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

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.

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

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
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.

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

2019 ◽  
Vol 50 (04) ◽  
pp. 253-256
Author(s):  
A. Barth ◽  
I. Nassenstein ◽  
R.B. Tröbs ◽  
A. Tannapfel ◽  
H. Dercks ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Mr Imaging ◽  
Ovarian Tumor ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Adolescent Females ◽  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis

AbstractN-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare antibody-mediated autoimmune encephalitis often associated with an ovarian teratoma in adolescent females. Here we present a 17-year-old girl with only and unusual psychiatric symptoms as part of her NMDAR encephalitis in combination with a very small ovarian teratoma suspected by magnetic resonance (MR) imaging and finally histologically confirmed. We further review the literature of NMDAR encephalitis in combination with an ovarian teratoma and discuss the recommended radiological workup in children with a suspected ovarian tumor.

scholarly journals Anti-N-methyl-D-aspartate receptor encephalitis presenting as atypical psychosis in multiple sclerosis: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Khushminder Chahal ◽  
Tara Endeman ◽  
Sarah Scapinello ◽  
Michal Sapieha
Keyword(s):  
Multiple Sclerosis ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Psychiatric Inpatient ◽  
Treatment Protocol ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Atypical Symptoms ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis

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.

scholarly journals Anti-NMDAR encephalitis for psychiatrists: the essentials

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.

scholarly journals A Case of Teratoma-Associated Anti-NMDAR Encephalitis

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.

scholarly journals Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Ovarian Teratoma Improved by Prompt Surgery

2021 ◽  
Vol 39 (4) ◽  
pp. 340-342
Author(s):  
Seok-Yeol Yang ◽  
Wooryang Byun ◽  
Sung-Pa Park ◽  
Jong-Geun Seo
Keyword(s):  
Cerebrospinal Fluid ◽  
Clinical Outcome ◽  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Clinical Manifestations ◽  
Early Surgery ◽  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Early Removal ◽  
Nmdar Encephalitis

Anti-N-methyl-D aspartate receptor (NMDAR) encephalitis is often accompanied with ovarian teratomas. It has a variety of clinical manifestations including psychiatric symptoms, seizure, and motor dysfunctions. The diagnosis can be definite when clinical symptoms are present and anti-NMDAR antibodies in cerebrospinal fluid are detected. However, in patients with suspected anti-NMDAR encephalitis with teratomas, early surgery may help the clinical outcome even if the antibodies are initially negative. The authors report a patient whose clinical symptoms improved significantly after early removal of teratoma.

Frequency and temporal sequence of clinical features in adults with anti-NMDA receptor encephalitis presenting with psychiatric symptoms

2018 ◽  
Vol 49 (16) ◽  
pp. 2709-2716 ◽  
Author(s):  
Ronald J. Gurrera
Keyword(s):  
Nmda Receptor ◽  
Clinical Features ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Signs And Symptoms ◽  
Nmda Receptor Encephalitis ◽  
Nmdar Encephalitis ◽  
Unexplained Fever ◽  
Study Inclusion

AbstractBackgroundAnti-NMDA receptor (NMDAr) encephalitis is the most common autoimmune encephalitis in adults. It mimics psychiatric disorders so often that most patients are initially referred to a psychiatrist, and many are misdiagnosed. Without prompt and effective treatment, patients are likely to suffer a protracted course with significant residual disability, or death. This study focuses on the frequency and chronology of salient clinical features in adults with anti-NMDAr encephalitis who are likely to be first evaluated by a psychiatrist because their presentation suggests a primary psychiatric disorder.MethodsA systematic search of PubMed and EMBASE databases identified published reports of anti-NMDAr encephalitis associated with prominent behavioral or psychiatric symptoms. After eliminating redundancies, the frequencies and relative timing of clinical features were tabulated. Signs and symptoms were assigned temporal ranks based on the timing of their first appearance relative to the first appearance of other signs and symptoms in each patient; median ranks were used to compare temporal sequencing of both individual features and major symptom domains.ResultsTwo hundred thirty unique cases (185 female) met study inclusion criteria. The most common features were seizures (60.4%), disorientation/confusion (42.6%), orofacial dyskinesias (39.1%), and mutism/staring (37.4%). Seizures, fever, and cognitive dysfunction were often the earliest features to emerge, but psychiatric features predominated and sequencing varied greatly between individuals.ConclusionsClinicians should consider anti-NMDAr encephalitis when new psychiatric symptoms are accompanied by a recent viral prodrome, seizures or unexplained fever, or when the quality of the psychiatric symptoms is unusual (e.g. non-verbal auditory hallucinations).

