scholarly journals A 24-year-old female with anti-N-methyl-D-aspartate receptor encephalitis: a case report

2021 ◽  
Author(s):  
Antoine Steeman ◽  
Ionut-Adrian Andriescu ◽  
Guy Mazairac
Keyword(s):  
Case Report ◽  
Nmda Receptor ◽  
Autoimmune Encephalitis ◽  
Disease Diagnosis ◽  
Young Female ◽  
Psychiatric Disease ◽  
Tumour Resection ◽  
Female Ratio ◽  
Nmda Receptor Encephalitis ◽  
Acute Confusion

Abstract Background: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a form of autoimmune encephalitis with paraneoplastic and nonparaneoplastic origin. Anti-NMDA receptor encephalitis preferentially affects children and young adults with a male/female ratio of 1/4. In case of clinical suspicion, electroencephalogram and brain magnetic resonance imaging (MRI) are useful. The diagnosis is confirmed by the detection of IgG antibodies directed against NMDA receptors. Treatment includes immunosuppression and tumour resection when indicated.Case Report: We report the case of a 24-year-old female admitted to the emergency room following the onset of acute confusion. Due to the rapid deterioration of consciousness and swallowing disorders, the patient was transferred to the intensive care unit. On day 23 after presentation, MRI suggested autoimmune limbic encephalitis. Serologic and cerebrospinal fluid results were positive for anti-NMDA antibodies. After intravenous methylprednisolone therapy and plasmapheresis and a second line therapy with corticosteroid therapy and mycophenolic acid, the patient’s clinical condition gradually improved.Why should an emergency physician be aware of this? : This disease is still underdiagnosed. The possibility of Anti-NMDA encephalitis must be excluded especially for young female adults and children presenting to emergency department with acute confusion before concluding a psychiatric disease diagnosis. Neurological deterioration typically occurs 1 to 3 weeks after the onset of symptoms.

scholarly journals A 24-Year-Old Female With Anti-NMDA Receptor Encephalitis: A Case Report.

2020 ◽  
Author(s):  
Antoine Steeman ◽  
Ionut-Adrian Andriescu ◽  
Guy Mazairac
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Nmda Receptor ◽  
Psychiatric Symptoms ◽  
Delayed Diagnosis ◽  
Young Patients ◽  
Tumour Resection ◽  
Female Ratio ◽  
Nmda Receptor Encephalitis ◽  
Initial Symptoms

Abstract Background: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a form of autoimmune encephalitis with paraneoplastic and nonparaneoplastic origin. Patients with this disease usually arrive at the intensive care unit without any diagnosis or with complications as a result of a delayed diagnosis. This disease is not well known and is underdiagnosed. Due to the variability of the initial symptoms and the lack of knowledge regarding these symptoms, anti-NMDA receptor encephalitis is not only underdiagnosed but also can be misdiagnosed as viral encephalitis or other pathologies. Anti-NMDA receptor encephalitis preferentially affects children (from 8 months) and young adults, and it has a male/female ratio of 1/4. In case of clinical suspicion, electroencephalogram (EEG) and brain magnetic resonance imaging (MRI) are useful. The diagnosis is confirmed by the detection of IgG antibodies directed against NMDA receptors in serum and CSF. Treatment for this disease includes immunosuppression and tumour resection when indicated.Case presentation: We report the case of a 24-year-old female admitted to the emergency room following the onset of acute confusion. Due to the rapid deterioration of consciousness and swallowing disorders, the patient was transferred to the intensive care unit. On day 23 after presentation, MRI suggested autoimmune limbic encephalitis. Serologic and CSF results were positive for anti-NMDA antibodies. After IV methylprednisolone therapy and plasmapheresis and a second line therapy with corticosteroid therapy and mycophenolic acid, the patient’s clinical condition gradually improved.Conclusions: Anti-NMDA receptor encephalitis typically occurs in young patients with no history of acute psychiatric symptoms. The possibility of this pathology should be taken into account before diagnosing a patient with a psychiatric illness.

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

2018 ◽  
Vol 49 (16) ◽  
pp. 2709-2716 ◽  
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).

scholarly journals RARE-36. BORTEZOMIB WOKE UP A PATIENT WITH ANTI-NMDA RECEPTOR ENCEPHALITIS REFRACTORY TO STANDARD THERAPY AND LONG TERM FOLLOW-UP

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi229-vi229
Author(s):  
Kong Xiao-Tang ◽  
Leonid Groysman ◽  
Cyrus Dastur ◽  
Beverly Fu ◽  
Daniela Bota
Keyword(s):  
Nmda Receptor ◽  
Standard Therapy ◽  
Autoimmune Encephalitis ◽  
Ivig Therapy ◽  
Term Care ◽  
Good Communication ◽  
Nmda Receptor Encephalitis ◽  
Long Term Follow Up ◽  
One Year

Abstract OBJECTIVE To report a case with refractory NMDA encephalitis in comatose for 18 months, who was treated successfully with bortezomib. BACKGROUND Anti-NMDA encephalitis is a rare autoimmune encephalitis. Standard therapy include corticosteroid, IVIG or plasma exchange, cyclophosphamide, rituximab, and tumor removal. Refractory cases are very severe and often stay in ICU on ventilation for several months to years. Bortezomib for the treatment of refractory anti-NMDA receptor encephalitis was reported. We have applied the treatment to our refractory case and successfully woke up the patient. And we have followed up the patient for 3 years. METHODS Case report. RESULTS A 40 yo male was diagnosed as anti-NMDA encephalitis. Standard therapy was applied. After stabilization, the patient was eventually discharged to ICU at a long term care subacute hospital. The patient was brought back for more Rituxan or steroid or IVIG therapy. The condition had not improved at all. Eighteen months in comatose, the patient had worsening NMDA titer in CSF to 1:640. Decision was made to start bortezumib as reported with modification: 1.3 mg/m2 bortezomib were administered on days 1, 8, 11 and 14 and allowed two weeks off therapy. After first cycle, the patent started to talk first word “hurt.” After 6 cycles, the patient sat up and started riding bicycles for physical therapy. The NMDA titer in CSF was reduced to 1:40 at the end of 6 cycles. One year later, the patent stood up and ambulated with a walker. One and half year later, the patient walks without assistance and his speech and cognition have significantly improved with good communication with family members and staff. CONCLUSIONS Proteasome inhibitor bortezomib might be considered to be the third line therapy as early as possible if the first line and second line are ineffective to treat anti-NMDA receptor encephalitis.

scholarly journals Anti-NMDA receptor encephalitis in a psychiatric Covid-19 patient: A case report

2020 ◽  
Vol 87 ◽  
pp. 179-181 ◽  
Author(s):  
Adelaide Panariello ◽  
Roberta Bassetti ◽  
Anna Radice ◽  
Roberto Rossotti ◽  
Massimo Puoti ◽  
...  
Keyword(s):  
Case Report ◽  
Nmda Receptor ◽  
Nmda Receptor Encephalitis
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