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

Abstract Background Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disorder which often presents with neuropsychiatric symptoms. A large proportion of cases are associated with an identifiable tumor, most commonly ovarian teratoma. However, recent literature has also described an overlap of anti-NMDAR encephalitis and demyelinating syndromes. Cases have been reported of anti-NMDAR encephalitis in patients with ADEM, optic neuritis, myelitis and multiple sclerosis. This link is considered rare, however has important clinical implications as treatments and prognosis may differ. Case presentation A 33-year-old female with a history of multiple sclerosis presented with new-onset neuropsychiatric symptoms. After substance-induced psychosis was ruled out, she was admitted to the medical ward for work up of psychosis secondary to multiple sclerosis. However, the consultation-liaison psychiatry service noted atypical symptoms which were concerning for autoimmune encephalitis. Admission to a psychiatric inpatient ward was deferred. Anti-NMDAR encephalitis was diagnosed with CSF analysis demonstrating lymphocytic pleocytosis and anti-NMDAR antibodies. In addition to first-line treatment of encephalitis with steroids, second-line immunotherapies were also implemented given the patient’s underlining demyelinating syndrome. The patient’s neurologic and psychiatric symptoms began to improve. Conclusions There is literature to demonstrate a possible connection between anti-NMDAR encephalitis and demyelinating syndromes. As such, autoimmune encephalitis should be considered in patients with multiple sclerosis presenting with atypical symptoms. Determining the correct diagnosis is crucial to inform the appropriate treatment protocol, and to improve prognosis.

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Anti-N-methyl-D-aspartate receptor encephalitis and ovarian teratomas: is there any association?

Voprosy ginekologii akušerstva i perinatologii ◽

10.20953/1726-1678-2020-5-124-131 ◽

2020 ◽

Vol 19 (5) ◽

pp. 124-131

Author(s):

N.M. Podzolkova ◽

◽

V.V. Korennaya ◽

O.S. Levin ◽

E.E. Vasenina ◽

...

Keyword(s):

Pathological Condition ◽

Rare Complication ◽

Autoimmune Encephalitis ◽

Neurological Deficits ◽

Ovarian Teratoma ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Life Threatening ◽

Causal Treatment ◽

Ovarian Teratomas

This article aims to explore the problem of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis and also describes our own experience in treating anti-NMDAR encephalitis in patients with ovarian teratomas. This pathological condition is a rare complication of cancer. Practicing gynecologists are often unfamiliar with it, although its consequences can be life-threatening. Early diagnosis and causal treatment, particularly teratoma removal, are critical for the outcome and can prevent death or the development of neurological deficits in patients. Key words: anti-NMDAR, autoimmune encephalitis, ovarian cysts, complications, ovarian teratoma, encephalitis, encephalitis in women

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The diffuse involvement of anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

BMC Neurology ◽

10.1186/s12883-019-1456-6 ◽

2019 ◽

Vol 19 (1) ◽

Cited By ~ 1

Author(s):

Yun Jiang ◽

Jianpeng Ma ◽

Tao Gong ◽

Hongjun Hao ◽

Haibo Chen

Keyword(s):

Basal Ganglia ◽

Brain Mri ◽

Clinical Manifestations ◽

Autoimmune Encephalitis ◽

Ovarian Teratoma ◽

Mass Effects ◽

Glucose Levels ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. Anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special case of an anti-NMDAR encephalitis who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, as well as the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions Anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. Recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

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Anti-NMDA Receptor Encephalitis Presenting as an Acute Psychotic Episode in a Young Woman: An Underdiagnosed yet Treatable Disorder

Case Reports in Psychiatry ◽

10.1155/2014/868325 ◽

2014 ◽

Vol 2014 ◽

pp. 1-3 ◽

Cited By ~ 6

Author(s):

Shikma Keller ◽

Pablo Roitman ◽

Tamir Ben-Hur ◽

Omer Bonne ◽

Amit Lotan

Keyword(s):

Nmda Receptor ◽

Cognitive Rehabilitation ◽

Psychiatric Symptoms ◽

Immunosuppressive Treatment ◽

Autoimmune Encephalitis ◽

Memory Deficits ◽

Ovarian Teratoma ◽

Psychotic Episode ◽

Acute Psychosis ◽

Nmdar Encephalitis

Anti-NMDA receptor (NMDAR) encephalitis is a recently identified autoimmune disorder with prominent psychiatric symptoms. Patients usually present with acute behavioral change, psychosis, catatonic symptoms, memory deficits, seizures, dyskinesias, and autonomic instability. In female patients an ovarian teratoma is often identified. We describe a 32-year-old woman who presented with acute psychosis. Shortly after admission, she developed generalized seizures and deteriorated into a catatonic state. Although ancillary tests including MRI, electroencephalogram, and cerebrospinal fluid (CSF) analysis were unremarkable, the presentation of acute psychosis in combination with recurrent seizures and a relentless course suggested autoimmune encephalitis. The patient underwent pelvic ultrasound which disclosed a dermoid cyst and which led to an urgent cystectomy. Plasmapheresis was then initiated, yielding partial response over the next two weeks. Following the detection of high titers of anti-NMDAR antibodies in the CSF, the patient ultimately received second line immunosuppressive treatment with rituximab. Over several months of cognitive rehabilitation a profound improvement was eventually noted, although minor anterograde memory deficits remained. In this report we call for attention to the inclusion of anti-NMDAR encephalitis in the differential diagnosis of acute psychosis. Prompt diagnosis is critical as early immunotherapy and tumor removal could dramatically affect outcomes.

