Open Access ISSN: 2329-6895 Journal of Neurological Disorders Case Report Volume 8:3, 2020 DOI: 10.37421/J Neurol Disord.2020.8.422 New-Onset Super-Refractory Status Epilepticus Following Fever: More than a Case of NORSE Abstract We present the case of a healthy 32-year-old woman who came to our hospital due to fever and left otalgia. She subsequently developed dizziness and gait instability, opsoclonus-myoclonus syndrome, and altered level of consciousness. She was admitted to the intensive care unit, developing status epilepticus, which was refractory to third-line treatment with propofol and barbiturates. A thorough assessment including autoimmunity studies, viral testing, and mitochondrial disease testing yielded negative results. A brain magnetic resonance imaging scan revealed signal hyperintensities in both caudate nuclei and putamina, and gadolinium-enhancing small punctiform lesions in the left hemisphere and left cerebellar tonsil. Suspecting an immune-mediated disorder, we started treatment with high-dose steroids and plasmapheresis, in addition to different combinations of antiepileptic drugs. The patient was refractory to these treatments; electroconvulsive therapy improved the EEG tracing and may have helped manage status epilepticus. Subsequent examinations revealed paralysis of left cranial nerves IX, X, and XII, which resolved nearly completely in the final days of hospitalisation, leaving the patient practically asymptomatic. Our findings point to encephalitis with brainstem involvement and manifesting as cryptogenic new-onset refractory status epilepticus. Keywords: Electroconvulsive therapy • Encephalitis • NORSE • Opsoclonus-myoclonus • Refractory status epilepticus. Alicia Hernando-Asensio 1 , María Asunción Martín-Santidrián 1 , Jesús Macarrón-Vicente 1 , Ana Isabel Gómez-Menéndez 2 , Beatriz García-López 2 , Mónica Bártulos-Iglesias 1 and Daniel Pascual-Carrascal 1 1 Neurology Department, Hospital Universitario de Burgos, Burgos, Spain 2 Neurophysiology Department, Hospital Universitario de Burgos, Burgos, Spain *Address for Correspondence: Alicia Hernando-Asensio, Neurology Department, Hospital Universitario de Burgos, Burgos, Spain, Tel: +34 620192357; E-mail: ahernandoas@saludcastillayleon.es Copyright: © 2020 Hernando-Asensio A, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited Received 13 May, 2019; Accepted 20 May, 2020; Published 27 May, 2020 Introduction Status epilepticus (SE) is a common neurological emergency requiring early diagnosis and treatment [1]. In up to 40% of cases, SE is refractory to first- and second-line treatment [2,3]. Some of these refractory cases are healthy patients with no history of epilepsy or other relevant conditions. This type of SE has recently been termed new-onset refractory status epilepticus (NORSE) and is generally associated with a poor prognosis [4]. However, favourable outcomes have been reported in some cases. The aetiology of NORSE often remains unknown; infectious, autoimmune, or genetic causes may be underdiagnosed [5]. There is no specific treatment for NORSE. Anecdotally, it has been suggested that immunomodulatory therapy may be effective in these patients, especially when administered early [6-8]. Such other treatments as hypothermia, the ketogenic diet, surgery, vagus nerve stimulation, and electroconvulsive therapy (ECT) have been used in some patients; however, there is insufficient evidence for conclusions to be drawn regarding their effectiveness and safety [9-13]. We present a case of NORSE with favourable outcome in the context of probable encephalitis associated with brainstem involvement and no identifiable cause. Case Report Our patient was a 32-year-old woman with a 5-day history of left otalgia and fever (39°C). She was diagnosed with otitis media and started antibiotic treatment with amoxicillin/clavulanic acid. Otalgia improved, but the patient returned to our hospital due to dizziness, nausea, and vomiting, and was admitted to the emergency department for further testing. On the same day she was admitted, she began to present gait instability and involuntary eye movements. The neurological examination revealed a fluctuating level of consciousness, opsoclonus-myoclonus, generalised hyperreflexia, and ataxic gait. An emergency head CT scan revealed no alterations and a CSF analysis yielded normal results. The patient was admitted to the intensive care unit (ICU) due to oxygen desaturation and increased drowsiness. Suspecting rhombencephalitis, we started antibiotic treatment with ceftriaxone, ampicillin, gentamicin, and corticosteroids. Several hours after admission to the ICU, the patient presented simple focal motor seizures; according to the EEG, the irritative zone was located in the right frontocentral region. Focal motor seizures progressed to multifocal seizures associated with altered level of consciousness, that is, multifocal status epilepticus (Figures 1 and 2). The patient received pharmacological treatment for SE, including valproic acid, levetiracetam and lacosamide, displaying no improvement; she was subsequently treated with phenytoin, clonazepam, and low-dose propofol infusion. Midazolam and ketamine were then added. Lack of response to treatment led us to administer thiopental. After good initial response, associated with a burst-suppression pattern, video-EEG revealed marked, diffuse involvement, associated with a discontinuous burst-suppression pattern and bilateral, independent signs of irritation. She also received high- dose intravenous methylprednisolone (1 g for 3 days), immunoglobulins (0.4 mg/kg/day for 5 days), and plasmapheresis (5 days). Due to lack of response, we decided to administer ECT. The patient received 2 sessions, each delivering 3 pulses at increasing doses (700 to 1000 mC). Additionally, 500 mg suxamethonium chloride and 400 mg caffeine were administered intravenously prior to the treatment to increase its effectiveness. ECT caused no seizures but was associated with marked improvements in EEG pattern.