Anti-NMDA Receptor Encephalitis Presenting Research Articles

Teaching NeuroImage: Anti-NMDA Receptor Encephalitis Presenting With Cerebellitis in a Pediatric Patient.

Teaching NeuroImage: Anti-NMDA Receptor Encephalitis Presenting With Cerebellitis in a Pediatric Patient.

Anti-NMDA Receptor Encephalitis Presenting with Language Impairment and Bradycardia

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is known to have various clinical symptoms, but usually shows acute behavioral changes, memory deficit, speech problems and dysregulation of autonomic nervous system. Recently, it has been found that NMDA receptors perform important roles not only in central nervous system but also in peripheral organs, like control the cardiac rhythm by located in the myocardium and cardiac conduction system. The authors would like to report a case of bradycardia accompanied by anti-NMDA receptor encephalitis.

Case of Anti-NMDA Receptor Encephalitis Presenting in a Toddler With Hemorrhagic Cavernomas

Objective N/A. Background N/A. Design/Methods Introduction: Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis signifies an autoimmune antibody-mediated neuropsychiatric disease that often presents with a set of well-described clinical characteristics and other times manifests with more rare features. The heterogeneity of patient presentation can propose a diagnostic challenge to even the best clinical neurologist. Anti-NMDA receptor encephalitis should be considered for patients who possess an alternative existing diagnosis that shows atypical progression because early recognition and treatment of the disease can help reduce long-term complications. Results Case Report: We illustrate a 14 month-old previously healthy boy with anti-NMDA receptor encephalitis who first presented with focal seizures. Initial neurologic imaging revealed intracranial hemorrhage with underlying cavernous malformations. He responded well to a single anti-seizure agent, but re-presented one week later with transient weakness that was ultimately attributed to worsening intracranial hemorrhage with surrounding edema. Upon his third presentation, he developed dyskinesias, sleep dysfunction, autonomic instability, cognitive changes, and motor regression, prompting further work-up with lumbar puncture. Cerebrospinal fluid analysis showed a positive NMDA antibody titer of 1:40. Treatment with intravenous steroids, plasma exchange (PLEX), and intravenous immune globulin (IVIg), followed by infusions of Rituximab and Cyclophosphamide resulted in gradual, marked clinical improvement. Conclusions This case study and literature review explores the relationship between cavernous malformations, intracranial hemorrhage, and anti-NMDA receptor encephalitis, and how these diagnoses respond to escalating immunomodulation therapies. Consideration of this entity should be made when the neurologic examination does not follow an expected course of a previously established diagnosis. With timely recognition and aggressive treatment approaches, patients can achieve substantial clinical improvement.

Balint Syndrome as an Atypical Presentation of Paraneoplastic Anti-NMDA Receptor Encephalitis: A Case Report and Review of the Literature (P5-1.007)

Balint Syndrome as an Atypical Presentation of Paraneoplastic Anti-NMDA Receptor Encephalitis: A Case Report and Review of the Literature (P5-1.007)

Abnormal presentation and imaging finding of anti-NMDA receptor encephalitis, a report of two cases

Meningitis and myelitis are a very rare atypical presenting feature of anti-NMDA receptor encephalitis. We describe an unusual clinical presentation of anti-NMDA receptor encephalitis, the first case with a typical picture of meningitis followed by encephalitis, the second case with myelitis followed by psychiatric prodroma. We aim to widen the differential diagnosis in two patients presenting with clinical meningitis and myelitis followed by other common clinical symptoms and signs.

Anti-NMDA Receptor Encephalitis Presenting with Autonomic Dysfunction. (5154)

Anti-NMDA Receptor Encephalitis Presenting with Autonomic Dysfunction. (5154)

Anti-NMDA receptor encephalitis presenting as new onset refractory status epilepticus in COVID-19

• Autoimmune encephalitis and status epilepticus in COVID-19 can be challenging. • Immunotherapy could be useful for anti-NMDAr encephalitis and SARS-Cov-2 infection. • Cytokines storm could play a role for NORSE presentation in NDMA-R encephalitis.