scholarly journals Non-inflammatory cerebrospinal fluid delays the diagnosis and start of immunotherapy in anti-NMDAR encephalitis

2018 ◽  
Vol 76 (1) ◽  
pp. 2-5 ◽  
Author(s):  
Mariana Espinola-Nadurille ◽  
Paola Bautista-Gomez ◽  
Jose Flores ◽  
Veronica Rivas-Alonso ◽  
Rodrigo Perez-Esparza ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Symptom Onset ◽  
Correct Diagnosis ◽  
Retrospective Data ◽  
Csf Analysis ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Neuropsychiatric Manifestations ◽  
Inflammatory Pattern ◽  
Autoimmune Encephalopathy

ABSTRACT Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a form of autoimmune encephalopathy that presents with a wide variety of symptoms, including neuropsychiatric manifestations. The authors’ aim for this study was to analyze the results of paraclinical studies of patients with a diagnosis of anti-NMDAR encephalitis and the association between symptom onset and diagnosis, and start of immunotherapy. Retrospective data of 29 patients with anti-NMDAR encephalitis were gathered and analyzed. Abnormal EEG was found in 27 patients (93.1%), whereas MRI was abnormal in 19 patients (65.5%). In contrast, an inflammatory pattern on CSF analysis was found in only 13 patients (44.8%). The absence of pleocytosis or increased proteins in the CSF was associated with a longer time from symptom onset to diagnosis and treatment (p = 0.003). The authors conclude that noninflammatory CSF may delay the correct diagnosis and start of immunotherapy in anti-NMDAR encephalitis. In the presence of suggestive clinical features, extensive studies including EEG are recommended.

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

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

scholarly journals Autoimmune encephalitis: the clinical evolution as a key to the diagnosis

2019 ◽  
Vol 12 (9) ◽  
pp. e231094 ◽  
Author(s):  
Sara Teixeira ◽  
Joana Caldeira Santos ◽  
Marta Vila Real ◽  
Fátima Santos
Keyword(s):  
Neurological Examination ◽  
Psychiatric Symptoms ◽  
Focal Epilepsy ◽  
Disease Onset ◽  
Autoimmune Encephalitis ◽  
Lower Limbs ◽  
Response To Treatment ◽  
Late Response ◽  
Nmdar Encephalitis ◽  
Age Appropriate

Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a devastating disease, that despite being increasingly diagnosed, there are no consensus guidelines for the optimal management. A previously healthy 3-year-old-boy brought to the emergency department due to seizures. Neurological examination was normal, and electroencephalogram (EEG) suggested focal epilepsy. Anticonvulsive medication was initiated. He progressively lost age-appropriate language skills, presented behavioural changes and psychiatric symptoms. Neurological examination at that time revealed symmetric gross motor weakness of the lower limbs. Brain and spinal cord MRI and cerebrospinal fluid were normal. Repeated EEG showed global lentification. Steroid therapy was initiated for the suspicion of autoimmune encephalitis, later confirmed as NMDAR encephalitis. He became clinically improved after 10 days of treatment but only returned to his baseline after 3 months of disease onset. The authors emphasised the variable course of the disease and possible late response to treatment.

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