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New-Onset Refractory Status Epilepticus

NORSE


Presentation

  • 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]
Cat Scratch
  • Serologic results were positive for Bartonella henselae, and she was diagnosed with cat-scratch disease (CSD). She did not have the typical presentation of symptoms of lymphadenopathy, however, which is common in CSD.[ncbi.nlm.nih.gov]
Lymphadenopathy
  • She did not have the typical presentation of symptoms of lymphadenopathy, however, which is common in CSD. Doxycycline 100 mg and rifampin 300 mg twice daily were added to the patient's anticonvulsant and corticosteroid therapy.[ncbi.nlm.nih.gov]
Intermittent Fever
  • In the week preceding his presentation, the patient had headaches, intermittent fever, nausea, and vomiting. Magnetic resonance imaging (MRI) brain with and without contrast was unremarkable.[mdedge.com]
Sputum
  • , bacterial and fungal stains and cultures, VDRL, PCR for HSV1, HSV2, VZV, EBV, HIV, M Tb Recommended in immunocompromised patients, in addition to above: Serologic: IgG Cryptococcus species, IgM and IgG Histoplasma capsulatum, IgG Toxoplasma gondii Sputum[mdedge.com]
Vomiting
  • Case 1 was a 26 year old male with a prodrome of headache and vomiting. He developed refractory multifocal and generalised seizures, which required admission to intensive care unit and administration of general anaesthetic.[ncbi.nlm.nih.gov]
  • In the week preceding his presentation, the patient had headaches, intermittent fever, nausea, and vomiting. Magnetic resonance imaging (MRI) brain with and without contrast was unremarkable.[mdedge.com]
Nausea
  • In the week preceding his presentation, the patient had headaches, intermittent fever, nausea, and vomiting. Magnetic resonance imaging (MRI) brain with and without contrast was unremarkable.[mdedge.com]
Learning Difficulties
  • Four months after presentation with SE, the patient showed deficits in short-term verbal and visuospatial memory, learning difficulties, and executive function problems. At 6 months she returned to her normal life with family support.[journal.frontiersin.org]
Gait Ataxia
  • On discharge his neurological examination showed mild gait ataxia, limb dysmetria, with intermittently perseverative speech and hyper-religiosity.[journal.frontiersin.org]
Dysmetria
  • On discharge his neurological examination showed mild gait ataxia, limb dysmetria, with intermittently perseverative speech and hyper-religiosity.[journal.frontiersin.org]
Cognitive Deficit
  • Moreover, 65% of the patients developed cognitive deficits. These deficits were reported to be mild for 60% and moderate to severe for 40% of these patients.[journal.frontiersin.org]
Anomia
  • On initial exam, he was afebrile, lethargic, disoriented, and had anomia, but normal cranial nerve, motor and reflex examinations. In the week preceding his presentation, the patient had headaches, intermittent fever, nausea, and vomiting.[mdedge.com]

Workup

  • 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]
  • […] autoantibodies involved in autoimmune encephalitis and epilepsy and general pathophysiological hypotheses on the role of immunity in seizure genesis are discussed, specific epilepsy syndromes in which autoimmune components have been studied are summarized, workup[pediatrics.aappublications.org]
  • Diagnostic workup The steps included in the diagnostic workup should be completed as soon as possible and occur simultaneously and in parallel with treatment.[jnaccjournal.org]
  • In our opinion genetic aetiology may not be an absolute contraindication for surgery; phenotypic expression and a personalized pre-surgical workup could help to select which patients could benefit from surgery.[ccrejournal.eu]
  • Although most of the symptoms are not specific to NCSE, rapid and unexpected emergence of any of them in the absence of another plausible explanation calls for a prompt workup that includes EEG.[nature.com]
Xanthochromia
  • Cerebrospinal fluid findings (CSF) studies showed a glucose of 74 mg/dL (normal), protein of 24 mg/dL (normal), no xanthochromia, 1 red blood cell, and 13 white blood cells, all lymphocytes. Acyclovir was started and phenytoin was loaded.[mdedge.com]
Bartonella Henselae
  • Serologic results were positive for Bartonella henselae, and she was diagnosed with cat-scratch disease (CSD). She did not have the typical presentation of symptoms of lymphadenopathy, however, which is common in CSD.[ncbi.nlm.nih.gov]
  • Serum and CSF: IgG and IgM testing for Chlamydia pneumoniae, Bartonella henselae, Mycoplasma pneumonia, Coxiella burnetii, Shigella species, and Chlamydia psittaci Nares: Respiratory viral DFA panel CSF: Cell counts, protein, and glucose, bacterial and[mdedge.com]
Chlamydia
  • Serum and CSF: IgG and IgM testing for Chlamydia pneumoniae, Bartonella henselae, Mycoplasma pneumonia, Coxiella burnetii, Shigella species, and Chlamydia psittaci Nares: Respiratory viral DFA panel CSF: Cell counts, protein, and glucose, bacterial and[mdedge.com]
Coxiella Burnetii
  • Serum and CSF: IgG and IgM testing for Chlamydia pneumoniae, Bartonella henselae, Mycoplasma pneumonia, Coxiella burnetii, Shigella species, and Chlamydia psittaci Nares: Respiratory viral DFA panel CSF: Cell counts, protein, and glucose, bacterial and[mdedge.com]
Toxoplasma Gondii
  • gondii Sputum: M Tb Gene Xpert Serum and CSF: Toxoplasma IgG CSF: Eosinophils, silver stain for CNS fungi, PCR for JC virus, CMV, HHV6, EEE, Enterovirus, Influenza A/B, WNV, Parvovirus.[mdedge.com]

