In this case, NORSE was the presenting feature of HSE and the refractoriness of her seizures was terminated only after treating the exact cause, that is, encephalitis. [ncbi.nlm.nih.gov]

Poster presentation at the American Epilepsy Society Meeting 2012. 12. [pserg.org]

Multicentre collaboration is required to establish the diagnostic criteria and appropriate management of patients presenting with NORSE. [research.manchester.ac.uk]

• Epilepsy

  Platform presentation Dec 8, 2014 at the American Epilepsy Society Meeting, Seattle, WA. (Epilepsy Currents, 2014, in press). 3. [pserg.org]

  Epilepsy and paroxysmal conditions. 2020;12(3):169-176. (In Russ.) https://doi.org/10.17749/2077-8333/epi.par.con.2020.034 Views: 858 [epilepsia.su]

  Epilepsy is a general term for the tendency to have seizures. Epilepsy is usually diagnosed only after a person has had more than one seizure. When identifiable, the causes of epilepsy usually involve some form of injury to the brain. [webmd.com]

  Epilepsy develops in most cases. The role of anesthetics and immune therapies warrants further investigation. © 2015 American Academy of Neurology. [ncbi.nlm.nih.gov]

• Fever

  Despite the fever, no known infection has been found to cause FIRES, and the fever may or may not be present when the seizures begin. [rarediseases.info.nih.gov]

  FIRES FIRES is a subcategory of NORSE that requires a prior febrile infection, with fever starting between 2 weeks and 24 h prior to the onset of refractory status epilepticus, with or without fever at the onset of status epilepticus. [kauveryhospital.com]

  Abstract A 39–years–old woman presented with generalized seizures after 7 days of fever. Generalized seizures did not stop despite administration of several anti–epileptic drugs and sedatives. [jstage.jst.go.jp]

  This was an interesting case of a patient who presented with fever, NORSE, and a rare secondary cause of paraneoplastic syndrome. [consultant360.com]

  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. [go.gale.com]

• Fatigue

  New-onset refractory status epilepticus is an acute encephalopathy with inflammation-mediated status epilepticus characterized by an acute refractory status epilepticus, typically of the tonic-clonic type, following prodromal symptoms of confusion, fever, fatigue [orpha.net]

  The symptoms may include upper respiratory tract infections, gastroenteritis, fever, fatigue, or confusion.2-4 The most common type of seizure is focal motor that can transition into tonic-clinic seizures. [consultant360.com]

  Symptoms and signs The course of the syndrome begins as a mild fever, malaise, fatigue, and upper respiratory tract or gastrointestinal infection. Two third of patients of NORSE sometimes hallucinate, apathy or agitation, and amnesia. [kauveryhospital.com]

  Case description A previously healthy 18-year-old male was admitted to the hospital with new-onset seizures following episodes of staring, drooling, and unresponsiveness in addition to flu-like symptoms and fatigue on the day prior to admission. [link.springer.com]

  View Full Report Print / Download as PDF Next section > < Previous section Next section > Signs & Symptoms In two-thirds of NORSE cases, the course of the syndrome begins with a mild febrile illness, associated with malaise, fatigue and symptoms of upper [rarediseases.org]

• Vietnamese

  Seizures and raised intracranial pressure in Vietnamese patients with Japanese encephalitis. Brain. 2002; 125: 1084-1093 Bien C.G. Urbach H. Schramm J. Soeder B.M. Becker A.J. Voltz R. et al. [jns-journal.com]

• Surgical Procedure

  These can range from structural conditions such as stroke, traumatic brain injury and surgical procedures, to infectious causes, to medications, to metabolic causes such as renal failure, drug withdrawal or overdose, and electrolyte imbalance. [clinicaladvisor.com]

• Seizure

  He developed refractory multifocal and generalised seizures, which required admission to intensive care unit and administration of general anaesthetic. Seizures recurred on withdrawal of barbiturate anaesthetic until day 29. [ncbi.nlm.nih.gov]

  Patient presented torpid evolution, persisting fever and seizures, despite anti-seizure drugs and barbituric sedation. [pesquisa.bvsalud.org]

  His seizure persisted even with multiple anti-epileptic drugs and required prolonged general anesthesia under mechanical ventilation. [go.gale.com]

• Convulsions

  HIRSCH: It’s very common after someone has convulsive seizures, if you treat them and they stop but they don’t wake up, those people are at very high risk of having non-convulsive seizures and non-convulsive status. [ilae.org]

  Although several anticonvulsants were used, convulsive attacks were not resolved and intravenous anesthetics were used to stop status epileptics. [jstage.jst.go.jp]

