Successful treatment of anti-NMDA receptor encephalitis with early teratoma removal and plasmapheresis

Abstract

RationaleThis report describes a Successful treatment of anti-NMDA receptor encephalitis with early teratoma removal and plasmapheresis.Patient ConcernsWe present a 31-year-old Caucasian nulliparous patient who was admitted as an emergency with general illness status accompanied by holocranial cephalalgia and fever.DiagnosesThe previous symptoms were followed by disorientation, persecutory delusion, incoherent language, and tonic-clonic seizure.InterventionsThe patient was admitted in the intensive care unit (ICU) with Glasgow score 7.OutcomesMost of complementary exams (brain CT, brain MRI, blood analysis, PCR for virus on CSF) were normal except CSF leucocytosis and hyperproteinorrhachia. An abdominopelvic ultrasound revealed a 5-cm solid-cystic tumor in the left adnexal region, suggestive of teratoma. At that stage, the possibility of autoimmune encephalitis was considered, and confirmed later.LessonsThis disease can only be successfully treated with fast surgical intervention and an early implementation of immunosuppressive therapies. The optimal timing of initiation and duration of therapeutic plasma exchange necessary to achieve good outcomes in patients with NMDAR remains unknown. This case report intends to increase awareness about the importance of early surgical treatment and early implementation of this potentially life-saving therapy and of continuing the treatment until complete remission of symptoms.

Clinical approach to the diagnosis and successful management of anti-NMDA receptor encephalitis when antibody testing is not possible

“…This patient’s clinical presentation did suggest anti-NMDA receptor encephalitis as a differential diagnosis. In this scenario, an underlying ovarian lesion should be sought as a potential target for treatment 1–7. The necessary imaging investigations should be performed in a timely manner as recovery can be prolonged if the disease proceeds unchecked 3.…”

N-Methyl-D-Aspartate Receptor Encephalitis with Psychiatric Symptoms and an Ovarian Teratoma Detected by MRI in a 17-Year-Old Girl

“…Since timely initiation of treatment in autoimmune mediated encephalitis is associated with a better outcome, 3,5,8,9 we decided to start treatment even before having received the autoantibody results on the basis of the rapid and severe onset of symptoms and the mildly elevated CSF cell count. This approach and the escalation of treatment with rituximab lead to sustained improvement and an excellent outcome and may have been the reason why our patient did not develop further neurological symptoms.…”

“…1,2,4,7,8 Early initiation of therapy is associated with a better prognosis. 3,5,8,9 First-line therapy consists of intravenous (IV) glucocorticoids, IV immunoglobulins (IVIg), or plasmapheresis, followed in severe or relapsing cases by rituximab and/or cyclophosphamide. [2][3][4][5]9,10 In the presence of an ovarian teratoma, removal is strongly recommended.…”

“…3,5,8,9 First-line therapy consists of intravenous (IV) glucocorticoids, IV immunoglobulins (IVIg), or plasmapheresis, followed in severe or relapsing cases by rituximab and/or cyclophosphamide. [2][3][4][5]9,10 In the presence of an ovarian teratoma, removal is strongly recommended. 2,3,5,[8][9][10] Approximately three of four patients recover or have only mild neurological sequelae.…”

“…[2][3][4][5]9,10 In the presence of an ovarian teratoma, removal is strongly recommended. 2,3,5,[8][9][10] Approximately three of four patients recover or have only mild neurological sequelae. Up to 20% of patients continue to have severe neurological symptoms.…”