Abstract

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is the most common autoimmune encephalitis. To date, there has been no study on the relationship between antibody (Ab) titers and clinical phenotype. This study aims to clarify the relationship between cerebrospinal fluid Ab titers and clinical manifestations of anti-NMDAR encephalitis at onset. Seventy-six consecutive patients with a definite diagnosis were enrolled. The relationship between Ab titers and different onset symptoms including psychiatric symptoms, seizures, and memory deficits were analyzed. We further investigated the correlation between Ab titers and clinical severity as assessed by the modified Rankin scale (mRS) and the clinical assessment scale for autoimmune encephalitis (CASE), respectively. The Ab titers had a median value of 1:10 (range 1:1–1:100). There was no significant difference in titers among various clinical factors including gender and combination of tumor and other diseases (each p > 0.05). Patients presenting with psychiatric symptoms at onset had higher titers than those with seizures (p = 0.008) and memory deficits (p = 0.003). The mRS scores revealed a significant but weak correlation with Ab titers (r = 0.243, p = 0.034), while CASE scores did not correlate with the titers (p = 0.125). Our findings indicated that the Ab titers were associated with the type of onset symptoms, with a higher level of patients with psychiatric symptoms. Regarding the clinical severity, the titers showed a weak correlation with the mRS, but no correlation with the CASE.

Highlights

  • Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common subtype of autoimmune encephalitis (AE), characterized by a prodromal phase and the subsequent progression of multiple symptoms, involving psychiatric symptoms, seizures, and memory deficits [1,2]

  • In a cohort of anti-NMDAR encephalitis patients in China, we aimed to investigate the clinical implications of cerebrospinal fluid (CSF) Ab titers in terms of disease phenotype and activity

  • Inclusion criteria were: (1) aged ≥ 16 years; (2) cell-based analyses (CBA) for CSF samples at the acute phase of disease before immunotherapy; (3) first attack and definite diagnosis of anti-NMDAR encephalitis using the criteria of Graus et al [5]

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Summary

Introduction

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common subtype of autoimmune encephalitis (AE), characterized by a prodromal phase and the subsequent progression of multiple symptoms, involving psychiatric symptoms, seizures, and memory deficits [1,2]. Immunoglobulin G (IgG) antibodies (Abs) to the NR1 subunit of NMDA-type glutamate receptors play an important role in this pathological process [3]. The detection of these Abs in serum and/or cerebrospinal fluid (CSF) is essential for a definite diagnosis. The increase of CSF titers has a better correlation with clinical relapses (new onset of other symptoms or an increase in the mRS score by over one point) [4]. In a cohort of anti-NMDAR encephalitis patients in China, we aimed to investigate the clinical implications of CSF Ab titers in terms of disease phenotype and activity

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