  […] epilepsy ( G40.- ) R56.1 ) seizure (convulsive) NOS ( ICD-10-CM Diagnosis Code R56.9 Unspecified convulsions 2016 2017 2018 2019 Billable/Specific Code Applicable To Convulsion disorder Fit NOS Recurrent convulsions Seizure(s) (convulsive) NOS R56.9 [icd10data.com]

  It is important to understand that a seizure is a CNS event and may present as convulsive or non-convulsive. [healthjade.net]

• Encephalopathy

  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. [go.gale.com]

  Homepage Rare diseases Search Search for a rare disease Disease definition New-onset refractory status epilepticus is an acute encephalopathy with inflammation-mediated status epilepticus characterized by an acute refractory status epilepticus, typically [orpha.net]

  Results: A 23-year-old male, with history of a cousin with epileptic encephalopathy, presented refractory convulsive status epilepticus (SE), preceded by febrile episodes, requiring non-barbituric sedation and admission to intensive care unit (ICU). [pesquisa.bvsalud.org]

  Febrile infection-related epilepsy syndrome Synonyms Acute non-herpetic encephalitis with severe refractory status epilepticus, Fever-induced refractory epileptic encephalopathy in school-aged children Febrile infection-related epilepsy syndrome (FIRES [en.wikipedia.org]

• Generalized Tonic-Clonic Seizure

  However, he developed generalized tonic-clonic seizures on day 6 of admission that worsened despite treatment with multiple AEDs. [e-jnc.org]

  He had presented to the emergency department with new-onset generalized tonic-clonic seizure activity. [resident360.nejm.org]

  Case A 28-year old female, a known hypertensive for 2 years, had 2 episodes of generalized tonic-clonic seizures (GTCS) following 2 days of fever. She was conscious in the interictal period. She had no prior history of seizures. [j-epilepsy.org]

  Soon after arriving at the ED, he showed generalized tonic-clonic seizure (GTCS) starting from his left limbs, which ceased after intravenous diazepam 10 mg. Weakness, pyramidal signs, and meningeal irritation signs were not seen. [jintensivecare.biomedcentral.com]

  CORRESPONDENCE: Amanda Nayberg, PA, South Shore University Hospital, 301 East Main Street, Bay Shore, NY 11706 ([email protected]) A 78-year-old man presented to our emergency department with generalized tonic-clonic seizures of unknown duration and fever [consultant360.com]

• Somnolence

  #4) fourth-line: phenobarbital Phenobarbital is an effective antiseizure medication, but it is difficult to titrate and may lead to somnolence. Thus, for most patients phenobarbital isn't a front-line agent. [emcrit.org]

  On initial clinical exam, the patient appeared somnolent although neurologic exam (cranial nerves, motor strength, sensory) was without deficits. Two-hour EEG monitoring revealed continuous 1.5–2-Hz right frontal lateralized periodic discharges. [link.springer.com]

  Level of consciousness: (Alert/somnolent/confused = 0, stuporous/comatose = 1), Worst seizure type (Simple (partial/complex partial, absence, myoclonic) = 0, generalized-convulsive = 1, non-convulsive status epilepticus in coma = 2), Age (≤65 years = [dovepress.com]

  It is generally well tolerated and common side effects include ataxia, blurred vision, and somnolence. [annalsofian.org]

  Memory issues and daytime somnolence due to medications limited his ability to return to employment. The Prodromal Phase The patients affected by this condition are generally young (mean age at SE onset is 25 years; mode 29 years). [frontiersin.org]

The patient had a very extensive diagnostic workup including autoimmune, viral and genetic investigations. A tentative immune therapy was proposed with high doses of steroids and plasma exchanges. [sciensano.be]

CSF bacterial, viral, and autoimmune workups were all negative, JE workup was also negative; another systemic workup was negative. [kauveryhospital.com]

This case highlights the complex diagnostic workup of patients with NORSE and the utility of validated prediction models for diagnosis and prognosis. [karger.com]

Her workup included computed tomography and magnetic resonance imaging of the head, a lumbar puncture, toxicology screening, and extensive testing for multiple infectious and inflammatory etiologies. [ncbi.nlm.nih.gov]

Furthermore, it is advised in further studies to publish all the extensive workup done including some techniques already reported to increase the diagnostic accuracy of NORSE. Financial support and sponsorship Nil. [cmijournal.org]

Anakinra in the treatment of new-onset refractory status epilepticus: Our experience from a clinical case European Journal of Neurology ; 28(SUPPL 1):699, 2021. [pesquisa.bvsalud.org]

Treatment There is currently no known specific therapy for NORSE and studies are urgently needed to determine what the best treatment options are. [epilepsy.com]

