Subacute sclerosing panencephalits mimicking anti-NMDA receptor encephalitis
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Anti-NMDA Receptor Encephalitis
Introduction Anti-N-methyl-D-aspartate receptor (anti-NMDA-R) encephalitis is well-characterised autoimmune encephalitis with prominent psychiatric manifestations, neurological manifestations like speech dysfunction, seizures, dyskinesias and other movement abnormalities, decreased level of consciousness and autonomic instability. This disorder affects primarily children and adults up to 45 years. Females are 4 times more common than males and may have association with ovarian teratoma. Objectives To identify anti-NMDA receptor encephalitis based on clinical features, facilitate early screening and relevant investigations to prevent delay in treatment. Methods A case study of 36 year old female presented with clinical manifestations of autoimmune encephalitis syndrome. Results Diagnosis confirmed by presence of NMDA receptor antibodies in serum and cerebrospinal fluid. Conclusions Early recognition of clinical features of Anti-NMDA receptor encephalitis and early initiation of treatment has shown to improve outcomes, speed recovery and reduce the risk of relapses. Disclosure No significant relationships.
Anti-NMDA Receptor Encephalitis
Autoimmune encephalitis
Ovarian Teratoma
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Introduction: Anti-NMDA receptor encephalitis is one of many autoimmune diseases that need further recognization. Its signs and symptoms are indefinite and there is no specific pathognomic sign that can be use to properly diagnosed the disease immediately. Therefore more often these patients are prone to misdiagnosis. Many reports claimed that it is directly associated with ovarian teratomas in women though in some reports it can also be seen in males. This case helps in further recognizing anti-NMDA receptor encephalitis especially in our country where there are only few cases reported. Case: The case presented is about an 18-year old female from Mindoro Philippines who was previously well and had sudden changes in behavior with seizures episodes. Patient had consult at their local hospital where she was being treated as a case of viral meningitis but apparently no improvement was noted and subsequently transferred to a tertiary hospital in Metro Manila for further evaluation and management. During her hospital stay at Amang Rodriguez Memorial Medical Center patient presented as seizures with abnormal psychiatric features where she was eventually suspected to have anti-NMDA (anti-N-methyl-D-aspartate) receptor encephalitis but further testing was done initially to rule out other neurologic diseases. Almost all laboratories and radiologic tests turn out to be normal hence a video electroencephalogram (EEG) was requested and result was that the patient is suggestive to have the said disease. Definitive test for anti-NMDA receptor encephalitis was done which turned out positive. The patient was then treated as a case of anti-NMDA receptor encephalitis and manage accordingly. Improvement was noted and the patient was then discharged subsequently. Conclusion: There may be increasing cases of anti-NMDA receptor encephalitis worldwide further research must still be done to be able to understand its features diagnose and manage it appropriately. And since it is more common in young adult females anti-NMDA receptor encephalitis must be suspected and be taken into consideration when there is a patient who presented with sudden changes in behavior with psychosis and seizures.
Anti-NMDA Receptor Encephalitis
Autoimmune encephalitis
Viral encephalitis
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Autoimmune encephalitis
Anti-NMDA Receptor Encephalitis
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Recent clinical management of anti-NMDA receptor encephalitis is reviewed. This illness is required the management of the neurological emergency. Typical symptoms of anti-NMDA receptor encephalitis develop in several stages 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 respiratory instability. The diagnosis is depended on the detection of the NMDA receptor antibody in CSF or serum under the above characteristic symptoms of encephalitis. The disorder predominantly affects children and young adults, occurs with or without tumor association. The presence of a tumor (usually an ovarian teratoma) is dependent on age and sex, being more frequent in women older than 18 years. Anti-NMDA receptor encephalitis should be treated with tumor resection and immunotherapy (corticosteroids, intravenous immunoglobulin, or plasma exchange) responded faster to treatment and less frequently needed second-line immunotherapy (cyclophosphamide or rituximab, or both).
Anti-NMDA Receptor Encephalitis
Catatonia
Autoimmune encephalitis
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Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis has clinical features of psychiatric symptoms, loss of memory, seizure, dyskinesia and autonomic dysfunction. While Anti-NMDA receptor encephalitis was initially reported in young women with ovarian teratoma, viral infections can trigger anti-NMDA receptor encephalitis as well. Among them, herpes virus is the most common. We report a patient who developed the anti-NMDA receptor encephalitis 47 days after herpes virus encephalitis, which is, to our knowledge, the first case in Korea. Key Words: Anti-NMDA receptor encephalitis, Herpes encephalitis
Anti-NMDA Receptor Encephalitis
Viral encephalitis
Autoimmune encephalitis
Ovarian Teratoma
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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.
Anti-NMDA Receptor Encephalitis
Memantine
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Introduction: An anti-NMDA-receptor encephalitis is a severe, more and more diagnosed form of encephalitis with characteristic clinical features. Anti-NMDA-receptor encephalitis had initially been described in young women with ovarian teratoma, but is also common in children and without neoplasm.
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Aims: The aim of this case report is to highlight the frequently overlooked association between Anti-NMDA Receptor Encephalitis and a preceding Herpes Simplex Viral Encephalitis trigger, the clinical settings under which to suspect this autoimmune disease, the need for appropriate workup to clinch the diagnosis and the necessity of speedy initiation of immunotherapy, in a disease where timely treatment is paramount.
Presentation of Case: A middle aged woman presented with a history of fever and syncope, followed by slurring of speech, quadriparesis, seizures and altered sensorium. She was diagnosed with HSV-1 Encephalitis on basis of CSF studies and MRI brain. Despite a timely 28-day course of intravenous acyclovir therapy, she showed incomplete clinical recovery. A serum and CSF autoantibody panel was performed, which clinched a diagnosis of Anti-NMDA Receptor Encephalitis. A course of steroids and IVIg was given and the patient was discharged after showing clinical improvement.
Discussion: Anti-NMDA Receptor Encephalitis is an autoimmune encephalitis characterized by complex neuropsychiatric features and presence of IgG antibodies against NR1 subunit of NMDA receptor detectable in CSF and serum. It is associated with various malignancies, chiefly ovarian teratomas, and with HSV-1 viral encephalitis. Diagnosis involves autoantibody detection in CSF or serum and first line treatment is with steroids, IVIg or plasma exchange, and tumour resection.
Conclusion: Prompt diagnosis of Anti-NMDA receptor encephalitis is crucial as it enables treatment with timely immunosuppression and tumour resection. This disease must be suspected in adults or children presenting with subacute onset of neuropsychiatric symptoms, with CSF lymphocytic pleocytosis and presence of autoantibodies to NMDA receptor in CSF or serum. Early treatment is associated with good outcomes.
Autoimmune encephalitis
Anti-NMDA Receptor Encephalitis
Immunosuppression
Viral encephalitis
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Anti-NMDA Receptor Encephalitis
Pleocytosis
Autoimmune encephalitis
Convulsion
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N-methyl-D-aspartate (NMDA) receptor antibody encephalitis is an immunotherapy-responsive panencephalitis. Patients usually present with characteristic clinical features in a specific order. We describe a patient who developed anti NMDA receptor encephalitis with a positive liquor EBV titer, suggesting formation of antibodies including anti NMDA. After a prolonged ICU stay the patient had a slow but full recovery. Despite severe neurological symptoms, in general, the prognosis of anti NMDA receptor encephalitis is good and warrants prolonged intensive care treatment when indicated.
Anti-NMDA Receptor Encephalitis
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