Anti- NMDAR Autoimmune Encephalitis Presenting as Acute Psychosis: A Case Report

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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Psychiatric Symptoms in a Patient with NMDA Receptor Encephalitis

Journal of Psychiatrists Association of Nepal ◽

10.3126/jpan.v9i1.31343 ◽

2020 ◽

Vol 9 (1) ◽

pp. 66-68

Author(s):

L Paudel ◽

S Samadarshi ◽

S Limbu ◽

R Sharma ◽

P Chalise ◽

...

Keyword(s):

Psychiatric Symptoms ◽

Autoimmune Disorder ◽

Behavioral Changes ◽

Psychiatric Consultation ◽

Course Of Illness ◽

Nmda Receptor Encephalitis ◽

Abnormal Movements ◽

Aspartate Receptor ◽

Autonomic Instability ◽

Psychiatric Manifestations

Anti N methyl D aspartate receptor (NMDA-R) encephalitis is an autoimmune disorder that encompasses various psychiatric and neurological symptoms. Psychiatric manifestations are often the presentation of the disease that often delay or mislead the diagnosis. Complex psychiatric symptoms, seizures, autonomic instability, abnormal movements, behavioral changes and impaired cognitive functions are present during the course of illness. Prodromal psychiatric symptoms often confuse the physicians and even patient initially seeks psychiatric consultation. Here we present a case of 19 years female who presented with initial psychiatric symptoms that progressed to seizure, autonomic instability, abnormal movement and other encephalitic symptoms.

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Management And Psychiatric Manifestations Of Anti-NMDA Receptor Encephalitis, A Case Report

European Psychiatry ◽

10.1016/j.eurpsy.2016.01.1946 ◽

2016 ◽

Vol 33 (S1) ◽

pp. S525-S526

Author(s):

R. Gallego ◽

A. Flores

Keyword(s):

Case Report ◽

Nmda Receptor ◽

Psychiatric Symptoms ◽

Multidisciplinary Treatment ◽

Autoimmune Disorder ◽

Clonic Seizure ◽

Nmda Receptor Encephalitis ◽

Abnormal Movements ◽

Nmdar Encephalitis ◽

Psychiatric Manifestations

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.

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Differences and diversity of autoimmune-mediated encephalitis in 77 cases from a single tertiary care center

10.21203/rs.2.1811/v1 ◽

2019 ◽

Author(s):

Abhinbhen Wasontiwong Saraya ◽

Kanthita Worachotsueptrakun ◽

Kritchai Vutipongsatorn ◽

Chanikarn Sonpee ◽

Thiravat Hemachudha

Keyword(s):

Behavioral Change ◽

Care Center ◽

Tertiary Care ◽

Autoimmune Encephalitis ◽

Numerical Data ◽

Clinical Findings ◽

Exact Test ◽

Abnormal Movements ◽

Surface Antibody ◽

Surface Antibodies

Abstract Background Since the discovery of N-methyl-D-aspartate receptor (NMDAr) antibody in 2007, the incidence of autoantibody-mediated encephalitis has risen globally. Here we analyzed and compared groups of autoantibody-associated encephalitis patients based on clinical findings and laboratory results in order to find differences between two major groups of autoantibody-mediated encephalitis: intracellular and neuronal surface antibodies. Methods 77 records of autoimmune encephalitis/encephalomyelitis patients admitted to King Chulalongkorn Memorial Hospital (KCMH) between October 2010 and February 2017 were reviewed. Patients with infections or those with classic central nervous system demyelinating features were excluded. Categorical data was analyzed using chi-square and Fisher’s exact test. Unpaired, two-tailed t-test was performed to analyze numerical data. Results Of 77 patients, 40% presented with neuronal surface antibodies and 33% had intracellular antibodies. The most common autoantibody detected in each group was anti-NMDA receptor antibody (25/31, 81%) and anti-Ri antibody (7/25, 28%) respectively. In the neuronal surface antibody group, behavioral change was the most common complaint (45%), followed by seizures (39%), abnormal movements (29%) and psychosis/mood disorder (23%). In the latter group, seizure was the most common presenting symptom (32%), followed by motor weakness (20%), behavioral change (16%) and abnormal movements (16%). Patients with neuronal surface antibodies were significantly younger (35 vs 48 years old, p=0.04) and were more likely to present with behavioral change (45% vs 16%, p=0.02). Mortality rate was higher in the intracellular group although this was statistically insignificant (16% vs 3.2%, p=0.09). No significant differences were detected in magnetic resonance imaging and cerebrospinal fluid (CSF) profile. Conclusions The prevalence of neuronal surface antibody group was much higher than the intracellular group. In the earlier stages of the disease, both groups have comparable clinical outcomes. Furthermore, it is difficult to distinguish autoantibody-associated encephalitis patients based on clinical data, neuroimaging and CSF profile. Therefore, we recommend that patients with features of autoimmune encephalitis should be screened for both the neuronal surface and intracellular antibodies regardless of clinical presentation.