Initial treatment included up to 3 anticonvulsants (including one anesthetic), followed by additional anticonvulsants/anesthetics along with antimicrobials, followed by steroids, plasma exchange, hypothermia and ketogenic diet. [ncbi.nlm.nih.gov]

At 48 hours: Assess returned testing, initiate appropriate treatments If patient continues to have refractory status epilepticus or coma, transfer to higher level of care for appropriate further treatment of NORSE at a center with experience in these [mdedge.com]

If a specific etiology is found, treatment should be directed at that etiology. [practicalneurology.com]

It is a neurologic emergency, and determining the underlying etiology is an important factor for effectively managing and predicting the prognosis of NORSE. [ncbi.nlm.nih.gov]

In these common cases, the prognosis for full recovery is good. [pharmacytimes.com]

PURPOSE: To summarize the clinical features, suggested work-up, treatment and prognosis of new-onset refractory status epilepticus (NORSE), a condition recently defined as the occurrence of refractory status epilepticus (RSE) in a patient without active [read.qxmd.com]

This case highlights the complex diagnostic workup of patients with NORSE and the utility of validated prediction models for diagnosis and prognosis. [karger.com]

The prognosis for NORSE survivors is poor as 50% of survivors develop a chronic cognitive or functional disability and often have epilepsy moving forward. [operativeneurosurgery.com]

CONCLUSIONS: Though an underlying etiology remains unproven in these patients, we contend that NORSE is etiologically heterogeneous, with a proportion of cases due to non-infectious causes. [ncbi.nlm.nih.gov]

Conclusions Though an underlying etiology remains unproven in these patients, we contend that NORSE is etiologically heterogeneous, with a proportion of cases due to non-infectious causes. [jns-journal.com]

Epidemiology of status epilepticus. J Clin Neurophysiol. 1995;12:316-325. DeLorenzo RJ, Hauser WA, Towne AR. A prospective, population-based epidemiologic study of status epilepticus in Richmond, Virginia. Neurology. 1996;46:1029-1035. [pharmacytimes.com]

A prospective, population-based epidemiologic study of status epilepticus in Richmond, Virginia. Neurology. 1996; 46: 1029-1035 Knake S. Rosenow F. Vescovi M. Oertel W.H. Mueller H.H. Wirbatz A. et al. Status Epilepticus Study Group Hessen (SESGH). [jns-journal.com]

Status epilepticus in older patients: epidemiology and treatment options. Drugs Aging. 2001. 18(2):133-42. [Medline]. Drislane FW, Blum AS, Lopez MR, Gautam S, Schomer DL. [emedicine.medscape.com]

A prospective, population-based epidemiologic study of status epilepticus in Richmond, Virginia. Neurology. 1996 Apr. 46(4):1029-35. DeLorenzo RJ, Pellock JM, Towne AR, Boggs JG. Epidemiology of status epilepticus. [healthjade.net]

Guidelines for epidemiologic studies on epilepsy. Commission on epidemiology and prognosis, international league against epilepsy. Epilepsia 1993;34:592-6. [ PUBMED ] 2. Lowenstein DH, Alldredge BK. Status epilepticus. [annalsofian.org]

Better results have been observed with immunotherapy, more recently with anti-cytokine drugs, such as tocilizumab and anakinra, consistent with the immunological/inflammatory mechanisms proposed for the pathophysiology of NORSE. [revecuatneurol.com]

Conclusion: Given the proposed pathophysiology for NORSE, anakinra is an interesting treatment option. We report a positive, although incomplete, response to anakinra. More evidence is required to support this indication. [pesquisa.bvsalud.org]

Although the pathophysiology remains elusive, nascent evidence suggests a role for a postinfectious cytokine-mediated mechanism, which should be further investigated. [epilepsia.sen.es]

We propose early use of plasma exchange therapy (Class IV evidence) in hopes to prevent the complications of status epilepticus and prolonged hospitalization. [ncbi.nlm.nih.gov]

In the context of prevention and treatment processes, it is not only necessary to terminate epileptic seizures, but to also address the disruption of the patient’s internal environment and provide critical life support. [books.google.com]

Other new treatments include NMDA receptor antagonists which promote neuronal cell membrane repolarization and so prevent the excitation from prolonging, thus also helping to prevent the potential damage to the depolarized neurons as a result of excitotoxin [news-medical.net]

Pierre, MD May 1, 2019 Journal of Clinical Outcomes Management April 3, 2019 Aspirin for primary prevention: USPSTF recommendations for CVD and colorectal cancer April 3, 2019 Dustin K. Smith, DO, et al Dustin K. [jcomjournal.com]

Matthew Zack and Rosemarie Kobau from the US Center for Disease Control and Prevention ( CDC) regarding their latest study on the increase in number of epilepsy cases in the US. [player.fm]