Super refractory status epilepticus

BACKGROUND: The risk of developing epilepsy after de novo status epilepticus (SE) is nonnegligible. The individualized management of patients with high risk of subsequent epilepsy could improve long-term quality of life and cognitive impairment. We aimed to ascertain potential biomarkers of subsequent epilepsy and to construct a scoring system possessing predictive value for the diagnosis of post-SE epilepsy during follow-up. METHODS: The study data were obtained from a prospective registry of all SE episodes occurring in patients over 16 years attended in our tertiary center from February 2011 to April 2022...

We report a case of super refractory status epilepticus uncontrolled by multiple anti-seizure medications in an individual with acute liver failure due to hepatic cirrhosis and an obstructive ileocecal mass plus multiple bilateral lung lesions presumed to be metastatic. A ketamine infusion was initiated late in his hospitalization which eliminated the convulsive seizures in less than an hour. The abatement of convulsive seizures allowed his grieving wife to return to her husband's bedside to witness the withdrawal of life sustaining treatment and be present during the final 24 hours of his life...

Focal cortical dysplasia (FCD) represents the most common cause of drug-resistant epilepsy in adult and pediatric surgical series. However, genetic factors contributing to severe phenotypes of FCD remain unknown. We present a patient with an exceptionally rapid development of drug-resistant epilepsy evolving in super-refractory status epilepticus. We performed multiple clinical (serial EEG, MRI), biochemical (metabolic and immunological screening), genetic (WES from blood- and brain-derived DNA) and histopathological investigations...

Introduction: This study aims to test the hypothesis that increased ketone body production resulting from a ketogenic diet (KD) will correlate with reductions in pro-inflammatory cytokines and lipid subspecies and improved clinical outcomes in adults treated with an adjunctive ketogenic diet for super-refractory status epilepticus (SRSE). Methods: Adults (18 years or older) were treated with a 4:1 (fat: carbohydrate and protein) ratio of enteral KD as adjunctive therapy to pharmacologic seizure suppression in SRSE...

Paraneoplastic neurological disorders are rare in children, with paraneoplastic cerebellar degeneration (PCD) considered highly atypical. We describe a 13-year-old girl with progressive neurobehavioral regression, cerebellar ataxia, and intractable epilepsy presenting in super-refractory status epilepticus. After an extensive evaluation, her clinical picture was suggestive of probable autoimmune encephalitis (AE). Further diagnostic testing revealed a molecularly undefined neural-restricted autoantibody in both serum and CSF, raising suspicion over an adrenal mass previously considered incidental...

RATIONALE: Super-refractory status epilepticus is a serious illness with high morbidity and mortality, which is defined as an SE that continues or recurs 24 hours or more after the onset of anesthesia. Anesthetic agents can be either pro-convulsant or anticonvulsant or both. PATIENT CONCERNS: Epilepsy occurred at the age of 3 years. At the age of 4 years, generalized tonic-clonic seizure occurred for the first time. The patient was hospitalized at the age of 27 and 28 years for treating status epilepticus...

Febrile infection-related epilepsy syndrome (FIRES) of unknown aetiology is an extremely rare but severe epilepsy syndrome. It is characterized by a nonspecific febrile infection a few days before the onset of super-refractory status epilepticus, followed by refractory epilepsy and high morbidity in previously healthy children and young adults. To date, FIRES is incurable and irreversible. The clinical course may depend more on time than on therapy, while the outcome may depend more on the clinical spectrum than on therapy...

Coronavirus disease 2019 (COVID-19) is one of the worst pandemics in history, caused by severe acute respiratory syndrome coronavirus-2, a novel zoonotic coronavirus. COVID-19 disease can present from asymptomatic or mild infection to rapidly progressive, acute respiratory distress syndrome, and death. Neurological presentation is not so uncommon now. Super refractory status epilepticus (SRSE) can be a possible manifestation of COVID-19 disease. Here, we report a patient affected by COVID-19 who presented with SRSE...

New-onset refractory status epilepticus (NORSE) is a rare and devastating condition and the prognosis is often poor, with half to two-thirds of survivors experiencing drug-resistant epilepsy, residual cognitive impairment, or functional disability, and the mortality rate is 16% to 27% for adults. We describe a patient with cryptogenic NORSE and favorable recovery from drug-resistant super-refractory SE after the use of intravenous lidocaine. The patient experienced fever and presented with refractory generalized tonic-clonic seizures...

INTRODUCTION: The aim of this study was to explore the differences in status epilepticus (SE) management among pediatric neurology, emergency medicine, and intensive care specialists in Turkey. METHODS: A 22-item questionnaire regarding first-, second-, and third-line management strategies of SE including demographic characteristics and common etiologies according to the specialty of participants was mailed to 370 specialists working in Turkey. RESULTS: A total of 334 participants (response rate 90%) comprising 136 pediatric neurologists, 102 pediatric emergency medicine specialists, and 96 pediatric intensive care specialists completed the survey...

The pediatric febrile infection-related epilepsy syndrome (FIRES) manifests with encephalopathy with super-refractory status epilepticus (SE) a few days after or accompanying a febrile illness. It often results in refractory epilepsy and cognitive dysfunction in previously healthy children and adolescents. The underlying pathomechanism is unknown, which is why causative neuronal and/or synaptic antibodies have been discussed. We report a prospective consecutive cohort of 14 children (10 male, four female) diagnosed with FIRES in the acute phase, whose serum and CSF were comprehensively screened for underlying synaptic/neuronal autoantibodies...

