Complete Recovery in NORSE Observed in İntensive Care Unit: A Case Report

doi:10.21203/rs.3.rs-2464507/v1

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Case Report

Complete Recovery in NORSE Observed in İntensive Care Unit: A Case Report

https://doi.org/10.21203/rs.3.rs-2464507/v1

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New-onset refractory status epilepticus (NORSE) is a definition used to describe clinical conditions with no history of seizures, no identifiable structural or etiological cause, and for which a diagnostic result cannot be reached by research. Its treatment is often done with intravenous anesthetic drugs and its mortality is quite high. In this article, a 33-year-old patient whose etiology could not be determined as a result of extensive research in our intensive care unit and who recovered completely is presented.

Critical Care & Emergency Medicine

status epilepticus

magnetic resonance

lumbar puncture

Approximately 23–43% of status epilepticus (SE) patients are resistant to treatment and are usually associated with an underlying acute, severe, and potentially fatal etiology (eg, encephalitis, severe stroke, or malignant brain tumors). The mortality of refractory SE (RSE) is reported to be 23–61%, and intravenous (iv) anesthetic treatment and intensive care follow-up are often required (1, 2). New-onset RSE (NORSE) occurs in healthy individuals with long-term resistant seizures without an easily identifiable cause (2). New-onset RSE is a rare clinical picture rather than a specific diagnosis. An etiology has been identified in approximately 50% of adult NORSE cases. Most cases with a known etiology are due to sporadic or paraneoplastic autoimmune encephalitis (1–3).

A 33-year-old female patient with no other disease other than known migraine was orotracheal intubated in an external center because of abdominal pain and generalized tonic-clonic seizures 8 hours after normal vaginal delivery. No pathology was detected in the computed tomography and diffusion-weighted magnetic resonance imaging (MRI) taken immediately afterwards.

LP (lumbar puncture) was performed, Bos was slightly cloudy, no leukocytes, 1–2 erythrocytes were seen. The patient, whose seizures continued under antiepileptic treatment and pentothal infusion was started, was referred to our intensive care unit with a preliminary diagnosis of sporadic encephalitis on the 2nd day of his hospitalization. The patient's vital signs were stable, his electrocardiography was in normal sinus rhythm, and his biochemistry and hemogram parameters were within normal limits. With the information she received from her doctor, a history of two units of erythrocyte suspension replacement was obtained due to bleeding during vaginal delivery, and her examinations were expanded to exclude Sheehan's Syndrome. No history of drug use or toxic exposure. In her family history, her brother had a history of epilepsy after appendectomy. Homocysteine level and MTHFR gene analysis were sent and they were found to be at normal levels.

On the second day of his hospitalization, cranial MR and MR Venography (Fig. 1) was repeated and no finding in favor of cerebral venous thrombosis was detected. Electroencephalography background activity consisted of 6–7 Hz diffuse and slow theta activity and was not reported as epileptiform anomaly. Lumbar puncture (LP) was repeated to the patient. Cerebrospinal fluid (CSF) cell count and biochemistry were within normal limits. Viral and bacterial meningitis panels were negative. Central nervous system infection was not considered in the foreground in the patient who did not have fever, leukocytosis and nuchal rigidity. All markers for autoimmune encephalitis were negative. The sedation of the patient who had no seizure activity under triple antiepileptic therapy was discontinued and he was extubated in a controlled manner. On the 40th day of his hospitalization, he was referred to the neurology clinic for IVIG treatment. The patient, whose IVIG treatment was completed, was discharged. No complaints or seizures were observed in the 6-month follow-up.

NORSE is defined as a clinical condition of resistant, unexplained seizure activity for the first time in a patient without a known history of epilepsy or neurological disorder. Although it is a rare condition, it cannot be controlled with first and second-line antiepileptic agents. It is necessary to follow up in the intensive care unit with intravenous anesthetic agents. Although some patients may have multiple EEG changes, CSF anomalies, and hyperintensity detected in MRI imaging, there is no specific laboratory test or imaging method. For the differential diagnosis, all infectious, inflammatory, metabolic and toxic causes should be detected as negative (4 ).

Autoimmune encephalitis ranks first as a rare cause of SE. The most frequently identified antibodies target the N-methyl-D-aspartate receptor (4–6).

In our case, a 33-year-old postpartum female patient with no previous history of epilepsy was followed up in our intensive care unit and no infective, autoimmune, metabolic or paraneoplastic cause was found as a result of the examinations. A slowdown in activity was observed in EEG, her imaging and CSF examinations were found to be normal.

NORSE is a rare clinical condition that occurs in previously healthy patients, is difficult to manage, often requires multiple intravenous anesthetic treatments, leads to prolonged hospital and intensive care unit stays, but can be cured without any sequelae and good results (4–6) .

1.Epilepsi Tanı ve Tedavi Rehberi,Türk Nöroloji Derneği Yayınları-1 , Galenos Yayınevi, İstanbul, Kasım 2021

2. Gaspard N, Foreman BP, Alvarez V, Cabrera Kang C, Probasco JC, Jongeling AC, Meyers E, Espinera A, Haas KF, Schmitt SE, Gerard EE, Gofton T, Kaplan PW, Lee JW, Legros B, Szaflarski JP, Westover BM, LaRoche SM, Hirsch LJ; Critical Care EEG Monitoring Research Consortium (CCEMRC). New-onset refractory status epilepticus: Etiology, clinical features, and outcome. Neurology. 2015 Nov 3;85(18):1604-13. doi: 10.1212/WNL.0000000000001940. Epub 2015 Aug 21. PMID: 26296517; PMCID: PMC4642147.

3. Sculier C, Gaspard N. New onset refractory status epilepticus (NORSE). Seizure. 2019 May;68:72-78. doi: 10.1016/j.seizure.2018.09.018. Epub 2018 Sep 29. PMID: 30482654.

4. Aurangzeb S, Prisco L, Adcock J, Speirs M, Raby S, Westbrook J, Sen A. New-onset super refractory status epilepticus: A case-series. Seizure. 2020 Feb;75:174-184. doi: 10.1016/j.seizure.2019.10.005. Epub 2019 Nov 9. PMID: 31757748.

5. Mantoan Ritter L, Nashef L. New-onset refractory status epilepticus (NORSE). Pract Neurol. 2021 Mar 5:practneurol-2020-002534. doi: 10.1136/practneurol-2020-002534. Epub ahead of print. PMID: 33674412.

6. Türkan Acar, Sena Boncuk, Bilgehan Acar, Yeşim Güzey Aras. Complete Recovery in Cryptogenic NORSE with Early Immunotherapy: A Case Report. . 2021; 27(1): 79-81

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript.

Patient consent for this study was given verbally.

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