Treatment

  • 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]
  • Multiple immune modulatory medications were provided for treatment of presumed autoimmune encephalitis, including, corticosteroids, rituximab, IVIG, and plasma exchange.[neurology.org]
  • Effective treatment calls for multi-disciplinary support from neurology, neurosurgery, emergency care, critical care medicine, and other fields.[books.google.com]
  • There is currently no evidence that cryptogenic NORSE, including FIRES, is hereditary. [1] [2] [3] If an underlying cause for NORSE is found, the treatment depends on the cause. There is no standard treatment for cryptogenic NORSE, including FIRES.[rarediseases.info.nih.gov]

Prognosis

  • 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]
  • Refractory status epilepticus: effect of treatment aggressiveness on prognosis. Arch Neurol. 2005;62:1698-1702. Holtkamp M, Othman J, Buchheim K, et al.[icmcasesummaries.com]
  • Aetiology is all important for prognosis • Aetiology appears to more important for determining prognosis than duration of status or age of the patient • For example refractory status epilepticus associated with hypoxic brain injury has an almost universally[slideshare.net]
  • […] display a very poor prognosis and SE duration is the determinant of duration of GA.[ccforum.biomedcentral.com]

Etiology

  • 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]

Epidemiology

  • 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]
  • Burneo, MD, MSPH, Associate Professor of Neurology, Epidemiology, and Biostatistics, and Director of the EEG Laboratory and Co-Director, Epilepsy Programme at the Univ ...… In this episode of Epilepsy.com's Hallway Conversations, Dr.[player.fm]
  • 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]
  • Epidemiology: We estimate the following annual incidences in the United States, though these are likely to be underestimates: Status epilepticus: 45,000 cases.[mdedge.com]
  • Epidemiology of status epilepticus. J. Clin. Neurophysiol. 12, 316–325 (1995). 11. Rosenow, F., Hamer, H. M. & Knake, S. The epidemiology of convulsive and nonconvulsive status epilepticus. Epilepsia 48 (Suppl. 8), 82–84 (2007). 12. Dunne, J.[nature.com]
Sex distribution
Age distribution

Pathophysiology

  • More specifically, various autoantibodies involved in autoimmune encephalitis and epilepsy and general pathophysiological hypotheses on the role of immunity in seizure genesis are discussed, specific epilepsy syndromes in which autoimmune components have[pediatrics.aappublications.org]
  • CSF samples showed lymphocytic pleiocytosis and oligo-clonal bands, which supports a role for the immune system in the pathophysiology of this condition.[journal.frontiersin.org]
  • […] diagnosis characterised by: volitional or non-volitional preserved consciousness or purposeful movements asymmetric movements poorly coordinated thrashing back arching eyes held shut head rolling pelvic thrusting bicycling/ pedalling movements of legs PATHOPHYSIOLOGICAL[lifeinthefastlane.com]
  • The pathophysiology of propofol infusion syndrome: a simple name for a complex syndrome. Intensive Care Med 2003;29:1417-25. Crossref 35. Rossetti AO, Lowenstein DH.[hkmj.org]
  • Status epilepticus: pathophysiology and management in adults. Lancet Neurol. 2006;5(3):246–56.[resus.com.au]

Prevention

  • 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]
  • 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]
  • 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]
  • Resuscitation to prevent secondary brain injury and maintain cerebral perfusion pressure 2. Terminate seizure 3. Decrease cerebral metabolic rate 4. Diagnose and treat cause 5.[lifeinthefastlane.com]

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