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The diffuse involvement of Anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

10.21203/rs.2.1815/v1 ◽

2019 ◽

Author(s):

Yun Jiang ◽

Jianpeng Ma ◽

Tao Gong ◽

Hongjun Hao ◽

Haibo Chen

Keyword(s):

Basal Ganglia ◽

Brain Mri ◽

Clinical Manifestations ◽

Autoimmune Encephalitis ◽

Ovarian Teratoma ◽

Mass Effects ◽

Glucose Levels ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. The anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special anti-NMDAR encephalitis patient who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, and the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions The anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. The recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

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The diffuse involvement of Anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

10.21203/rs.2.1815/v2 ◽

2019 ◽

Author(s):

Yun Jiang ◽

Jianpeng Ma ◽

Tao Gong ◽

Hongjun Hao ◽

Haibo Chen

Keyword(s):

Basal Ganglia ◽

Magnetic Resonance ◽

Magnetic Resonance Image ◽

Brain Mri ◽

Clinical Manifestations ◽

Ovarian Teratoma ◽

Mass Effects ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Brain Magnetic Resonance Image

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. Anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special case of an anti-NMDAR encephalitis who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, as well as the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions Anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. Recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments. Key words: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, clinic, brain, magnetic resonance image

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Clinical Relevance of Cerebrospinal Fluid Antibody Titers in Anti-N-Methyl-d-Aspartate Receptor Encephalitis

Brain Sciences ◽

10.3390/brainsci12010004 ◽

2021 ◽

Vol 12 (1) ◽

pp. 4

Author(s):

Meng-Ting Cai ◽

Yang Zheng ◽

Sa Wang ◽

Qi-Lun Lai ◽

Gao-Li Fang ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Psychiatric Symptoms ◽

Autoimmune Encephalitis ◽

Memory Deficits ◽

Clinical Severity ◽

Weak Correlation ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Significant Difference ◽

The Relationship

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is the most common autoimmune encephalitis. To date, there has been no study on the relationship between antibody (Ab) titers and clinical phenotype. This study aims to clarify the relationship between cerebrospinal fluid Ab titers and clinical manifestations of anti-NMDAR encephalitis at onset. Seventy-six consecutive patients with a definite diagnosis were enrolled. The relationship between Ab titers and different onset symptoms including psychiatric symptoms, seizures, and memory deficits were analyzed. We further investigated the correlation between Ab titers and clinical severity as assessed by the modified Rankin scale (mRS) and the clinical assessment scale for autoimmune encephalitis (CASE), respectively. The Ab titers had a median value of 1:10 (range 1:1–1:100). There was no significant difference in titers among various clinical factors including gender and combination of tumor and other diseases (each p > 0.05). Patients presenting with psychiatric symptoms at onset had higher titers than those with seizures (p = 0.008) and memory deficits (p = 0.003). The mRS scores revealed a significant but weak correlation with Ab titers (r = 0.243, p = 0.034), while CASE scores did not correlate with the titers (p = 0.125). Our findings indicated that the Ab titers were associated with the type of onset symptoms, with a higher level of patients with psychiatric symptoms. Regarding the clinical severity, the titers showed a weak correlation with the mRS, but no correlation with the CASE.

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Clinical Characteristics and Outcome of Neuronal Surface Antibody-Mediated Autoimmune Encephalitis Patients in a National Cohort

Frontiers in Neurology ◽

10.3389/fneur.2021.611597 ◽

2021 ◽

Vol 12 ◽

Author(s):

Zsófia Hayden ◽

Beáta Bóné ◽

Gergely Orsi ◽

Monika Szots ◽

Ferenc Nagy ◽

...

