To report a case of refractory anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis with multiple relapses during pregnancy and peripartum with successful use of rituximab during pregnancy and resultant healthy neonatal outcome.
Anti-NMDAR encephalitis is a severe but treatable autoimmune encephalitis which often affects women of childbearing age. The clinical presentation in pregnancy is usually characterized by progressive neuropsychiatric manifestations, seizures and dysautonomia posing a danger to the fetus and a challenge to the treating neurologist. Refractory presentations often need escalation to second-line immunotherapies which have limited safety data in pregnancy.
Case report and literature review.
A 25-year-old primiparous Caucasian woman, 24 weeks pregnant presented with a 3-week history of worsening behavioral/personality changes, confusion, dysarthria, emotional lability and a single generalized tonic-clonic seizure. Brain MRI showed T2/FLAIR hyperintensity in the right frontal lobe suggestive of remote cortical infarct vs cortical dysplasia. CSF showed pleocytosis (WBC 19; 75% lymphocytes) with >10 CSF-restricted oligoclonal bands. Due to high clinical suspicion for an immune-mediated process, she was started on IV methylprednisolone and IVIG. Subsequently, NMDAR antibody returned positive in serum (1:10) and CSF (1:128). Abdominal/pelvic ultrasound and MRI showed no ovarian teratoma. She clinically improved and was discharged, but subsequently relapsed 10 days later at 27 weeks needing treatment with plasma exchange and rituximab, and her symptoms improved. She delivered a healthy baby at 37 weeks but had another relapse immediately post-partum necessitating further immunotherapy. Since then, she has remained stable on maintenance rituximab with no further relapses. At one-year follow up, the baby remains healthy.
This case illustrates the successful use of rituximab during pregnancy for anti-NMDAR encephalitis. Our literature review showed only a handful of such patients treated with rituximab and one patient with cyclophosphamide. Further studies are needed to assess the safety and long-term outcomes of using rituximab for anti-NMDAR encephalitis during pregnancy.