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NMDAR Encephalitis: A Rare Presentation with Bilateral Dermoid Cysts
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Journal of Clinical Case Reports

ISSN: 2165-7920

Open Access

Case Report - (2023) Volume 13, Issue 5

NMDAR Encephalitis: A Rare Presentation with Bilateral Dermoid Cysts

Zahra Azeem* and Evangelia Bakali
*Correspondence: Zahra Azeem, Department of Obstetrics & Gynaecology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK, Email:
Department of Obstetrics & Gynaecology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK

Received: 18-Aug-2023, Manuscript No. jccr-23-110702; Editor assigned: 21-Aug-2023, Pre QC No. P-110702; Reviewed: 01-Sep-2023, QC No. Q-110702; Revised: 08-Sep-2023, Manuscript No. R-110702; Published: 19-Sep-2023 , DOI: 10.37421/2165-7920.2023.13.1577
Citation: Azeem Z, Bakali E. “NMDAR Encephalitis: A Rare Presentation with Bilateral Dermoid Cysts.” J Clin Case Rep 13 (2023): 1577.
Copyright: © 2023 Azeem Z, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

The NMDA receptor is a protein in the brain that helps control thoughts, mood and movements and therefore antibodies against NMDA receptors are likely to have an important role in altering these functions. About 1% of dermoid cysts present with neurological symptoms. This is the case of a young girl was admitted in Critical Care with encephalitis of unknown aetiology and was extensively investigated for the cause. She was found to have small bilateral dermoid cysts following the removal of which she had prompt recovery.

Anti-NMDAR encephalitis is a serious, potentially fatal condition that is often initially confused with schizophrenia spectrum mental illness which leads to inevitable delays in diagnosis and management. Thinking out of the box and involving MDT is the key to management in more complex and rare cases. Dermoid cysts or mature ovarian teratomas should be the first differential in young women presenting with NMDAR encephalitis.

Keywords

NMDA receptor • Brain • Aetiology

Introduction

Dermoid cysts are most common benign neoplasms in young women and are usually asymptomatic or found incidentally on pelvic imaging. They may present with pain and pressure symptoms. However, literature also describes a rare paraneoplastic syndrome associated with Anti-N-methyl-D-aspartate (NMDAR) encephalitis in patients with dermoid cysts; reported incidence being 1.17% [1]. These patients present with psychiatric and neurological symptoms of acute and sub- acute nature which makes it very difficult to differentiate it from organic and psychiatric causes. A study described the most common presentation was psychosis followed by seizures and memory impairment [2]. It is more common in adolescent females [3,4] with the youngest case reported at 4 months [5].

Encephalitis can have either an infectious or autoimmune aetiology. NMDA encephalitis is the most common form of autoimmune encephalitis, caused by the interaction between an antibody and its target, located on glutamate receptor type N-Methyl-D-Aspartate (NMDA) of neuronal surface. An association of encephalitis and ovarian teratomas was first described in 2005 [5], with NMDA receptor antibodies reported in 2007 [6]. Studies suggest that ovarian teratomas are associated with 50% cases of NMDA encephalitis [7].

Although several case reports [8-13] have been described in literature, there is still little awareness about the condition among the gynecologists, who have a major role in the treatment. More cases are published mainly in psychiatric and pediatric journals that have limited access to gynecologists. Therefore, in this case we wanted to highlight the gynecological aspect of the management. Another aspect that makes this case more distinguished is the fact that the patient presented with bilateral dermoid cysts while most of the other cases described in literature are with unilateral dermoid cysts.

Case Presentation

A teenage girl presented to A and E with neuropsychiatric symptoms, acute confusional state and hallucinations. ACT head and lumbar puncture was carried out at the time of presentation which were unremarkable. She was admitted with the suspicion of NMDA encephalitis and started on levetiracetam and I/V methylprednisolone empirically. MRI head was performed as inpatient which was also normal.

One week after admission the patient had an episode of seizures leading to aspiration and was ventilated because of low GCS. In the next three weeks of her stay she was treated with three doses of methylprednisolone followed by a maintenance Prednisolone 60 mg. She also had 5 cycles of plasma exchange. However, she did not make adequate progress. Moreover, she also developed hospital acquired pneumonia and upper limb DVT as additional co morbidities and had a tracheostomy. The neurology team started her on I/V immunoglobulins. A whole body CT scan was carried out and no other pathology was found, except for bilateral dermoid cysts. Opinion from the gynecology team was sought and after gynae-oncology MDT discussion a laparoscopic bilateral cystectomy was recommended. Tumor markers were sent which normal apart from LDH which was 56 (slightly raised) Figure 1.

clinical-case-reports-laparoscopy

Figure 1. The pictures were taken at laparoscopy. (a) Panaromic view of pelvis, (b) Left ovarian dermoid, (c) Left ovarian dermoid and (d) Right ovarian dermoid.

