Resolution of EEG findings and clinical improvement in a patient with encephalopathy and ESES with a combination of immunomodulating agents other than corticosteroids: A case report

Jyonouchi, Harumi; Geng, Lee

doi:10.1016/j.ebr.2020.100379

Cited by 11 publications

(8 citation statements)

References 33 publications

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“…Clinical response to corticosteroids and other immunomodulating agents are reported, 35,36 but improvement with benzodiazepines and antiseizure medications is also seen. 37 Based on the age of onset and Hebbian mechanisms of synaptic strengthening, it has been theorized that abnormal circuitry and deficient pruning results in regional patterns of disrupted neural network development leading to long-term developmental sequela, even after the EEG abnormalities and seizures subside. 34 Interestingly, some of the genetic links found in CSWS patients are also reported in autism spectrum disorder and schizophrenia populations.…”

Section: Discussionmentioning

confidence: 99%

Disease Course and Response to Immunotherapy in Children With Childhood Disintegrative Disorder: A Retrospective Case Series

Sullivan,

Gupta,

Taylor

et al. 2023

J Child Neurol

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Childhood disintegrative disorder is a poorly understood neurobehavioral disorder of early childhood characterized by acute to subacute profound regression in previously developed language, social behavior, and adaptive functions. The etiology of childhood disintegrative disorder remains unknown and treatment is focused on symptomatic management. Interest in neuroinflammatory mechanisms has grown with the increased recognition of autoimmune brain diseases and similarities between the presenting symptoms of childhood disintegrative disorder and pediatric autoimmune encephalitis. Importantly, a diagnosis of pediatric autoimmune encephalitis requires evidence of inflammation on paraclinical testing, which is absent in childhood disintegrative disorder. Here we report 5 children with childhood disintegrative disorder who were initially diagnosed with possible autoimmune encephalitis and treated with immunotherapy. Two children had provocative improvements, whereas 3 did not change significantly on immunotherapy. Additionally, a sixth patient with childhood disintegrative disorder evaluated in our Autoimmune Brain Disease Clinic showed spontaneous improvement and is included to highlight the variable natural history of childhood disintegrative disorder that may mimic treatment responsiveness.

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Section: Discussionmentioning

confidence: 99%

Disease Course and Response to Immunotherapy in Children With Childhood Disintegrative Disorder: A Retrospective Case Series

Sullivan,

Gupta,

Taylor

et al. 2023

J Child Neurol

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“…The patient showed a 30%, 50%, and 100% reduction in the ESES with the sequential addition of three immune-modulating agents selected on the basis of monocyte cytokine profiles: respectively, anakinra, IV immunoglobulin, and sirolimus. Also, the patient's speech and behavior gradually improved [130]. The ketogenic diet (KD), already used for a long time with often satisfactory results in epileptic encephalopathies, may theoretically be considered as a treatment for ESES due to its influence on the GABA systems and its inflammation-reducing action.…”

Section: Treatments Regardless Of Neurosurgerymentioning

confidence: 99%

Continuous Spike–Waves during Slow Sleep Today: An Update

Posar,

Visconti

2024

Children

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In the context of childhood epilepsy, the concept of continuous spike–waves during slow sleep (CSWS) includes several childhood-onset heterogeneous conditions that share electroencephalograms (EEGs) characterized by a high frequency of paroxysmal abnormalities during sleep, which have negative effects on the cognitive development and behavior of the child. These negative effects may have the characteristics of a clear regression or of a slowdown in development. Seizures are very often present, but not constantly. The above makes it clear why CSWS have been included in epileptic encephalopathies, in which, by definition, frequent EEG paroxysmal abnormalities have an unfavorable impact on cognitive functions, including socio-communicative skills, causing autistic features, even regardless of the presence of clinically overt seizures. Although several decades have passed since the original descriptions of the electroclinical condition of CSWS, there are still many areas that are little-known and deserve to be further studied, including the EEG diagnostic criteria, the most effective electrophysiological parameter for monitoring the role of the thalamus in CSWS pathogenesis, its long-term evolution, the nosographic location of Landau–Kleffner syndrome, standardized neuropsychological and behavioral assessments, and pharmacological and non-pharmacological therapies.

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“…One paper on multifocal neutrophilic meningoencephalitis (MNM) was excluded as did not directly analyze the effect on the epileptic phenotype (104). Twelve articles were finally included in this review (Figure 3), for a total of 37 cases of epilepsy with different etiologies (105)(106)(107)(108)(109)(110)(111)(112)(113)(114)(115)(116). All publications were in English, six were from the USA, three were from Europe, one from Asia, and two were international studies, involving different countries.…”

Section: Study Selectionmentioning

confidence: 99%

Targeting Inflammatory Mediators in Epilepsy: A Systematic Review of Its Molecular Basis and Clinical Applications

et al. 2022

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IntroductionRecent studies prompted the identification of neuroinflammation as a potential target for the treatment of epilepsy, particularly drug-resistant epilepsy, and refractory status epilepticus. This work provides a systematic review of the clinical experience with anti-cytokine agents and agents targeting lymphocytes and aims to evaluate their efficacy and safety for the treatment of refractory epilepsy. Moreover, the review analyzes the main therapeutic perspectives in this field.MethodsA systematic review of the literature was conducted on MEDLINE database. Search terminology was constructed using the name of the specific drug (anakinra, canakinumab, tocilizumab, adalimumab, rituximab, and natalizumab) and the terms “status epilepticus,” “epilepsy,” and “seizure.” The review included clinical trials, prospective studies, case series, and reports published in English between January 2016 and August 2021. The number of patients and their age, study design, specific drugs used, dosage, route, and timing of administration, and patients outcomes were extracted. The data were synthesized through quantitative and qualitative analysis.ResultsOur search identified 12 articles on anakinra and canakinumab, for a total of 37 patients with epilepsy (86% febrile infection-related epilepsy syndrome), with reduced seizure frequency or seizure arrest in more than 50% of the patients. The search identified nine articles on the use of tocilizumab (16 patients, 75% refractory status epilepticus), with a high response rate. Only one reference on the use of adalimumab in 11 patients with Rasmussen encephalitis showed complete response in 45% of the cases. Eight articles on rituximab employment sowed a reduced seizure burden in 16/26 patients. Finally, one trial concerning natalizumab evidenced a response in 10/32 participants.ConclusionThe experience with anti-cytokine agents and drugs targeting lymphocytes in epilepsy derives mostly from case reports or series. The use of anti-IL-1, anti-IL-6, and anti-CD20 agents in patients with drug-resistant epilepsy and refractory status epilepticus has shown promising results and a good safety profile. The experience with TNF inhibitors is limited to Rasmussen encephalitis. The use of anti-α4-integrin agents did not show significant effects in refractory focal seizures. Concerning research perspectives, there is increasing interest in the potential use of anti-chemokine and anti-HMGB-1 agents.

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Resolution of EEG findings and clinical improvement in a patient with encephalopathy and ESES with a combination of immunomodulating agents other than corticosteroids: A case report

Cited by 11 publications

References 33 publications

Disease Course and Response to Immunotherapy in Children With Childhood Disintegrative Disorder: A Retrospective Case Series

Disease Course and Response to Immunotherapy in Children With Childhood Disintegrative Disorder: A Retrospective Case Series

Continuous Spike–Waves during Slow Sleep Today: An Update

Targeting Inflammatory Mediators in Epilepsy: A Systematic Review of Its Molecular Basis and Clinical Applications

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