Abstract

The natural long-term course of antibody titers in patients with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis remains uncertain. We measured anti-NMDAR antibodies in a patient with anti-NMDAR encephalitis who had a severe disease course for 5 years. The antibody titers in the first serum and cerebrospinal fluid (CSF) samples were 1:1600 and 1:320, respectively. During gradual recovery, the serum and CSF titers initially decreased, but remained unchanged during the last 2 years. Four years after the onset of disease, the serum and CSF antibody titers were 1:200 and 1:80, respectively. Anti-NMDAR antibodies can persist for prolonged periods in patients with a poor clinical course.

Highlights

  • Paraneoplastic encephalitis is a rare neurological disorder associated with small cell lung carcinomas, lymphomas, thymomas, and testicular tumors

  • In 2007, a specific autoantibody to the N-methyl-Daspartate receptor (NMDAR) related to the paraneoplastic encephalitis associated with ovarian teratoma was identified [2]

  • The serum and cerebrospinal fluid (CSF) anti-NMDAR antibody titers persisted during the prolonged disease course

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Summary

Introduction

Paraneoplastic encephalitis is a rare neurological disorder associated with small cell lung carcinomas, lymphomas, thymomas, and testicular tumors. The 2-year outcomes of 577 patients with anti-NMDAR encephalitis were reported, and second-line immune treatment such as rituximab or cyclophosphamide was recommended for patient’s refractory to firstline therapy [4]. Two of these 3 patients with anti-NMDAR encephalitis received first-line (steroids, plasmapheresis, or IVIG) or second-line immune treatments (immunosuppressants) [7,8].

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