Abstracts

Rationale:
NORSE (New-onset refractory status epilepticus) is a recently described condition, with undetermined etiology in about 50% of cases. Acute treatment includes antiepileptic drugs (AED) and immunomodulatory agents (IA). Long term outcomes include refractory epilepsy (RE) and cognitive impairment. We aim to describe findings in additional NORSE cases,emphasizing clinical,MRI and CSF findings,long-term outcome and comorbidities,to contribute to the understanding of this rare condition.
Method:
Retrospective analysis of 5 NORSE cases. We evaluated presence of fever, acute treatment, neuroimaging and CSF findings, AED and IA use, personal and family history (FH) of immune conditions and long-term outcome.
Results:
Case 1–JVO, 10 year-old with coma, followed by recurrent convulsive seizures, preceded by fever. FH is remarkable for Systemic Lupus Erythematosus (SLE) and antiphospholipid syndrome (father), and positive ANA in JVO. Treated with AED drip, IV steroids and rituximab, had a severe allergic reaction (DRESS syndrome). CSF PCR was positive for enterovirus. After 5 years,the patient maintains weekly seizures and has mild cognitive impairment. Case 2–7 year-old girl with an autoinflammatory condition. 3 weeks prior to NORSE, she had fever, decreased level of consciousness and recurrent convulsive seizures. CSF showed 70 cells. MRI showed non-specific acute lesions. She was treated with IV AED drip and pulse steroids. The patient maintains refractory focal dysperceptive seizures, and has memory and attentional deficits. Case 3–11 year old boy was found unresponsive, followed by recurrent convulsive seizures, preceded by fever. He received continuous intravenous AEDs. MRI showed bilateral fronto-temporal lesions. CSF showed 85 cells. The paternal grandmother has SLE. Father and grandfather had lymphoma. RE was treated with AEDs, VNS, IVIG and pulse IV steroids. He has academic difficulties and psychotic symptoms. Case 4–20 year old man found unresponsive, followed by a convulsive seizure, preceded by fever. CSF showed “inflammatory changes”. Treated with IV AED drip, and pulse IV steroids. Developed long term RE (treated with AEDs, VNS, IV steroids and IVIG). Developed low B cell count, and had an allergic reaction (DRESS syndrome). He dropped out of College due to academic difficulties. Case 5–22 year-old found unresponsive and febrile. Admitted to ICU with non- convulsive RSE. Received AEDs, IVIG and underwent plasma-exchange therapy. CSF showed a mild pleocytosis. She had a history of Hashimoto’s thyroiditis. She developed occasional focal dysperceptive seizures and is planning to return to College.
Conclusion:
We described cases of NORSE preceded by fever (FIRES),4 of which cryptogenic. Comorbidities were remarkable for autoimmune diseases, severe allergic reactions in 2 and low B cell count in 1. All patients developed recurrent seizures (RE in 4),and 4 of them developed academic difficulties.The role of inflammatory mediators in the brain of these patients should be further studied, as well as the potential use of monoclonal antibodies directed to inflammatory targets.
Funding:
:No funding received