Abstract Details

Title Tocilizumab in New Onset Refractory Status Epilpeticus (NORSE) - A Report of the First Case from India.

Topic Autoimmune Neurology

Presentation(s) P9 - Poster Session 9 (12:00 PM-1:00 PM)

Poster/Presentation
Number 15-004

Objective We report a case of cryptogenic super-refractory New Onset Refractory Status Epilepticus (NORSE) treated with Tocilizumab, yielding a favourable outcome in our patient.

Background NORSE is a debilitating condition with significant mortality and morbidity despite treatment.Cytokine storm, especially mediated by IL-6 is an important pathophysiologic mechanism in cryptogenic NORSE. Thus, tocilizumab, an anti IL-6 monoclonal antibody is an appealing immunomodulatory therapy in cryptogenic NORSE.

Design/Methods Case report

Results A 19 year old gentleman presented with history of headache for two days followed by seizures.Seizures became refractory within 24 hours of onset.He was extensively evaluated. MRI showed bilateral putaminal T2/FLAIR hyperintensities.CSF analysis including pan-viral PCR,pan-fungal PCR and autoimmune encephalitis panel done twice over one week was unremarkable. Seizures in the ICU were highly recurrent and of multiple semiologies including GTCS,left faciobrachial seizures and generalized myoclonic jerks. Continuous EEG monitoring showed generalized theta range slowing of the background with highly recurrent generalized and multifocal epileptic discharges.Epidemiology-based Mortality Score in Status Epilepticus (EMSE score) was 52.The patient received early and aggressive anticonvulsants including phenytoin,phenobarbitone,valproate,carbamazepine,lacosamide,perampenel,levitiracetam,lamotrigine and topiramate.Immunomodulatory therapy including IV methylprednisolone and IV Immunoglobulin were used.At 62 days of ICU admission,seizures continued despite high doses of intravenous midazolam,thiopentone,ketamine and propofol.Tocilizumab was given at two doses of 200 mg intravenous injection one week apart.Patient's seizures stopped two days after first dose of Tocilizumab.Despite the protracted course of NORSE, patient had a favourable outcome.Presently,at 5 months of follow-up, patient is seizure-free,has subtle memory impairment, no speech problems and no physical disability.He resumed college and is now a marathon runner.

Conclusions Our patient represents the first case report of super-refractory NORSE treated with Tocilizumab in India. Tocilizumab has a time-tested safety record when used in other auto-immune disorders. It can therefore be an effective immunomodulatory therapy in cryptogenic NORSE as it targets cytokine storm, the core underlying pathophysiologic mechanism.

Authors/Disclosures
Manoj Bharath Alluru PRESENTER	No disclosure on file
Ramesh S. Patil, DM (NIMHANS Bangalore)	No disclosure on file
Srikanteshwara Praveen Kumar, MBBS, DM	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
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	No disclosure on file

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Abstract Details