pattern have been observed in children, and non‐specific EEG abnormalities, such as diffuse slowing, are common . Neuroimaging is rarely helpful in making an early diagnosis of anti‐NMDArE, although magnetic resonance imaging may be abnormal in more than half of patients within 3 months of illness onset, and a magnetic resonance imaging measure of large‐scale brain network connectivity shows promise as a diagnostic tool …”

Section: Discussionmentioning

confidence: 99%

Recognizing psychiatric presentations of anti‐NMDA receptor encephalitis in children and adolescents: A synthesis of published reports

Gurrera

2019

Psychiatry Clin Neurosci

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Aim The aim of this study was to improve early recognition of anti‐N‐methyl‐d‐aspartate receptor encephalitis (anti‐NMDArE) in children and adolescents by identifying characteristic temporal patterns of clinical features in patients likely to be referred for psychiatric evaluation. In this form of autoimmune encephalitis, NMDAr hypofunction is caused by autoantibodies to receptor surface components. Clinical outcomes following prompt immunotherapy are usually good, but delayed treatment often results in a protracted course with significant residual disability or death. Anti‐NMDArE frequently mimics psychiatric disorders, so most patients are referred initially to a psychiatrist and treated for days or weeks before being correctly diagnosed. Methods A systematic search of PubMed and EMBASE electronic databases identified all published reports of antibody‐confirmed anti‐NMDArE associated with psychiatric symptoms in patients <19 years old. Redundant reports were eliminated manually. For each patient, the order in which each feature was first observed was ranked relative to others. Median temporal ranks were used to compare the sequencing of individual features and major symptom domains. Results One hundred and sixty seven cases (121 females) met the inclusion criteria. The most common features were dyskinesias (77.8%), seizures (72.5%), mutism or staring (40.7%), insomnia (39.5%), language dysfunction (36.5%), fever (31.1%), disorientation/confusion (28.7%), reduced arousal (28.1%), and memory disturbance (26.9%). The configuration and temporal sequencing of features were highly variable between individuals. Conclusion Clinicians need to suspect this disorder: if new behavioral symptoms arise in the context of a recent viral prodrome; if they are accompanied by dyskinesias, seizures or insomnia; or if psychiatric symptoms are unusual (e.g., non‐verbal auditory hallucinations).

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“…For instance, the EEG pattern named extreme delta brush has been found in children and adults with anti-NMDAR encephalitis and is highly characteristic of this disorder although it is only detectable in a subgroup of patients. [54,69] Extensive multifocal cortical-subcortical T2/FLAIR MRI abnormalities occur in approximately 80% of patients with anti-GABA A R encephalitis, but rarely occur in other AE. [47,52*]…”

Section: Introductionmentioning

confidence: 99%

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

Spatola

Dalmau

2017

Current Opinion in Neurology

147

138

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Purpose of review To assess the seizure manifestations and risk of epilepsy in encephalitis associated to antibodies against neuronal cell-surface (AE) or myelin-associated antigens, and to review several chronic epileptic disorders including, Rasmussen’s encephalitis (RE), fever-induced refractory epileptic syndromes (FIRES), and new-onset refractory status epilepticus (NORSE). Recent findings Seizures are a frequent manifestation of AE. Some AE may associate with characteristic features: faciobrachial dystonic seizures (anti-LGI1 encephalitis), EEG extreme delta brush (anti-NMDAR), or multifocal FLAIR-MRI abnormalities (anti-GABAAR). In anti-LGI1 encephalitis, cortical, limbic, and basal ganglia dysfunction results in different types of seizures. AE or myelin-antibody associated syndromes are often immunotherapy-responsive and appear to have a low risk for chronic epilepsy. In contrast patients with seizures related to GAD65-antibodies (an intracellular antigen) frequently develop epilepsy and have suboptimal response to treatment (including surgery). RE or FIRES may occur with autoantibodies of unclear significance and rarely respond to immunotherapy. A study of patients with NORSE showed that 30% developed chronic epilepsy. Summary Although seizures are frequent in all types of AE, the risk for chronic epilepsy is dependent on the antigen: lower if located on the cell-surface, and higher if intracellular. For other disorders (RE, FIRES, NORSE) the prognosis remains poor.

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Epileptic phenotypes, electroclinical features and clinical characteristics in 17 children with anti-NMDAR encephalitis

Cited by 36 publications

References 15 publications

Clinical features and influencing prognostic factors in patients with anti-NMDAR encephalitis: a cohort follow-up study in Chinese patients

Clinical features and influencing prognostic factors in patients with anti-NMDAR encephalitis: a cohort follow-up study in Chinese patients

Recognizing psychiatric presentations of anti‐NMDA receptor encephalitis in children and adolescents: A synthesis of published reports

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

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