Keyword(s):

Clinical Characteristics ◽

Clinical Symptoms ◽

Psychiatric Symptoms ◽

Brain Mri ◽

Disease Onset ◽

Neuronal Cell ◽

Autoimmune Encephalitis ◽

Ovarian Teratoma ◽

Test Results ◽

Nmdar Encephalitis

Background: In our previous single-center study of autoimmune encephalitis (AE) related autoantibody test results we found positivity in 60 patients out of 1,034 with suspected AE from 2012 through 2018 as part of a Hungarian nationwide program. In our current multicenter retrospective study, we analyzed the clinical characteristics and outcome of AE patients with positive neuronal cell surface autoantibody test results.Methods: A standard online questionnaire was used to collect demographic and clinical characteristics, laboratory and imaging data, therapy and prognosis of 30 definitive AE patients in four major clinical centers of the region.Results: In our study, 19 patients were positive for anti-NMDAR (63%), 6 patients (20%) for anti-LGI1, 3 patients for anti-GABABR (10%) and 3 patients for anti-Caspr2 (10%) autoantibodies. Most common prodromal symptoms were fever or flu-like symptoms (10/30, 33%). Main clinical features included psychiatric symptoms (83%), epileptic seizures (73%) and memory loss (50%). 19 patients (63%) presented with signs of central nervous system (CNS) inflammation, which occurred more frequently in elder individuals (p = 0.024), although no significant differences were observed in sex, tumor association, time to diagnosis, prognosis and immunotherapy compared to AE patients without CNS inflammatory markers. Anti-NMDAR encephalitis patients were in more severe condition at the disease onset (p = 0.028), although no significant correlation between mRS score, age, sex and immunotherapy was found. 27% of patients (n = 8) with associated tumors had worse outcome (p = 0.045) than patients without tumor. In most cases, immunotherapy led to clinical improvement of AE patients (80%) who achieved a good outcome (mRS ≤ 2; median follow-up 33 months).Conclusion: Our study confirms previous publications describing characteristics of AE patients, however, differences were observed in anti-NMDAR encephalitis that showed no association with ovarian teratoma and occurred more frequently among young males. One-third of AE patients lacked signs of inflammation in both CSF and brain MRI, which emphasizes the importance of clinical symptoms and autoantibody testing in diagnostic workflow for early introduction of immunotherapy, which can lead to favorable outcome in AE patients.

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Anti-NMDAR encephalitis for psychiatrists: the essentials

BJPsych Bulletin ◽

10.1192/bjb.2021.35 ◽

2021 ◽

pp. 1-7

Author(s):

Matthew Beattie ◽

John Goodfellow ◽

Maria Oto ◽

Rajeev Krishnadas

Keyword(s):

Psychiatric Symptoms ◽

Autoimmune Encephalitis ◽

Female Adolescents ◽

Resonance Imaging ◽

Long Term Outcomes ◽

Optimal Care ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Uncommon Condition

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis predominantly presents with psychiatric symptoms. Psychiatrists need to be alert to this diagnostic possibility, especially in female adolescents and young adults, as exemplified by the real (de-identified) case outlined below. Earlier diagnosis and immunotherapy improves long-term outcomes. Collaboration with neurology colleagues is essential for optimal care. ‘Red flags’ for autoimmune encephalitis and ‘diagnostic clues’ act as helpful aide memoires for this uncommon condition. The gold standard for testing is the detection of NMDAR antibodies in cerebrospinal fluid, but serum can be tested as a more accessible (but less reliable) preliminary step. The results of routine blood tests, magnetic resonance imaging of the head and electroencephalograms can be normal or show non-specific changes. Diagnostic criteria exist to define probable and definite cases. Immunotherapy for anti-NMDAR encephalitis is effective for many patients, but recovery is prolonged and relapses can occur.

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A Case of Teratoma-Associated Anti-NMDAR Encephalitis

Current Opinion in Gynecology and Obstetrics ◽

10.18314/cogo.v1i1.1270 ◽

2018 ◽

Vol 1 (1) ◽

pp. 88-94

Author(s):

Weihua Lou ◽

Bin Yan ◽

You Wang ◽

Di W

Keyword(s):

Laboratory Tests ◽

Psychiatric Symptoms ◽

Minimally Conscious State ◽

Autoimmune Encephalitis ◽

Conscious State ◽

Response To Treatment ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Minimally Conscious ◽

Ultrasound Investigation

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune encephalitis that is associated with the NMDA receptor and has a good response to treatment. However, only a small number of cases related teratoma has been reported in literature. Here we report a case of teratoma-associated anti-NMDAR encephalitis. A 25-year-old woman with the presentation of fever for 20 days and psychiatric symptoms for 9 days was admitted to the hospital. This patient progressed to a minimally conscious state consistent with encephalitis. Considering the possibility of autoantibody-mediated encephalitis, laboratory tests were undertaken to observe the anti-NMDAR antibodies in cerebrospinal fluid (CSF) and serum, which confirmed the diagnosis of anti-NMDAR encephalitis. Furthermore, gynecological ultrasound investigation detected teratoma in the left ovary. After resection of the teratoma with Laparoscopic adnexectom and treatment with immunosuppressive therapy, the patient recovered gradually and was discharged one month after the operation. Due to the lack of clinical practice guidelines for the prevention and treatment of anti-NMDAR encephalitis, the disease is still difficult to diagnose because of its vague manifestations. For the further study, the clinic data of one case of teratoma-related anti-NMDAR encephalitis was analyzed and the relevant literatures were reviewed.

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