Anti-NMDA Receptor Encephalitis: Retrospective Analysis of 15 Cases, Literature Review, and Implications for Gynecologists

Abstract

Background. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a rare form of autoimmune encephalitis caused by anti-NMDA receptor antibodies. This disease mainly affects women of childbearing age and is commonly associated with ovarian teratoma. However, the relationship between anti-NMDA receptor encephalitis and ovarian teratoma and the role of anti-NMDA receptor antibody in the relationship remain unclear. Objectives. This study aimed to describe 15 cases of anti-NMDA receptor encephalitis (5 with ovarian teratoma), review literature, and reinforce the gynecologist's knowledge of this disorder. Methods. Clinical data of 15 patients from January 2015 to December 2020 admitted to The Second Hospital of Hebei Medical University were collected and analyzed. The diagnosis of anti-NMDA receptor encephalitis was based on the presence of anti-NMDA receptor antibodies in cerebrospinal fluid (CSF) and/or serum. Laparoscopic teratoma removal was performed in patients with ovarian teratoma. All patients had received immunotherapy. In addition, a review of the literature was performed to reinforce the gynecologist's knowledge of this disorder. Results. A total of 15 patients with anti-NMDA receptor encephalitis were screened, of whom 5 patients were confirmed with ovarian teratoma by pathology. The most common symptoms of anti-NMDAR encephalitis with teratoma are fever (5/5, 100%), seizure (5/5, 100%), mental and behavioral disorders (4/5, 80%), and decreased consciousness (4/5, 80%). Conversely, the most common symptoms of patients without teratoma were neuropsychiatric symptoms, including headache (6/10, 60%) and mental and behavioral disorders (7/10, 70%). All patients underwent immunotherapy, including steroids, intravenous immunoglobulin (IVIG), plasma exchange, and cyclophosphamide, and 4 out of 5 patients with ovarian teratomas underwent surgical treatment. All patients had a good outcome after systemic, surgical, and immunotherapy treatment. No patient who underwent surgical treatment developed a recurrence. Conversely, 2 of 10 patients without teratoma developed an anti-NMDA receptor encephalitis recurrence. Conclusions. Patients with anti-NMDA encephalitis show severe mental and neurological symptoms. Resection of teratoma is beneficial to the relief or disappearance of symptoms and has a good prognosis. This disorder should be fully recognized by gynecologists, who play an important role in diagnosis and treatment.

Canadian Consensus Guidelines for the Diagnosis and Treatment of Autoimmune Encephalitis in Adults

“…173,176 Patients face a higher risk of drug induced movement disorders, worsening agitation, rhabdomyolysis, and neuroleptic malignant syndrome, especially with medications with high affinity for the D2 dopamine receptor, such as haloperidol. 177 If required, atypical antipsychotics are preferred. 172,178 Options are detailed in Table 5 including strategies to target sleep disturbance.…”

The Leading Role of Brain and Abdominal Radiological Features in the Work-Up of Anti-NMDAR Encephalitis in Children: An Up-To-Date Review

“…In a recent case series of both adult and pediatric patients, more than half of them developed one or more of the following: tonic–clonic seizures (79%), focal seizures (74%), focal seizures without impaired awareness (55%), focal seizures with impaired awareness (42%), status epilepticus (35%), and refractory status epilepticus (21%) [ 5 ]. Specifically, fever, seizure, mental and behavioral disorders, and decreased consciousness are most frequently observed in NMDARe patients with an underlying teratoma, whereas neuropsychiatric symptoms and headache have been predominantly described in patients without teratoma [ 21 ]. Several patients may present with a prodromal headache or a viral-like infection state [ 4 , 6 ].…”

“…According to a recent review of the literature, the majority of patients with (150/155) who underwent surgical intervention had a favorable disease course [ 55 ]. Laparoscopic teratoma removal is widely performed on patients with NMDARe [ 21 ]. Encephalitis with no associated tumor and adolescent onset are considered the two most important risk factors for relapse, which is commonly observed in 15–24% of patients, even after several years [ 56 ].…”

Anti-NMDAR antibodies, the blood–brain barrier, and anti-NMDAR encephalitis

“…The presence of nervous tissues in ovarian teratomas may play a role in the pathogenesis of anti-NMDAR encephalitis. The resection of teratomas is beneficial for the relief of symptoms in patients ( 44 ). The controversy surrounding the role of anti-NMDAR antibodies and the impact of the peripheral immune system on anti-NMDAR encephalitis is a topic that requires further investigation.…”