Case report: meningitis as a presenting feature of anti-NMDA receptor encephalitis

BackgroundMeningitis is a very rare atypical presenting feature of anti-NMDA receptor encephalitis. In our case report, we describe an unusual clinical presentation of anti-NMDA receptor encephalitis with a biphasic pattern of meningitis followed by encephalitis and discuss potential mechanisms underlying this presentation. We aim to widen the differential diagnosis to be considered in a patient presenting with clinical meningitis and pyrexia.Case presentationThis is a case of a 33-year old Caucasian woman who initially presented with a lymphocytic meningitis attributed to a viral infection. She subsequently developed fluctuating consciousness, agitation, visual hallucinations, dyskinetic movements, a generalized tonic-clonic seizure, and autonomic instability. Investigations revealed a diagnosis of anti-NMDA receptor encephalitis secondary to a previously unidentified ovarian teratoma. She made an excellent recovery with immunotherapy and removal of the teratoma.ConclusionClinicians should consider autoimmune encephalitides in individuals with meningitis, particularly where extensive investigations fail to identify a causative pathogen and there is rapid development of an encephalitic phenotype.

Akut Psikoz Bulguları ile Başvuran Anti-NMDA Reseptör Ensefalitli Bir Olgu Sunumu

Akut Psikoz Bulguları ile Başvuran Anti-NMDA Reseptör Ensefalitli Bir Olgu Sunumu

Anti-NMDA Receptor Encephalitis Presenting with Acute Psychosis

Anti-NMDA Receptor Encephalitis Presenting with Acute Psychosis

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

Anti-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. A 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. Two 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. Clinicians 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).

Refining the psychiatric syndrome of anti-N-methyl-d-aspartate receptor encephalitis.

To review the psychiatric symptoms of anti-N-methyl-d-aspartate (NMDA) receptor encephalitis, in an attempt to differentiate the presentation from a primary psychiatric disorder. A systematic literature review of PubMed and EMBASE of all published cases of anti-NMDA receptor encephalitis was performed from inception to January 2018. There were 706 cases of anti-NMDA receptor encephalitis identified. Cases were typically young (mean age 22.6years, SD 14.8), female (F:M ratio 3.5:1) and presented with significant behavioural disturbance. Reported behaviour was most commonly severe agitation and aggression, abnormal speech, and catatonia. Psychosis occurred in 45.8% of cases. Investigation results were inconsistent (MRI abnormal in 35.6%, EEG abnormal in 83.0%) and non-specific. Psychiatric treatment often required multiple psychotropics, and there may be increased risk of significant side-effects such as neuroleptic malignant syndrome. Prognosis was usually good; however, cognitive and behavioral symptoms remained prominent during recovery, and psychiatrist involvement was required in this period. The presentation of anti-NMDA receptor encephalitis is variable. However, there are often psychiatric features which are atypical to a primary psychiatric illness, such as severe agitation, speech abnormalities, and catatonia, which may help early identification.

Anti-NMDA receptor encephalitis presenting as isolated aphasia in an adult

ABSTRACTAnti-NMDA receptor (NMDA-r) encephalitis is a relatively rare cause of autoimmune encephalitis with divergent clinical presentations. We report a case of an adult patient with anti-NMDA-r encephalitis presenting with isolated, abrupt-onset aphasia. Her condition remained unaltered over a period of 6 months. The patients’ electroencephalogram findings were typical for NMDA-r encephalitis; however, her magnetic resonance imaging and cerebrospinal fluid analysis were normal. She responded well to immunotherapy, and aphasia eventually resolved. The natural course of the present case contradicts the rapidly progressive nature of typical NMDA-r encephalitis. Furthermore, it broadens the clinical spectrum of anti-NMDA-r encephalitis, to incorporate isolated, nonprogressive aphasia.

Poster Number: EI 7 - Misdiagnosed and Misunderstood—an Atypical Presentation of Anti-NMDA Receptor Encephalitis

Poster Number: EI 7 - Misdiagnosed and Misunderstood—an Atypical Presentation of Anti-NMDA Receptor Encephalitis

Anti-NMDA receptor encephalitis: An emerging differential diagnosis in the psychiatric community.

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a new diagnosis, as recent as 2007, that develops as a result of autoantibodies to the NMDA receptor. The clinical manifestations of the disorder include complex psychiatric symptoms, seizures, movement disorders, cognitive dysfunction, and autonomic instability. Tumor resection, if present, and immunotherapy are the mainstays of therapy. Treatment should be initiated early and aggressively as it has been associated with better patient outcomes. A significant proportion of patients with anti-NMDA receptor encephalitis initially seek the help of a psychiatrist, highlighting the importance of its recognition within the mental health community. In an effort to promote disease awareness, this article will review a patient case and the pathophysiology, clinical presentation, diagnosis, and management of anti-NMDA receptor encephalitis.

