scholarly journals REMISSION OF TREATMENT RESISTANT SCHIZOPHRENIA FOLLOWING RESECTION OF OVARIAN TERATOMA: A CASE OF ANTI-NMDAR ENCEPHALITIS

2019 ◽  
Author(s):  
Su Tein Sim

Objective: Ovarian teratoma has been frequently associated with Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis in recent years since its discovery by Dalmao in the year 2007. Most patients with anti-NMDAR encephalitis develop a multistage illness that progresses from psychosis, memory deficits, seizures, and language disintegration into a state of unresponsiveness with catatonic features often associated with abnormal movements, and autonomic and breathing instability. Milder or incomplete forms of the disorder in which patients develop predominant or apparently isolated psychiatric symptoms are uncommon. The overlap of symptomatology between anti-NMDAR encephalitis and schizophrenia often result in misdiagnosis. This case represents a forme fruste of ovarian teratoma related anti-NMDAR encephalitis which only presents with psychiatric and cognitive symptoms without obvious neurological deficits. Method: We report a case of a 37-year-old lady diagnosed with Treatment-Resistant Schizophrenia (TRS) who had a poor response to treatment despite multiple trials of antipsychotics and electroconvulsive therapy. Results: Our patient achieved remission following the excision of the ovarian teratoma and had been maintaining well with minimal psychotropic intervention. Conclusion: This case suggests that clinicians should always have a high index of suspicion for anti-NMDAR encephalitis in young women presenting with new-onset psychiatric symptoms and/or treatment-resistant psychosis. More research is needed to shed light on the exact role of NMDAR within the brain circuitry and the effects of its modulation in both Schizophrenia and anti-NMDAR encephalitis.

2021 ◽  
Vol 12 ◽  
Author(s):  
Alexander Moldavski ◽  
Holger Wenz ◽  
Bettina E. Lange ◽  
Cathrin Rohleder ◽  
F. Markus Leweke

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a neuroinflammatory condition mediated by autoantibodies against the GluN1 subunit of the receptor. Clinically, it is characterized by a complex neuropsychiatric presentation with rapidly progressive psychiatric symptoms, cognitive deficits, seizures, and abnormal movements. Isolated psychiatric manifestations of anti-NMDAR encephalitis are rare and usually dominated by psychotic symptoms. We present a case of an 18-year-old female high school student—without a previous history of psychiatric disorders—with a rapid onset severe depressive syndrome. Surprisingly, we found pleocytosis and anti-NMDAR autoantibodies in the cerebrospinal fluid (CSF), despite an otherwise unremarkable diagnostic workup, including blood test, clinical examination, and cranial magnetic resonance imaging (MRI). After intravenous immunoglobulins treatment, a complete remission of the initial symptoms was observed. In a follow-up 5 years later, the young woman did not experience any relapse or sequelae. Anti-NMDAR encephalitis can present in rare cases as an organic disorder with major depressive symptoms without distinct concomitant psychotic or neurological symptoms. A clinical presentation such as a rapid onset of symptoms, distinct disturbance in the thought process, restlessness, and cognitive deficits should prompt screening for NMDAR- and other neural autoantibodies to rule out this rare but debilitating pathology.


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


2019 ◽  
Vol 12 (9) ◽  
pp. e231094 ◽  
Author(s):  
Sara Teixeira ◽  
Joana Caldeira Santos ◽  
Marta Vila Real ◽  
Fátima Santos

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.


2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Shikma Keller ◽  
Pablo Roitman ◽  
Tamir Ben-Hur ◽  
Omer Bonne ◽  
Amit Lotan

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.


2016 ◽  
Vol 33 (S1) ◽  
pp. S525-S526
Author(s):  
R. Gallego ◽  
A. Flores

IntroductionAnti-NMDA receptor (NMDAR) encephalitis, formally recognized in 2007 by Dalmau et al, is an autoimmune disorder with a complex presentation that includes psychiatric symptoms, memory deficits, and autonomic instability. The exact incidence is unknown but age, gender, and ethnicity may all play a role. Presence of antibodies against the GluN1 subunit of the NMDAR in the CSF and serum confirm the diagnosis of NMDAR encephalitis.Case reportWe report the case of a previously healthy, 19-year-old woman, 6 weeks pregnant. She had a generalized tonic-clonic seizure followed by psychiatric symptoms, including insomnia, emotional lability, delusions, and disorganized behavior. During the course of the disease, she demonstrated speech impairments and catatonic features associated with abnormal movements.She was provided lorazepam 1 mg twice a day to treat her catatonic symptoms, her insomnia and her speech improved. Olanzapine was introduced, reaching a dose of 20 mg/day for managing psychosis and agitation.DiscussionNMDA-R encephalitis is a novel disorder with prominent psychiatric manifestations that is widely underdiagnosed. Neuroleptics may be helpful for managing psychosis and agitation, but may exacerbate movement abnormalities. Benzodiazepines are helpful for agitation, insomnia and catatonia associated with this condition.ConclusionEarlier recognition of this illness is crucial as prompt diagnosis and multidisciplinary treatment, can potentially improve prognosis. There is an increasing need for psychiatrists to become aware of the disorder and consider it in their differential diagnosis, specially in patients with new onset psychosis, history of encephalitis or subtle neurological symptoms. Careful selection of psychopharmacological interventions may reduce suffering.Disclosure of interestThe authors have not supplied their declaration of competing interest.


