scholarly journals Anti-NMDAR encephalitis for psychiatrists: the essentials

2021 ◽  
pp. 1-7
Author(s):  
Matthew Beattie ◽  
John Goodfellow ◽  
Maria Oto ◽  
Rajeev Krishnadas

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.

Author(s):  
Belinda Lennox

Autoimmune encephalitis is a neuropsychiatric disorder. While it is generally managed by neurologists, many of the presenting symptoms and most of the challenges in management are psychiatric. The description of psychiatric symptoms as part of the presenting symptoms of encephalitis dates back to the first descriptions of the disorder. The more recent discovery of autoimmune causes of encephalitis has particular relevance for psychiatry: there is significant overlap between the initial presentation of encephalitis with other core psychiatric disorders such as depression and psychosis. The particular challenge for inpatient psychiatry is to screen and detect these disorders early. The current evidence indicates that the rapid detection and delivery of immunotherapy, rather than the use of psychiatric treatments, is associated with better long-term outcomes for patients. Conversely, the risks of not detecting encephalitis are of long-term disability, or even death in a proportion of patients. This chapter describes the evolution in understanding of autoimmune encephalitis, the characteristics associated with particular antibodies, and the investigations and management of patients with autoimmune encephalitis from a psychiatric perspective.


2019 ◽  
Vol 50 (04) ◽  
pp. 253-256
Author(s):  
A. Barth ◽  
I. Nassenstein ◽  
R.B. Tröbs ◽  
A. Tannapfel ◽  
H. Dercks ◽  
...  

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.


2019 ◽  
Vol 11 (3) ◽  
Author(s):  
Susanne Buechner ◽  
Igor Florio ◽  
Gabriele J. Sixt ◽  
Francesco Teatini

One of the best characterized autoimmune encephalitis is the Anti-Nmethyl-D-aspartate receptor (NMDAR) encephalitis, which may occur in the presence of cancer. First- and second-line immunotherapy and oncological investigations are suggested. We present here a case of an 18-year-old female who was our first patient suffering from Anti-NMDAR encephalitis more than 9 years ago. She was satisfactorily treated with intravenous immunoglobulins and high dose steroid therapy. After more than one year the patient had a relapse. First-line immunotherapy was repeated; however, a complete recovery was achieved only after plasmapheresis. Afterwards, she continued maintenance immunotherapy with steroids for two years and with Azathioprine for about five years associated to regular oncological assessment. In the last years our therapeutical approach of Anti-NMDARencephalitis has significantly changed. Nevertheless, established treatment guidelines are still missing and the role of long-term maintenance immunotherapy is largely unexplored. In addition, oncological revaluation might be indicated in selected patients.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Khushminder Chahal ◽  
Tara Endeman ◽  
Sarah Scapinello ◽  
Michal Sapieha

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.


2021 ◽  
Vol 12 (1) ◽  
pp. 4
Author(s):  
Meng-Ting Cai ◽  
Yang Zheng ◽  
Sa Wang ◽  
Qi-Lun Lai ◽  
Gao-Li Fang ◽  
...  

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.


2018 ◽  
Vol 1 (1) ◽  
pp. 88-94
Author(s):  
Weihua Lou ◽  
Bin Yan ◽  
You Wang ◽  
Di W

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.


Children ◽  
2021 ◽  
Vol 8 (3) ◽  
pp. 227
Author(s):  
Rudaina Banihani ◽  
Judy Seesahai ◽  
Elizabeth Asztalos ◽  
Paige Terrien Church

Advances in neuroimaging of the preterm infant have enhanced the ability to detect brain injury. This added information has been a blessing and a curse. Neuroimaging, particularly with magnetic resonance imaging, has provided greater insight into the patterns of injury and specific vulnerabilities. It has also provided a better understanding of the microscopic and functional impacts of subtle and significant injuries. While the ability to detect injury is important and irresistible, the evidence for how these injuries link to specific long-term outcomes is less clear. In addition, the impact on parents can be profound. This narrative summary will review the history and current state of brain imaging, focusing on magnetic resonance imaging in the preterm population and the current state of the evidence for how these patterns relate to long-term outcomes.


2018 ◽  
Vol 49 (16) ◽  
pp. 2709-2716 ◽  
Author(s):  
Ronald J. Gurrera

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).


2020 ◽  
Vol 19 (5) ◽  
pp. 124-131
Author(s):  
N.M. Podzolkova ◽  
◽  
V.V. Korennaya ◽  
O.S. Levin ◽  
E.E. Vasenina ◽  
...  

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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