Anti-NMDA receptor encephalitis presenting as an acute psychotic episode misdiagnosed as dissociative disorder: a case report.

BackgroundIn 2005, “anti-N-methyl-d-aspartate (NMDA) receptor encephalitis,” a syndrome with prominent psychiatric symptoms, memory loss, decrease in level of consciousness, and central hypoventilation, was described in young women with ovarian teratomas and antibodies against an antigen highly expressed in the hippocampus. This report highlights the growing need for increased awareness among psychiatrists and other relevant medical professionals about this under-diagnosed disorder, which should be considered in differential diagnoses.Case presentationA 19-year-old female with no psychiatric history presented to a district general hospital with acute psychosis, emotional lability, memory deficit, fluctuating behavioral changes such as wandering and babbling, and seizure. She was admitted to the hospital with a provisional diagnosis of dissociative disorder. Soon after admission, she developed aspiration pneumonia and was intubated for mechanical ventilation. She was transferred to our hospital for further assessment and admitted to the intensive care unit for ventilation. Laboratory test results were unremarkable, but her EEG showed non-specific slowing with no epileptiform activity, and brain computed tomography (CT) and MRI also showed no remarkable findings. Cerebrospinal fluid (CSF) analysis showed an elevated white blood cell count (15 cells/hpf; 70 % lymphocytes), and blood serum and CSF samples tested positive for NMDA receptor antibodies. Abdominal contrast-enhanced CT revealed an ovarian teratoma, which was subsequently removed laparoscopically. Postoperative immunotherapy (steroids, intravenous immunoglobulin, and plasmapheresis) led to gradual improvement. On day 25 of hospitalization, neuropsychological assessment demonstrated that overall, she had returned to her premorbid level of functioning. Her condition substantially improved over several months of cognitive rehabilitation, and she was eventually discharged on day 75.ConclusionsAnti-NMDA receptor encephalitis, a form of autoimmune encephalitis, is commonly associated with tumors and often misdiagnosed. Diagnosis can be confirmed by detecting NMDA receptor antibodies in the patient’s serum or CSF. Management can be achieved with immunosuppressive therapy and tumor resection.

A young child of anti-NMDA receptor encephalitis presenting with epilepsia partialis continua: the first pediatric case in Korea.

Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Briefly, a 3-year-old girl was admitted to the hospital due to right-sided, complex partial seizures without preceding febrile illness. The seizures evolved into epilepsia partialis continua and were accompanied by epileptiform discharges from the left frontal area. Three weeks after admission, the patient's seizures were reduced with antiepileptic drugs; however, she developed sleep disturbances, cognitive decline, noticeable oro-lingual-facial dyskinesia, and choreoathetoid movements. Anti-NMDAR encephalitis was confirmed by positive detection of NMDAR antibodies in the patient's serum and cerebrospinal fluid, and her condition slowly improved with immunoglobulin, methylprednisolone, and rituximab. At present, the patient is no longer taking multiple antiepileptic or antihypertensive drugs. Moreover, the patient showed gradual improvement of motor and cognitive function. This case serves as an example that a diagnosis of anti-NMDAR encephalitis should be considered when children with uncontrolled seizures develop dyskinesias without evidence of malignant tumor. In these cases, aggressive immunotherapies are needed to improve the outcome of anti-NMDAR encephalitis.

Acute Bulbar Palsy as Presentation of Anti-NMDA Receptor Encephalitis: A Case Report (P4.064)

Acute Bulbar Palsy as Presentation of Anti-NMDA Receptor Encephalitis: A Case Report (P4.064)

Anti-NMDA Receptor Encephalitis Presenting with Atrial Fibrillation (P4.023)

Anti-NMDA Receptor Encephalitis Presenting with Atrial Fibrillation (P4.023)

Total Intravenous Anesthesia Using N-Methyl-D-Aspartate (NMDA) Receptor-Sparing Drugs in a Patient with Anti-NMDA Receptor Encephalitis

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a recently identified syndrome characterized by psychosis and dysautonomia. Treatment includes resection of the underlying tumor. While the pathologic mechanism involves disruption of NMDA function by anti-NMDA receptor autoantibodies, there are few descriptions of the perioperative management or anesthetic approach for such patients. We report a classic presentation of anti-NMDA receptor encephalitis and describe the use of total IV anesthesia with NMDA receptor-sparing drugs. Modest postoperative analgesic requirements, not reported in prior cases, are also described in our report.