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Non-convulsive status epilepticus in Sotos syndrome: rare first presentation in a rare syndrome.

INTRODUCTION: Prior to illustration of the causative genetic mutation responsible for Sotos syndrome, diagnosis was based on clinical criteria. They include characteristic facial gestalt, developmental delay, and evidence of overgrowth, in addition to other minor features as cardiac &genitourinary congenital malformation, seizures, scoliosis, among other features. Non-convulsive status epilepticus (NCSE) was not previously reported among Sotos patients. CASE SUMMARY: An eleven-years old boy, with developmental delay, characteristic facial & skeletal features presented to the emergency departm…

Differentiation of psychogenic nonepileptic attacks from status epilepticus among patients intubated for convulsive activity.

BACKGROUND AND OBJECTIVE: Patients with psychogenic nonepileptic attacks (PNEA) sometimes receive aggressive treatment leading to intubation. This study aimed to identify patient characteristics that can help differentiate PNEA from status epilepticus (SE). METHODS: We retrospectively identified patients with a final diagnosis of PNEA or SE, who were intubated for emergent convulsive symptoms and underwent continuous electroencephalography (cEEG) between 2012 and 2017. Patients who had acute brain injury or progressive brain disease as the cause of SE were excluded. We compared clinical featu…

Sustained Effort Network for treatment of Status Epilepticus (SENSE) - A multicenter prospective observational registry.

Status epilepticus (SE) is an important neurological emergency lacking adequate evidence for efficacy and safety of treatment beyond the application of benzodiazepines as first treatment step. To bridge the gap between the few pivotal trials and retrospective uncontrolled case series, we established a prospective multicenter registry recruiting patients in experienced centers in German-speaking countries. We could document 1179 episodes of 1049 patients over a period of 5 years. First data analysis showed that in the majority of the episodes, established treatment guidelines were not followed.…

Dexmedetomidine administration in a patient with status epilepticus under color density spectral array monitoring.

BACKGROUND: Status epilepticus requires immediate treatment because treatment delay can cause permanent neurologic complications. Dexmedetomidine may be an option for the treatment of status epilepticus although its effect remains unclear with conflicting reports. CASE PRESENTATION: A 64-year-old woman with epilepsy with complex partial seizures underwent total knee arthroplasty. After emergence from general anesthesia, she developed status epilepticus and was transferred to the intensive care unit. Following initial treatment using benzodiazepines, phenytoin, and levetiracetam, dexmedetomidi…

[Neuronal intranuclear inclusion disease with prolonged impaired consciousness and status epilepticus: a case report].

Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease presenting with various manifestations including dementia, wea … e to our hospital and two additional seizures shortly after the arrival, which indicated convulsive status epilepticus (SE). The ictal EEG showed low amplitude fast activity arising from the left centro-parietal area with an evolutionary pattern. The clinical concomitant was the contralateral versive seizure evolving to a bilateral convulsion. Inter-ictal epileptifo …

Status epilepticus and bilateral middle cerebral artery infarction: A rare presentation after viper bite.

Owing to the antihemostatic property of viper venom, hemorrhagic complications including intracerebral hemorrhage are the most commonly encountered after viper bite. Ischemic strokes have been rarely reported after viper envenomation, and its occurrence has been attributed to multiple mechanisms. Postsnakebite seizures are known to occur after neurotoxic bite. Here, we report the case of a viper bite victim who developed status epilepticus within 3 h after viper bite. He had only mild signs of local envenomation, and prolonged whole blood clotting time was the only manifestation of systemic en…

Включает лимбическую систему, Spinal Cord и Peripheral Nerves.

Здоровая 28-летняя женщина неожиданно попала в неразрешимую эпилептику: припадок мозга превратился в общий припадок, которому предшествует высокая температура. Мозговые магнитные резонансные снимки указывают на двустороннюю гиперинтенсию в гиппокампе на Т2 взвешенной снимке. Электроэнцефалограммы постоянно демонстрируют диффузные острые волны и полипики. Комплексная иммунодибилизационная терапия и антиэпилептические препараты не привели к каким-либо улучшениям. Поэтому мы диагностировали у нее криптогенный лимфатический энцефалит и новообразованный рефрактический эпилептик (NORSE). Мы обнаружи

Совпадение в аннотации оригинала
A healthy 28-year-old woman presented suddenly with intractable status epilepticus: a focal seizure evolved into a generalized seizure preceded by a high fever. Brain magnetic resonance imaging indicated bilateral hyperintensities in the hippocampus on T2-weighted imaging. Electroencephalograms continuously demonstrated diffuse sharp waves and poly-spikes. C…

Inadequate benzodiazepine dosing may result in progression to refractory and non-convulsive status epilepticus.

Status epilepticus (SE) is defined as ongoing seizures lasting longer than five minutes or multiple seizures without recovery. Benzodiazepines (BZDs) are first-line agents for the management of SE. Our objective was to evaluate BZD dosing in SE patients and its effects on clinical/electrographic outcomes. A retrospective analysis was conducted from a prospective database of SE patients admitted to a university-based neurocritical care unit. The initial presentation and progression to refractory SE (RSE) and non-convulsive SE (NCSE) with coma was evaluated. Outcome measures included length of s…

Soman-induced status epilepticus, epileptogenesis, and neuropathology in carboxylesterase knockout mice treated with midazolam.