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Differences and diversity of autoimmune-mediated encephalitis in 77 cases from a single tertiary care center

10.21203/rs.2.1811/v2 ◽

2019 ◽

Author(s):

Abhinbhen Wasontiwong Saraya ◽

Kanthita Worachotsueptrakun ◽

Kritchai Vutipongsatorn ◽

Chanikarn Sonpee ◽

Thiravat Hemachudha

Keyword(s):

Behavioral Change ◽

Care Center ◽

Tertiary Care ◽

Autoimmune Encephalitis ◽

Numerical Data ◽

Clinical Findings ◽

Exact Test ◽

Abnormal Movements ◽

Surface Antibody ◽

Surface Antibodies

Abstract Background Since the discovery of N-methyl-D-aspartate receptor (NMDAr) antibody in 2007, the incidence of autoantibody-mediated encephalitis has risen globally. Here we analyzed and compared groups of autoantibody-associated encephalitis patients based on clinical findings and laboratory results in order to find differences between two major groups of autoantibody-mediated encephalitis: intracellular and neuronal surface antibodies. Methods 77 records of autoimmune encephalitis/encephalomyelitis patients admitted to King Chulalongkorn Memorial Hospital (KCMH) between October 2010 and February 2017 were reviewed. Patients with infections or those with classic central nervous system demyelinating features were excluded. Categorical data was analyzed using chi-square and Fisher’s exact test. Unpaired, two-tailed t-test was performed to analyze numerical data. Results Of 77 patients, 40% presented with neuronal surface antibodies and 33% had intracellular antibodies. The most common autoantibody detected in each group was anti-NMDA receptor antibody (25/31, 81%) and anti-Ri antibody (7/25, 28%) respectively. In the neuronal surface antibody group, behavioral change was the most common complaint (45%), followed by seizures (39%), abnormal movements (29%) and psychosis/mood disorder (23%). In the latter group, seizure was the most common presenting symptom (32%), followed by motor weakness (20%), behavioral change (16%) and abnormal movements (16%). Patients with neuronal surface antibodies were significantly younger (35 vs 48 years old, p=0.04) and were more likely to present with behavioral change (45% vs 16%, p=0.02). Mortality rate was higher in the intracellular group although this was statistically insignificant (16% vs 3.2%, p=0.09). No significant differences were detected in magnetic resonance imaging and cerebrospinal fluid (CSF) profile. Conclusions The prevalence of neuronal surface antibody group was much higher than the intracellular group. In the earlier stages of the disease, both groups have comparable clinical outcomes. Furthermore, it is difficult to distinguish autoantibody-associated encephalitis patients based on clinical data, neuroimaging and CSF profile. Therefore, we recommend that patients with features of autoimmune encephalitis should be screened for both the neuronal surface and intracellular antibodies regardless of clinical presentation.

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A patient with a long history of relapsing psychosis and mania presenting with anti-NMDA receptor encephalitis ten years after first episode

Dementia & Neuropsychologia ◽

10.1590/1980-57642015dn93000016 ◽

2015 ◽

Vol 9 (3) ◽

pp. 311-314 ◽

Cited By ~ 2

Author(s):

Mateus Mistieri Simabukuro ◽

Christian Henrique de Andrade Freitas ◽

Luiz Henrique Martins Castro

Keyword(s):

