Ovarian teratoma-associated anti-N-methyl-D-aspartate receptor encephalitis: a clinical analysis of 5 cases
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Abstract
Objectives: To analyse the clinical features, diagnosis, treatment, and prognosis of anti-N-methyl-D-aspartic acid receptor (NMDAR) encephalitis associated with ovarian mature teratomas. Material and methods: Retrospectively analysed the clinical-laboratory data of five patients with anti-NMDAR encephalitis combined with ovarian teratoma at a single centre between March 2016 and June 2019. Results: The mean age of the patients was 22.40 ± 2.89 years (range, 19–26 years). Five patients had premonitory fever symptoms, clinical manifestations of mental disorder or convulsions for starting, with varying degrees of involuntary movement. Brain MRI and electroencephalography lacked specificity, and cerebrospinal fluid resistance NMDAR antibody detection was the key to diagnosis. All patients experienced good outcomes in response to immunotherapy combined with ovarian tumour resection, with a median follow-up time of 36 months (range, 16–55 months). The MRS value of five patients decreased significantly half a year after surgery, and no encephalitis or ovarian tumour relapses were reported. Conclusions: Anti-NMDA encephalitis caused by ovarian teratoma is mostly a non-specific clinical manifestation of neurological and mental abnormalities, which can be easily misdiagnosed and delayed, and doctors should fully recognise the disease, early diagnosis, and timely surgical intervention to improve the prognosis of patients.
Keywords: anti-N-methyl-D-aspartate receptor (NMDAR)autoimmune encephalitisovarian teratomatumour resectionretrospective study
References
- Acién P, Acién M, Ruiz-Maciá E, et al. Ovarian teratoma-associated anti-NMDAR encephalitis: a systematic review of reported cases. Orphanet J Rare Dis. 2014; 9: 157.
- Gresa-Arribas N, Planagumà J, Petit-Pedrol M, et al. Human neurexin-3α antibodies associate with encephalitis and alter synapse development. Neurology. 2016; 86(24): 2235–2242.
- Dalmau J, Graus F. AntibodymMediated encephalitis. N Engl J Med. 2018; 378(9): 840–851.
- Vitaliani R, Mason W, Ances B, et al. Paraneoplastic encephalitis, psychiatric symptoms, and hypoventilation in ovarian teratoma. Ann Neurol. 2005; 58(4): 594–604.
- Dalmau J, Tüzün E, Wu Hy, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007; 61(1): 25–36.
- Titulaer M, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013; 12(2): 157–165.
- Gong S, Zhou M, Shi G, et al. Absence of NMDA receptor antibodies in patients with ovarian teratoma without encephalitis. Neurol Neuroimmunol Neuroinflamm. 2017; 4(3): e344.
- Dalmau J, Lancaster E, Martinez-Hernandez E, et al. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol. 2011; 10(1): 63–74.
- Thomas A, Rauschkolb P, Gresa-Arribas N, et al. Anti-N-methyl-D-aspartate receptor encephalitis: a patient with refractory illness after 25 months of intensive immunotherapy. JAMA Neurol. 2013; 70(12): 1566–1568.
- Dalmau J, Armangué T, Planagumà J, et al. An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. Lancet Neurol. 2019; 18(11): 1045–1057.
- Zhang Le, Wu MQ, Hao ZL, et al. Clinical characteristics, treatments, and outcomes of patients with anti-N-methyl-d-aspartate receptor encephalitis: A systematic review of reported cases. Epilepsy Behav. 2017; 68: 57–65.
- Hughes EG, Peng X, Gleichman AJ, et al. Cellular and synaptic mechanisms of anti-NMDA receptor encephalitis. J Neurosci. 2010; 30(17): 5866–5875.
- Barry H, Byrne S, Barrett E, et al. Anti-N-methyl-d-aspartate receptor encephalitis: review of clinical presentation, diagnosis and treatment. BJPsych Bull. 2015; 39(1): 19–23.
- Graus F, Titulaer M, Balu R, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016; 15(4): 391–404.
- Tanguturi YC, Cundiff AW, Fuchs C. Anti-N-Methyl d-Aspartate receptor encephalitis and electroconvulsive therapy: literature review and future directions. Child Adolesc Psychiatr Clin N Am. 2019; 28(1): 79–89.
- Pham HP, Daniel-Johnson JA, Stotler BA, et al. Therapeutic plasma exchange for the treatment of anti-NMDA receptor encephalitis. J Clin Apher. 2011; 26(6): 320–325.
- Zhang Y, Liu G, Jiang M, et al. Efficacy of therapeutic plasma exchange in patients with severe refractory anti-nmda receptor encephalitis. Neurotherapeutics. 2019; 16(3): 828–837.
- Titulaer M, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013; 12(2): 157–165.
- Seki M, Suzuki S, Iizuka T, et al. Neurological response to early removal of ovarian teratoma in anti-NMDAR encephalitis. J Neurol Neurosurg Psychiatry. 2008; 79(3): 324–326.
- Peery HE, Day GS, Doja A, et al. Anti-NMDA receptor encephalitis in children: the disorder, its diagnosis, and treatment. Handb Clin Neurol. 2013; 112: 1229–1233.
- Mitra AD, Afify A. Ovarian teratoma associated Anti-N-methyl-D-aspartate receptor encephalitis: a difficult diagnosis with a favorable prognosis. Autops Case Rep. 2018; 8(2): e2018019.