Differential diagnosis

Acute psychotic disorder: Patient presented with sudden onset of altered behaviour and visual hallucinations. She had been visualizing demons before onset of her altered behaviour to her mother. On admission initially diagnosis was psychosis but as the investigations progressed it was gathered that it was encephalopathy of autoimmune origin.

Infective encephalitis: Most of the patients that present with acute onset symptoms have an infective aetiology and therefore all the investigations and therapy is directed towards treating an infective cause unless ruled out.

Treatment

On the 44th day of her admission she underwent uncomplicated laparoscopic bilateral cystectomies. The histopathology report confirmed mature teratomas containing neurological tissue.

Outcome and follow-up

Patient started to recover soon after the operation regaining responsiveness. She is currently under joint care of neurology and rehabilitation team to help her recover from the residual disease.

Results and Discussion

NMDA encephalitis is a well reported condition with a proven association with ovarian teratomas. However, because of diagnostic difficulty and variation in presentation there is a significant delay in treatment which increases the morbidity in these patients. Detailed investigations of CSF and serum should be performed to rule out infections, metabolic and autoimmune causes. Imaging studies should also be performed to identify any tumors such as teratomas. Five cases have been reported with bilateral ovarian teratomas in the literature so far [14-18]. All of these patients have made good recovery following removal of the dermoid cysts and residual disease has not been reported in them.

It has been estimated that 25% of patients with anti-NMDAR encephalitis experience permanent neuropsychiatric debilitation or death even when they receive mainstay treatment. Relapse is estimated to occur in 15% to 24% of patients and is more common in individuals who do not have underlying tumours. Nonetheless, approximately 75% of patients with anti-NMDAR encephalitis recover or have mild sequelae [8]. In some cases it has taken up to a year to reach a diagnosis [13] with the average presentation to surgical removal reported as 74 days [6]. Anti-NMDA antibody encephalitis should not be ignored by gynaecologists whose role in management is central [19].

Conclusion

NMDAR encephalitis is a potentially lethal form of encephalitis associated with ovarian teratomas. A very low threshold of pelvic imaging should be considered in patients presenting with NMDAR positive encephalitis and early referral to gynae must be sought to improve prognosis of the patient. Robust pathways for diagnosis and early MDT involvement will ensure a better outcome.

References

  1. Yaguchi, Hiroaki, Takahiro Tsuji, Ichiro Yabe and Emi Hirayama, et al. "Incidence of anti-NMDAR encephalitis in patients undergoing resection of ovarian teratoma in a single institution." J Neurol Sci 409 (2020): 116608.
  2. Google Scholar, Crossref, Indexed at

  3. Xu, Qianhui, Yong Zhu, Qian Wang and Jing Han, et al. "Clinical features, treatments and outcomes of patients with anti-N-methyl-D-aspartate encephalitis-a single-center, retrospective analysis in China." Front Biosci Landmark 26 (2021): 496-503.
  4. Google Scholar, Crossref

  5. Giri, Yam R, Ijendu Korie, Seema Hashmi and Allison Parrill, et al. "Anti-NMDA receptor encephalitis masquerades as psychosis: A case report." J Psychiatr Prac 28 (2022): 72-77.
  6. Google Scholar, Crossref, Indexed at

  7. Sabbula, Bhanu R, Shravya Yemmanur, Raghavendra Sanivarapu and Deepthi Kagolanu, et al. "Finding the cause of psychosis: A challenging case of anti-NMDAR encephalitis." Case Rep Med 20 (2020): 2-4.
  8. Google Scholar, Crossref

  9. Zheng, Jing, Jinfeng Shen, Aiping Wang and Lingjuan Liu, et al. "Clinical characteristics of anti-N-methyl-d-aspartate receptor encephalitis in children." J Central South Univ Med Sci 45 (2020): 47-54.
  10. Google Scholar, Crossref

  11. Acién, Pedro, Maribel Acién, Eva Ruiz-Maciá and Carlos Martín-Estefanía. "Ovarian teratoma-associated anti-NMDAR encephalitis: A systematic review of reported cases." Orphanet J Rare Dis 9 (2014): 1-9.
  12. Google Scholar, Crossref, Indexed at

