New-onset refractory status epilepticus treated with vagus nerve stimulation: A case report

Refractory status epilepticus is defined as persistent seizures despite appropriate use of two intravenous medications, one of which is a benzodiazepine. It can be seen in up to 40% of cases of status epilepticus with an acute symptomatic etiology as the most likely cause. New-onset refractory status epilepticus (NORSE) is a recently coined term for refractory status epilepticus where no apparent cause is found after initial testing. A large proportion of NORSE cases are eventually found to have an autoimmune etiology needing immunomodulatory treatment. Management of refractory status epilepticus involves treatment of an underlying etiology in addition to intravenous anesthetics and antiepileptic drugs. Alternative treatment options including diet therapies, electroconvulsive therapy, and surgical resection in case of a focal lesion should be considered. Short-term and long-term outcomes tend to be poor with significant morbidity and mortality with only one-third of patients reaching baseline neurological status.

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“…Efficacy varied between more than 50% improvement and seizure freedom. It was tolerated very well [ 117 – 122 ].…”

Section: Treatmentmentioning

confidence: 99%

Updates in Refractory Status Epilepticus

Marawar

Basha

Mahulikar

et al. 2018

Critical Care Research and Practice

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“…NORSE patients are often in critically ill status because of prolonged anesthesia and ventilation; therefore, clinicians should evaluate which treatment would be appropriate for each clinical condition. Rituximab [ 2 ], cyclophosphamide [ 2 , 14 ], ketamine [ 2 , 15 ], and vagal nerve stimulation [ 16 ] have been reported as other possible treatment options for NORSE so far and should be considered if initial treatments are not effective.…”

Section: Discussionmentioning

confidence: 99%

A favorable outcome of intensive immunotherapies for new-onset refractory status epilepticus (NORSE)

et al. 2018

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BackgroundNew-onset refractory status epilepticus (NORSE) is a newly defined critical disease entity characterized by prolonged periods of refractory epileptic seizure with no readily identifiable cause in otherwise healthy individuals. Its etiology is uncertain, but autoimmune encephalitis is a possible candidate for the underlying cause of this condition. Immunotherapies could be considered for this condition, but its efficacy is not established.Case presentationA 31-year-old man with no prior history presented with refractory status epilepticus. His seizure persisted even with multiple anti-epileptic drugs and required prolonged general anesthesia under mechanical ventilation. Magnetic resonance imaging and cerebrospinal fluid did not indicate the cause of seizure, and autoantibodies related to encephalitis were not detected. It was speculated that the patient had occult autoimmune encephalopathy because of its acute-onset clinical course preceded by fever, even without definite evidence of an autoimmune mechanism. The patient received intravenous methylprednisolone, plasma exchange, and intravenous immunoglobulin in succession and manifested a favorable outcome after these treatments.ConclusionOur case supports the efficacy of immunotherapies for NORSE even though it does not manifest definite evidence for autoimmune background. Clinicians should consider these immunotherapies for NORSE as early as possible, because this condition is associated with high mortality and morbidity owing to prolonged seizure activity and long-term intensive care including general anesthesia and mechanical ventilation.

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“…PubMed search using the keywords autoimmune encephalitis and epilepsies and epilepsy surgery identified only eight studies that reported the outcome of epilepsy surgery in autoimmune encephalitis and antibody-associated drugresistant epilepsy 20,30,31,[33][34][35][36][37] and two reports on the efficacy of vagus nerve stimulation (VNS) therapy in antibodyassociated refractory status epilepticus, 38,39 one of the patients who had VNS had prior anterior four-fifth corpus callosotomy (►Table 1). 38 Majority of these patients were adults. In some of the patients who underwent resective surgery, surgery was performed with the provisional diagnosis of focal cortical dysplasia or tumor 30 and in others, autoimmune epilepsy was not known or suspected at the time the decision for surgery was made.…”

Section: Surgical Treatmentmentioning

confidence: 99%

“…44,45 Two cases of immunotherapy and drug refractory status epilepticus secondary to autoantibody-mediated encephalitis have been reported: one was associated with anti-NMDA receptor encephalitis 39 and the other with glutamate (Glu) receptor antibodies. 38 In both cases, rapid VNS titration was done within a few days, which resulted in successful weaning of intravenous (IV) anesthestics and significant improvement in seizure control. The role of VNS in the control and modulation of inflammatory response has been explored.…”

Section: Surgical Treatmentmentioning

confidence: 99%

Epilepsy Surgery in Antibody-Associated Autoimmune Encephalitis and Epilepsies: Review of Literature

Luat

2018

J Pediatr Epilepsy

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There is lack of studies that explore the role of epilepsy surgery in the treatment of drug-refractory antibody-associated autoimmune encephalitis and epilepsies. Some case reports and series suggest that surgical treatment may still be an option, but compared with other etiologies, its surgical outcome seems to be less favorable. The following review summarizes the existing experience on the surgical treatment of drug-refractory seizures associated with antibody-associated autoimmune encephalitis and epilepsies.

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New-onset refractory status epilepticus treated with vagus nerve stimulation: A case report

Cited by 24 publications

References 8 publications

Updates in Refractory Status Epilepticus

Updates in Refractory Status Epilepticus

A favorable outcome of intensive immunotherapies for new-onset refractory status epilepticus (NORSE)

Epilepsy Surgery in Antibody-Associated Autoimmune Encephalitis and Epilepsies: Review of Literature

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