Psychiatric Disorders ◽

Psychiatric Symptoms ◽

Bipolar Affective Disorder ◽

Autoimmune Encephalitis ◽

Autoimmune Disorder ◽

First Episode ◽

Nmda Receptor Encephalitis ◽

Nmdar Encephalitis ◽

Lack Of Information ◽

History Of

Anti-N-methyl- D-aspartate receptor (NMDAR) encephalitis is a recently discovered autoimmune disorder, in which antibodies target NMDARs in the brain, leading to their removal from synapses. Early in the disease course, patients often present with marked psychosis and mood disturbances (i.e. mania, depression), explaining why most of these patients are first seen by psychiatrists. Hence, autoimmune encephalitis is receiving growing attention from psychiatry, mainly owing to concerns over misdiagnosing immunomediated and potentially curable disorders as primary psychiatric disorders, such as schizophrenia or major depressive disorder. Although anti-NMDAR encephalitis occurs in the context of new-onset psychiatric symptoms, there is a lack of information on differential diagnosis and treatment of this disorder after a long-term diagnostic history of functional psychiatric disorders. We report a case of a patient with a long history of bipolar affective disorder evolving with anti-NMDAR encephalitis, initially misdiagnosed as non-organic psychosis.

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Psychiatric Symptoms As Onset of Anti-Nmdar Encephalitis

European Psychiatry ◽

10.1016/j.eurpsy.2016.01.1941 ◽

2016 ◽

Vol 33 (S1) ◽

pp. S525-S525

Author(s):

M.C. Cancino Botello ◽

A. Cercos López ◽

V. Chavarria Romero ◽

G. Sugranyes Ernest

Keyword(s):

Nmda Receptor ◽

Psychiatric Symptoms ◽

Autoimmune Encephalitis ◽

Autoimmune Disorder ◽

Psychotic Symptoms ◽

Nmdar Encephalitis ◽

Clinical Presentations ◽

Children And Young Adults ◽

Competing Interest ◽

Specific Symptoms

IntroductionEvery more often, there is evidence that shows a relationship between psychiatric symptoms and autoimmune disorders. Such is the case of anti-NMDAR encephalitis, in which it has been recently described the development of psychotic symptoms. Anti-NMDAR encephalitis is an autoimmune disorder that involves IgG autoantibodies against the NMDA receptor subunit GluN1. This last fact could support the relationship with the glutamatergic model of schizophrenia.ObjectiveTo conduct a current review to deepen the detection and management of anti-NMDAR encephalitis, due to the frequent existence of psychiatric symptoms at onset, which have contributed to the difficulty of diagnose.MethodSystematic review of the literature in English (PubMed), with the following keywords: “Autoimmune encephalitis”, “psychosis”, and “NMDA receptor”.ResultsAutoimmune encephalitis appears more frequently in children and young adults and it is characterized by a prodromal period, in which there usually are non-specific symptoms of headaches or fever. Next, it could progress to cognitive deficits, seizures, catatonic symptoms and psychosis. However, sometimes in the rarest clinical presentations, there is nothing but psychiatric symptoms at the onset of encephalitis, which leads to misdiagnose and lack of proper treatment. This fact has stimulated the curiosity of the psychiatry scientific community, since the anti-NMDAR encephalitis may mimic the glutamatergic model of schizophrenia.ConclusionsTo make an accurate and detailed diagnostic formulation in people with psychiatric symptoms as onset of any disorder is essential to determine whether it is a primary psychiatric disorder or symptoms associated to another disease.Disclosure of interestThe authors have not supplied their declaration of competing interest.

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

Psychological Medicine ◽

10.1017/s0033291718003665 ◽

2018 ◽

Vol 49 (16) ◽

pp. 2709-2716 ◽

Cited By ~ 3

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).

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Case Report: Severe Adolescent Major Depressive Syndrome Turns Out to Be an Unusual Case of Anti-NMDA Receptor Encephalitis

Frontiers in Psychiatry ◽

10.3389/fpsyt.2021.679996 ◽

2021 ◽

Vol 12 ◽

Author(s):

Alexander Moldavski ◽

Holger Wenz ◽

Bettina E. Lange ◽

Cathrin Rohleder ◽

F. Markus Leweke

Keyword(s):

Cognitive Deficits ◽

Psychiatric Symptoms ◽

Previous History ◽

Rapid Onset ◽

Depressive Syndrome ◽

Psychotic Symptoms ◽

Major Depressive ◽

Abnormal Movements ◽

Nmdar Encephalitis ◽

Initial Symptoms

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.

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

BMJ Case Reports ◽

10.1136/bcr-2019-231094 ◽

2019 ◽

Vol 12 (9) ◽

pp. e231094 ◽

Cited By ~ 1

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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