2020 ◽  
Vol 3 (1) ◽  
pp. 59-61
Author(s):  
Bibek Rajbhandari ◽  
Minani Gurung

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. We present a case of a 13-year old who presented with noticeably chirpiness, excessive talking with unknown people and wandering around the neighborhood without purpose.The main symptoms of the patient and the important clinical findings were irrelevant talking which later developed into slurring of speech, abnormal movements and memory loss.This case is an example of how easily we are misled towards diagnosis based on the present symptoms. The patient suffered the unnecessary stigma of a psychiatric illness, which might stay imprinted on her for a long time. In this report we call for attention to the inclusion of anti-NMDAR encephalitis in the differential diagnosis of acute psychosis. It adds on to show that NMDAR might present in the most unexpected and unpredictable ways, sometimes misleading the patient away from medical help.Prompt diagnosis is critical as early immunotherapy and tumor removal could dramatically affect outcomes.


2020 ◽  
pp. 1-4
Author(s):  
Lourdes Sala Climent ◽  
Ana R. Calvo Redol ◽  
F. Xavier González Tallada ◽  
Javier Medrano Juárez ◽  
Lourdes Sala Climent ◽  
...  

Objective: Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a paraneoplastic, immunemediated encephalopathy with a known association with ovarian tumors, more frequently with mature teratoma. Case Report: We present the case of a 20-year-old patient presenting with fever for 3 weeks and an acute onset of cognitive disturbances. The initial suspicion was that of an infective encephalopathy; however, the lumbar puncture results were negative. Electroencephalography reported epileptiform activity in anterior horns, supporting a diagnosis of encephalopathy. Extensive autoimmune screening was done and found to be positive for anti-NMDAR antibodies. A whole body-TC found a right adnexal tumor compatible with ovarian teratoma. At laparoscopy, the right adnexal tumor was excised, preserving most of the ovarian parenchyma; and histology confirmed a mature teratoma with neural tissue. There were no postoperative complications. The patient recovered normal speech and her former cognitive status, within 40 days from the day after surgery. Conclusion: The association ovarian teratoma-anti-NMDAR encephalitis is a serious and potentially fatal pathology occurring in young women and under-recognized in many countries and among gynaecologists. Given the recent discovery, special attention should be given to possible behavioural changes and psychiatric symptoms that may occur in young women with apparently unknown cause. It is also very important the diagnostic confirmation through the positivity of the anti-NMDAR antibodies, the diagnosis of the underlying tumour and the early tumour excision; since, the earlier the treatment is, the greater is the rate of success and the clinical remission.


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.


2018 ◽  
Vol 89 (6) ◽  
pp. A44.3-A45 ◽  
Author(s):  
Leon S Edwards ◽  
Michael H Barnett ◽  
Matthew C Kiernan

IntroductionNMDAR encephalitis is an autoimmune condition with antibodies to the NR1 subunit of the NMDAR. It has a variable clinical presentation with behavioural and psychiatric symptoms. There is frequently a relapsing course. A known association exists with tumours, particularly ovarian teratoma. Cases which are not associated with tumour often fail first line therapy. As such, therapy for this disease can be challenging and involves a spectrum of immunotherapy. Rituximab is widely considered to be second line treatment for this illness. We present two cases of refractory anti-NMDAR encephalitis.CasesBoth patients were confirmed antibody positive in the serum and cerebral spinal fluid. There was no tumour identified on whole body positron emission tomography and transvaginal pelvic ultrasound. Each patient was initially treated with first line therapy of intravenous immunoglobulin, plasmapheresis and methylprednisolone with limited symptomatic improvement. Second line therapy of two doses of rituximab was administered intravenously two weeks apart at a dose of 1000 mg. Mycophenolate mofetil was used at a dose of 1000 mg twice daily to minimise B-cell reconstitution. Response to treatment was monitored with measurement of CD 19+ Pan B cells in peripheral blood and clinical assessment by the consultant liaison psychiatry service. Both patients demonstrated significant response with undetectable CD 19+ Pan B cells on serial testing and sustained clinical improvement in initial behavioural symptoms.ConclusionIn patients with anti-NMDAR encephalitis, rituximab combined with maintenance mycophenolate mofetil may be an effective treatment.


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.


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