The study aimed to group acute symptomatic aetiologies of consecutive episodes of status epilepticus (SE) into different subcategories and explore their associations with clinical outcome. Aetiologies were firstly categorized as "acute", "remote", "progressive", "SE in defined electroclinical syndromes", and "unknown". Four subcategories of acute aetiologies were then defined: i) withdrawal, or low levels, or inappropriate prescription of antiseizure medications, or sleep deprivation in patients with pre-existing epilepsy; ii) acute insults to central nervous system (CNS) ("acute-primary CNS"); iii) CNS pathology secondary to metabolic disturbances, systemic infection, or fever ("acute-secondary CNS"); and iv) drug/alcohol intoxication or withdrawal...

BACKGROUND AND PURPOSE: This study aimed to evaluate whether differences in clinical outcomes exist according to treatments received and seizure activity resolution in patients with refractory status epilepticus (RSE). METHODS: Consecutive episodes of non-hypoxic status epilepticus (SE) in patients ≥ 14 years old were included. Episodes of RSE were stratified in: (i) SE persistent despite treatment with first-line therapy with benzodiazepines and one second-line treatment with antiseizure medications (ASMs), but responsive to successive treatments with ASMs (RSE-rASMs); (ii) SE persistent despite treatment with first-line therapy with benzodiazepines and successive treatment with one or more second-line ASMs, but responsive to anesthetic drugs [RSE-rGA (general anesthesia)]...

IMPORTANCE: Super-refractory status epilepticus (SRSE) is defined as status epilepticus (SE) that continues or recurs 24 hours or more after the onset of anesthetic therapy or recurs on the reduction/withdrawal of anesthesia. Current clinical knowledge of the disease and optimal treatment approach is sparse. OBJECTIVE: To systematically assess clinical characteristics, causes, outcomes, prognostic factors, and treatment approaches for patients with SRSE. DESIGN, SETTING, AND PARTICIPANTS: In this systematic review and meta-analysis, all studies reporting adult patients (18 years or older) diagnosed with nonanoxic SRSE were considered for inclusion, irrespective of study design...

BACKGROUND: Multiple studies have focused on medical and pharmacological treatments and outcome predictors of patients with status epilepticus (SE). However, a sufficient understanding of recurrent episodes of SE is lacking. Therefore, we reviewed recurrent SE episodes to investigate their clinical characteristics and outcomes in patients with relapses. METHODS: In this retrospective, multicenter study, we reviewed recurrent SE patient data covering 2011 to 2017 from the university hospitals of Frankfurt and Marburg, Germany...

HCN1 is one of four genes encoding hyperpolarization-activated cyclic nucleotide-gated channels. The phenotypic spectrum associated with HCN1 variants ranges from neonatal developmental and epileptic encephalopathy to idiopathic generalized epilepsy. We report a Japanese patient with repetitive focal seizures and super-refractory status epilepticus since early infancy caused by a de novo HCN1 variant, NM_021072.4, c.1195T>C, p.(Ser399Pro). This variant might have a dominant-negative effect on channel function, leading to severe epileptic encephalopathy...

OBJECTIVE: Possible long-term consequences of status epilepticus (SE) include cognitive and behavioral impairment or the development of chronic epilepsy. However, these aspects have not been systematically studied in clinical practice. We aimed to evaluate long-term seizure recurrence after SE and the potential risk factors for their development. METHODS: Data were obtained from a prospective registry of all SE episodes occurring in adult patients who attended our center from February 2011 to April 2022...

OBJECTIVE: Super-refractory status epilepticus (SRSE) has high rates of morbidity and mortality. Few published studies have investigated neurostimulation treatment options in the setting of SRSE. This systematic literature review and series of 10 cases investigated the safety and efficacy of implanting and activating the responsive neurostimulation (RNS) system acutely during SRSE and discusses the rationale for lead placement and selection of stimulation parameters. METHODS: Through a literature search (of databases and American Epilepsy Society abstracts that were last searched on March 1, 2023) and direct contact with the manufacturer of the RNS system, 10 total cases were identified that utilized RNS acutely during SE (9 SRSE cases and 1 case of refractory SE [RSE])...

INTRODUCTION: New-onset super-refractory status epilepticus (NOSRSE) is a neurological emergency characterised by the development of status epilepticus in a patient without epilepsy or any known prior neurological disease and with no clear structural, toxic or metabolic cause, which recurs after 24 hours of induced coma. The most common identifiable cause is inflammatory-autoimmune. Consequently, we present a case of NOSRSE related to SARS-CoV-2 vaccination as an opportunity to investigate the dysimmune origin of this pathology...

INTRODUCTION: Early and effective treatment is fundamental in status epilepticus (SE) management. At the initiative of the Epilepsy Council of Malaysia, this study aimed to determine the treatment gap in SE across different healthcare settings in Malaysia. METHODS: A web-based survey was sent to clinicians involved in the management of SE, across all states and at all levels of healthcare services. RESULTS: A total of 158 responses were received from 104 health facilities, including 23 tertiary government hospitals (95...