A case of very prolonged new onset refractory status epilepticus (NORSE) with no evidence of autoimmune activation and a
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LETTER TO THE EDITOR
A case of very prolonged new onset refractory status epilepticus (NORSE) with no evidence of autoimmune activation and a good neurological recovery Monica Ferlisi 1
&
Elena Greco 1 & Tiziano Zanoni 1 & Monica Zamagni 2 & Marilena Casartelli Liviero 3 & Paolo Zanatta 3
Received: 21 February 2020 / Accepted: 9 May 2020 # Fondazione Società Italiana di Neurologia 2020
To the Editor, New onset refractory status epilepticus (NORSE) is a rare condition characterized by the occurrence of a status epilepticus (SE) that is often prolonged and refractory to therapies, without any identifiable cause, in a non-epileptic patient [1]. Because an autoimmune cause is often suspected, the suggest therapy for NORSE includes steroids, intravenous immunoglobulins (IVIg), and plasma exchange [2]. We report the case of a 28-year-old woman who developed fever with generalized seizures. The electroencephalogram (EEG) showed bilateral sharp waves. Neurological examination, computerized tomography (CT), and magnetic resonance imaging (MRI) of the brain were normal. The patient’s history was unremarkable, except for a mild form of Axenfeld-Rieger syndrome (ARS), a rare genetic disorder characterized by dysgenesis of the anterior segment of the eye, craniofacial dysmorphism, dental, cardiac, and umbilical anomalies [3]. She had no history of epilepsy. A recent cat scratch was reported. Repeated lumbar puncture was negative for organisms; also negative were polymerase chain reaction (PCR) assay for viruses (herpes simplex 1-2, zoster, cytomegalovirus, West Nile virus, enterovirus, mycoplasma, parechovirus), bacterial and microscopic culture, and onconeural and surface antibody testing (Lgl1, CASPR2, NMDAR, GLyR, AMPAR1-2,
* Monica Ferlisi [email protected] 1
Unit of Neurology A, Ospedale Civile Maggiore, Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy
2
Neurology Department, Ospedale S. Maria della Misericordia, Rovigo, Italy
3
Department of Anaesthesia and Intensive Care, Azienda Ospedaliera Universitaria Integrata, Verona, Italy
GABABR, GAD65, DPPX, mGluR5). Rheumatological screening, lactic acid, monoclonal component, and antimitochondrial antibodies were negative. High-dose steroid pulse therapy for suspicion of autoimmune encephalitis was initiated. Seizures continued despite the administration of very high-dose IV antiepileptic therapy (levetiracetam and vaproate). The patient was admitted to the neurointensive care unit and treatment with continuous intravenous anesthetic drugs (CIVADs) was started (propofol and ketamine, then thiopental). Seizures recurred despite repeated attempts to decrease the depth of anesthesia. Hypothermia, plasmapheresis, and IVIg therapy brought no benefit. An EEG at that time showed an iatrogenic burst-suppression pattern, with isoelectric activity intercalated by epileptic bursts of prolonged, bilateral high-voltage polyspikes (Fig. 1a). Depending on the depth of anesthesia, electroclinical seizures arising from the right hemisphere were also recorde
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