Abstract

Intrathecal antibody production is thought to underly the pathogenesis and symptomatology of N-methyl-D-aspartate receptor encephalitis (NMDARE). In the present study, the clinical correlation of cerebrospinal fluid (CSF) restricted oligoclonal bands (OCBs), as a measure of intrathecal antibody synthesis, was examined in confirmed NMDARE cases. The present study included patients with a confirmed diagnosis of NMDARE who underwent initial CSF evaluation and were followed up for a minimum of 12 months. Disease severity was assessed at baseline and 1, 3, 6, 9, and 12 months. Data regarding duration of hospitalization and intensive care unit (ICU) stay, the presence of uncontrolled seizures, and antiepileptic drug requirement were obtained for each patient. Among the 14 confirmed NMDARE patients, seven had CSF-OCBs. The presence of CSF-OCBs was associated with a more severe disease at baseline (p = 0.004), worse final outcome (p = 0.005), and longer hospitalization (median, 19 vs. 173 days; p < 0.001) and ICU stay (median, 0 vs. 29 days; p = 0.006). CSF-OCB positivity was closely associated with treatment refractoriness within 4 weeks (p = 0.029). The presence of CSF-OCBs at the onset of disease in NMDARE patients was associated with initial treatment refractoriness and a more severe disease course leading to longer hospitalization, ICU admission, intractable seizures, and a poorer outcome. The results indicate that CSF-OCBs may be useful for prognostication. Furthermore, severe disease in NMDARE may be accompanied by oligoclonal expansion antibody-producing B cells.

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