  13. Koenig, Zachary A, Joel B. Yednock and James Lewis Akers. "Anti-N-methyl-D-aspartate receptor encephalitis as a paraneoplastic presentation of mature ovarian teratoma." Am J Case Rep 22 (2021): e933240-1.
  14. Google Scholar, Crossref, Indexed at

  15. Chahal, Khushminder, Tara Endeman, Sarah Scapinello and Michal Sapieha. "Anti-N-methyl-D-aspartate receptor encephalitis presenting as atypical psychosis in multiple sclerosis: A case report." BMC Psychiatry 21 (2021): 1-6.
  16. Google Scholar, Crossref, Indexed at

  17. Câmara-Pestana, Pedro, Andreia D. Magalhães, Tiago Mendes and Pedro Levy, et al. "Anti-NMDA receptor encephalitis associated with an ovarian teratoma presenting as first-episode psychosis: A case report." J Psychiatric Practice 28 (2022): 84-88.
  18. Google Scholar, Crossref, Indexed at

  19. Sarmiento, Robert Joseph C, Jose Danilo B. Diestro, Athena Kate D. Antonio and Mario B. Prado, et al. "Neuroleptic malignant syndrome in a patient with anti-N-methyl-D-aspartate receptor encephalitis: Case report and review of related literature." Neurohospitalist 12 (2022): 80-85.
  20. Google Scholar, Crossref, Indexed at

  21. Begum, Jasmina, Zaheda Aziz, Subrat Kumar Sahoo and Rishila Majumder, et al. "Anti-N-Methyl-D-Aspartate receptor encephalitis associated with mature ovarian teratoma in a young adolescent: A case report." J Pediatr Adolescent Gynecol 35 (2022): 400-403.
  22. Google Scholar, Crossref, Indexed at

  23. Lee, Jisun, Seongwoo Kang, Hye Jin Chang and Yong Hee Lee, et al. "Anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma in Korea: Three case reports." Yeungnam Univ J Med 38 (2021): 350-355.
  24. Google Scholar, Crossref, Indexed at

  25. Chetram, Deandra Kimberly, Kelsey Pan, Aisha Elfasi and Merry Markham. "Anti-NMDAR encephalitis presenting after immature teratoma resection." BMJ Case Reports CP 14 (2021): e244637.
  26. Google Scholar, Crossref

  27. Sarmiento, Robert Joseph C, Jose Danilo B. Diestro, Athena Kate D. Antonio and Mario B. Prado, et al. "Neuroleptic malignant syndrome in a patient with anti-N-methyl-D-aspartate receptor encephalitis: Case report and review of related literature." Neurohospitalist 12 (2022): 80-85.
  28. Google Scholar, Crossref, Indexed at

  29. Li, Wenchen, Dan Jia, Lan Tong and Zhijun Lun, et al. "Anti-N-methyl-D-aspartate receptor encephalitis induced by bilateral ovarian teratomas with distinct histopathologic types: A case report and brief literature review." Med 98 (2019): e18148.
  30. Google Scholar, Crossref, Indexed at

  31. Uchida, Yuto, Daisuke Kato, Yoriko Yamashita and Yasuhiko Ozaki, et al. "Failure to improve after ovarian resection could be a marker of recurrent ovarian teratoma in anti-NMDAR encephalitis: A case report." Neuropsychiatr Dis Treat (2018): 339-342.
  32. Google Scholar, Crossref, Indexed at

  33. Lee, Kuo-Wei, Li-Min Liou and Meng-Ni Wu. "Fulminant course in a patient with anti-N-methyl-D-aspartate receptor encephalitis with bilateral ovarian teratomas: A case report and literature review." Medicine 97 (2018).
  34. Google Scholar, Crossref, Indexed at

  35. Pattanayak, Puskar and Lilja B. Solnes. "Paraneoplastic syndrome with anti-NMDAR encephalitis associated with ovarian teratomas." Clin Nuclear Med 42 (2017): e128-e129.
  36. Google Scholar, Crossref, Indexed at

  37. Alžběta, Benická, Pilka Radovan and Flodr Patrik. "Autoimmune anti-N-methyl-D-aspartate receptor encephalitis-paraneoplastic syndrome of ovarian teratoma." Ceska Gynekologie 86 (2021): 397-399.
  38. Google Scholar, Crossref

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