OBJECTIVE: Exposure to chemical warfare nerve agents (CWNAs), such as soman (GD), can induce status epilepticus (SE) that becomes refractory to benzodiazepines when treatment is delayed, leading to increased risk of epileptogenesis, severe neuropathology, and long-term behavioral and cognitive deficits. Rodent models, widely used to evaluate novel medical countermeasures (MCMs) against CWNA exposure, normally express plasma carboxylesterase, an enzyme involved in the metabolism of certain organophosphorus compounds. To better predict the efficacy of novel MCMs against CWNA exposure in human ca…

VEGF treatment during status epilepticus attenuates long-term seizure-associated alterations in astrocyte morphology.

Vascular endothelial growth factor (VEGF) treatment during pilocarpine-induced status epilepticus (SE) causes sustained preservation of behavioral function in rats in the absence of enduring neuroprotection (Nicoletti et al., 2010), suggesting the possibility that other cells or mechanisms could be involved in the beneficial effects of VEGF during SE. Astrocytes have been reported to contribute to epileptiform discharges in the hippocampus (Tian et al., 2005; Kang et al., 1998) and to express VEGF receptors (Krum & Rosenstein, 2002). We report here that VEGF treatment significantly alters mult…

[Irreversible cerebral ischemia caused by febrile status epilepticus in Sturge-Weber syndrome type III].

We report a 9-year-old girl with Sturge-Weber syndrome (SWS) type III, whose motor function deteriorated after an episode of febrile status epilepticus. The patient had leptomeningeal angiomas in the left temporal, occipital, and parietal lobes. Complex partial seizures, which started at 8 months, were controlled by antiepileptic medications. At 9 years of age, she developed irreversible ischemic lesions in the left temporal and occipital regions after the febrile status epilepticus and her motor function deteriorated. In addition to antiepileptic medications, aspirin therapy was started.SWS t…

Diagnosis of Electrical Status Epilepticus During Slow-Wave Sleep With 100 Seconds of Sleep.

PURPOSE: Strategies for diagnosing electrical status epilepticus during slow-wave sleep (ESES) vary among interpreting neurologists. Our aim was to evaluate if the spike-wave index (SWI) for the first 100 seconds of sleep is reflective of the SWI when compared with a conventional method. METHODS: We reviewed EEGs from 2005 to 2011 that were considered diagnostic of ESES based on unspecified methods. The SWI for the first nonrapid eye movement sleep cycle (long method) was calculated by two neurophysiologists. Two different neurophysiologists calculated SWI for the first 100 seconds of sleep (…

Midazolam-ketamine dual therapy stops cholinergic status epilepticus and reduces Morris water maze deficits.

OBJECTIVE: Pharmacoresistance remains an unsolved therapeutic challenge in status epilepticus (SE) and in cholinergic SE induced by nerve agent intoxication. SE triggers a rapid internalization of synaptic γ-aminobutyric acid A (GABAA ) receptors and externalization of N-methyl-d-aspartate (NMDA) receptors that may explain the loss of potency of standard antiepileptic drugs (AEDs). We hypothesized that a drug combination aimed at correcting the consequences of receptor trafficking would reduce SE severity and its long-term consequences. METHODS: A severe model of SE was induced in adult Sprag…

Status Epilepticus After Subthalamic Deep Brain Stimulation Surgery in a Patient with Parkinson's Disease.

BACKGROUND: Subthalamic deep brain stimulation (DBS) is a well-established treatment for patients with Parkinson's disease who suffer from severe motor fluctuations. The most common surgery-related complications are temporary confusion, bleedings, infections, and seizures. Seizures have been described to occur mainly around the time of the implantation of electrodes and, at present, the best established risk factors for seizures in association with DBS surgery are bleedings. A postoperative status epilepticus as complication of DBS surgery has never been described before. CASE DESCRIPTION: We…

Blood-brain barrier leakage after status epilepticus in rapamycin-treated rats II: Potential mechanisms.

OBJECTIVE: Blood-brain barrier (BBB) leakage may play a pro-epileptogenic role after status epilepticus. In the accompanying contrast-enhanced magnetic resonance imaging (CE-MRI) study we showed that the mammalian target of rapamycin (mTOR) inhibitor rapamycin reduced BBB leakage and seizure activity during the chronic epileptic phase. Given rapamycin's role in growth and immune response, the potential therapeutic effects of rapamycin after status epilepticus with emphasis on brain inflammation and brain vasculature were investigated. METHODS: Seven weeks after kainic acid-induced status epil…

Observational study of intravenous lacosamide in patients with convulsive versus non-convulsive status epilepticus.

BACKGROUND AND OBJECTIVES: Status epilepticus (SE) is an important emergency situation associated with high morbidity and mortality. The goal of pharmacological therapy-rapid seizure termination-is only achieved in just over half of patients with first-line anti-epileptic drug (AED) therapy and many patients require second and higher lines of AEDs to achieve seizure termination; therefore, there is a clear need for more effective treatment options. Lacosamide is a relatively new AED and the intravenous formulation has shown promise for treatment of SE. The aim of the current study was to compa…