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- W4309682775 abstract "Persistent neutrophilic meningitis has been described in the setting of infections, systemic autoimmune disease, and certain neoplasms. However, we propose relapsing neutrophilic meningoencephalitis (RNME) as a distinct entity, which often remains idiopathic (IRNME) after the initial or subsequent episode. It is important to recognize and treat this disease early to prevent morbidity that accumulates from relapses. We identified five patients meeting criteria for IRNME which we defined as having a clinical presentation of a relapsing meningoencephalitis, multiple cerebrospinal fluid (CSF) analyses consistent with neutrophilic CSF pleocytosis (>25% neutrophils), a negative broad work-up for infectious pathogens and no clear identified underlying systemic or neurologic disease. Clinical presentations most frequently included encephalopathy, headaches, language deficits and seizures. All patients had some degree of persistent cognitive impairment following relapses. Notably 4/4 patients treated with steroids were steroid responsive, and two patients remain relapse free on infliximab and one patient on tocilizumab. IRNME is a distinct clinical syndrome, likely caused by a variety of underlying systemic diseases and often remaining idiopathic after multiple relapses. Many of these patients have features of underlying autoimmune disease, however at presentation they do not meet criteria for a rheumatologic diagnosis. Despite this, it is important to recognize this syndrome, perform an extensive work up, consider starting treatment with steroids and closely monitor these patients for recurrent disease to prevent morbidity. While further study is needed to determine the best long-term treatment in IRNME, our cases show success with infliximab and tocilizumab." @default.
- W4309682775 created "2022-11-29" @default.
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- W4309682775 date "2022-01-01" @default.
- W4309682775 modified "2023-09-30" @default.
- W4309682775 title "Idiopathic relapsing neutrophilic meningoencephalitis – a case series" @default.
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- W4309682775 doi "https://doi.org/10.1016/j.nerep.2022.100153" @default.
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