Systemic lupus erythematosus associated with recurrent anti-NMDA receptor encephalitis during pregnancy

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Systemic lupus erythematosus associated with recurrent anti-NMDA receptor encephalitis during pregnancy Shujiang Zhang 1 & Yuan Yang 1 & Ting Long 1 & Zuoxiao Li 1 Received: 30 October 2019 / Accepted: 3 November 2020 # Springer-Verlag GmbH Austria, part of Springer Nature 2020

Abstract Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is caused by autoantibodies against the NR1 subunit of NMDAR. Recurrent cases combined with systemic lupus erythematosus (SLE) during pregnancy have not been reported. We report the case of a 23-year-old woman with a past history of SLE who presented with the characteristic features of anti-NMDAR encephalitis during both of her two pregnancies. Keywords Systemic lupus erythematosus (SLE) . Anti-N-methyl-D-aspartate receptor encephalitis . Pregnancy . Relapse

Introduction

Case report

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was first reported in 2005. The underlying pathophysiology has not been fully elucidated, although the condition is principally associated with antibodies against the NR1 subunit of the NMDAR (Dalmau et al. 2011). The typical clinical manifestations of the disease are disturbed consciousness, psychiatric symptoms, seizures, and dyskinesia. Almost 50% of cases in young women are teratoma-associated. Several cases of anti-NMDAR encephalitis have been reported during pregnancy, some of them with concomitant autoimmune diseases such as hyperthyroidism or rheumatic arthritis (Lu et al. 2015). Cases of anti-NMDAR encephalitis associated with SLE and symptoms of anti-NMDAR encephalitis recur during pregnancy have not previously been reported.

A 23-year-old pregnant woman presented to the hospital with acute psychiatric symptoms and complex partial seizures. She had a history of anti-NMDAR encephalitis that showed similar symptoms during a previous pregnancy at 8 weeks of gestation 2 years prior. The psychiatric symptoms including visual hallucinations were characterized by seeing the crawling of animals on wall and persecutory delusion, and her yelling and screaming in the ward. She had never been pregnant before. She also had a history of systemic lupus erythematosus (SLE) and had been taking prednisone (15 mg/day) and hydroxychloroquine (0.4 g/day) since her SLE diagnosis at the age of 19. She had a long-term pancytopenia since she was diagnosed with SLE. Her first pregnancy occurs at the stable stage of SLE, when she was diagnosed with anti-NMDAR encephalitis during her first pregnancy; she received a 5-day course of intravenous pulse methylprednisolone (1 g/day) and intravenous immunoglobulin initially, methylprednisolone reduced to 0.5 g for 3 days; took 50 mg/day prednisone orally, reduced by 5 mg every 2 weeks; and subsequently took maintenance dose of 15 mg/day throughout the pregnancy. During the first pregnancy, her symptoms responded well to immunotherapy. The psychiatric symptoms disappeared 40 days after onset. She gave birth to a healthy boy and fully recovered without any sequelae of anti-NMDAR encephaliti