Status Epilepticus: Immunologic and Inflammatory Mechanisms

Miskin, Chandrabhaga; Hasbani, Daphne M.

doi:10.1016/j.spen.2014.09.001

Cited by 21 publications

(12 citation statements)

References 38 publications

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“…A greater understanding of the pathophysiology of SE has led to its recent redefinition . A new area of particular interest is the role of the immune system and inflammation . There is emerging evidence that proinflammatory cytokines are acutely increased in patients with seizures.…”

Section: Mechanismsmentioning

confidence: 99%

Rethinking status dystonicus

Ruiz-López

Fasano

2017

Movement Disorders

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Status dystonicus is a movement disorder emergency that has been a source of controversy in terms of terminology, phenomenology, and management since it was first described in 1982. Here we argue that the current use of the term status dystonicus falls well short of the precision needed for either clinical or academic use. We performed a critical review on this topic, describing possible pathophysiological mechanisms and areas of uncertainties. This review also addresses the problems derived by the extreme clinical heterogeneity of this condition, as the lack of an objective criterion useful for the definition, or the fact that status dystonicus may present not only in the context of a known dystonic syndrome. We propose a new possible definition that includes not only dystonia but also other hyperkinetic movements in the wide range of movement disorders that can be seen during an episode. The new definition keeps the term status dystonicus and highlights the fact that this is a medical emergency based on the impairment of bulbar and/or respiratory function requiring hospital admission as the principal feature. Furthermore, the new definition should not consider as necessary unspecific features as patient's condition at baseline, the distribution of dystonia, occurrence of systemic symptoms such as fever or laboratory findings. We hope that this proposal will stimulate the debate on this subject among our peers, further developing a clinical and pathophysiological understanding of status dystonicus. © 2017 International Parkinson and Movement Disorder Society.

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Section: Mechanismsmentioning

confidence: 99%

Rethinking status dystonicus

Ruiz-López

Fasano

2017

Movement Disorders

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“…These immunological approaches, which may be considered as corroborating the third-line of treatment (at the bottom of Figure 1 ), are not evidence-based, but in current clinical practice rely on the assumption that inflammation is the direct cause of SE; their administration typically occurs after the start of the refractory SE episode. However, as we will see in the following sections, inflammation may also play a determinant role in ongoing seizures independently of an infection or auto-immune cause [50,51]; this may thus lead to reconsideration of the treatment flow in SE patients.…”

Section: Framing the Problem From The Clinical Sidementioning

confidence: 99%

Immunity and inflammation in status epilepticus and its sequelae: possibilities for therapeutic application

Vezzani

Dingledine

Rossetti

2015

Expert Review of Neurotherapeutics

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Status epilepticus (SE) is a life-threatening neurological emergency often refractory to available treatment options. It is a very heterogeneous condition in terms of clinical presentation and causes, which besides genetic, vascular and other structural causes also include CNS or severe systemic infections, sudden withdrawal from benzodiazepines or anticonvulsants and rare autoimmune etiologies. Treatment of SE is essentially based on expert opinions and antiepileptic drug treatment per se seems to have no major impact on prognosis. There is, therefore, urgent need of novel therapies that rely upon a better understanding of the basic mechanisms underlying this clinical condition. Accumulating evidence in animal models highlights that inflammation ensuing in the brain during SE may play a determinant role in ongoing seizures and their long-term detrimental consequences, independent of an infection or auto-immune cause; this evidence encourages reconsideration of the treatment flow in SE patients.

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“…Progressive accumulation of inflammatory cytokines could then give way to a vicious cycle of aberrant hyperexcitability and/or a structural epileptogenic remodeling of brain networks [10]. Additionally, intrinsic factors, such as a genetic predisposition, may be responsible for the lack of an efficient resolution of the epileptogenic process [11].…”

Section: Etiology and Pathophysiologymentioning

confidence: 99%

“…Ann Child Neurol 2020;28(1): [8][9][10][11][12][13][14][15] Clinical manifestations FIRES in previously healthy children generally has three phases. The first phase is the unremarkable febrile illness.…”

Section: Etiology and Pathophysiologymentioning

confidence: 99%

Febrile Infection-Related Epilepsy Syndrome: Refractory Status Epilepticus and Management Strategies

Lee

2020

Ann Child Neurol

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Febrile infection-related epilepsy syndrome (FIRES) is a rare, catastrophic epileptic syndrome that strikes previously healthy children. Its pathogenesis is unknown, it has few treatments, and it is typically refractory. In FIRES, refractory status epilepticus or a cluster of seizures starts a few days after the onset of an acute febrile illness, and it may continue as drug-resistant epilepsy, with neuropsychological impairments occurring without latency. Clinical knowledge and guidelines on FIRES are limited because it is sporadic and extremely rare. To date, the absence of specific biomarkers poses a significant diagnostic challenge; nevertheless, early diagnosis is very important for optimal management. Despite treatment with multiple immunotherapies and anti-seizure medications, the majority of patients with FIRES are left with significant cognitive disabilities and refractory epilepsy. This review aims to highlight the most recent insights into the clinical features, terminology, epidemiology, pathogenesis, diagnostic challenges, and therapeutic options associated with FIRES.

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Status Epilepticus: Immunologic and Inflammatory Mechanisms

Cited by 21 publications

References 38 publications

Rethinking status dystonicus

Rethinking status dystonicus

Immunity and inflammation in status epilepticus and its sequelae: possibilities for therapeutic application

Febrile Infection-Related Epilepsy Syndrome: Refractory Status Epilepticus and Management Strategies

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