scholarly journals A refractory anti-NMDA receptor encephalitis successfully treated by bilateral ovariectomy and intrathecal injection of methotrexate and dexamethasone: A case report

2019 ◽  
Author(s):  
Dongmei Wang ◽  
Yongming Wu ◽  
Zhong Ji ◽  
Shengnan Wang ◽  
Yunqi Xu ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Intrathecal Injection ◽  
Young Female ◽  
Good Prognosis ◽  
Intravenous Methylprednisolone ◽  
Case Presentation ◽  
Bilateral Ovariectomy ◽  
Nmda Receptor Encephalitis ◽  
Intravenous Methylprednisolone Pulse ◽  
New Treatments

Abstract Background: Anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis is an autoimmune-mediated disease, which is predominant in young female patients with ovarian teratomas. With proper immunotherapy, most of the patients achieve good prognosis. Nevertheless, some patients may be refractory to first and second-line immunotherapy, thus new treatments are required to help these patients. Case presentation: We present a case of anti-NMDA receptor encephalitis with ovary teratoma. After the prompt removal of the teratoma and strong immunotherapy including intravenous methylprednisolone pulse (IVMP), intravenous immunoglobin (IVIG), plasmapheresis, immunoadsorption, intravenous cyclophosphamide, and rituximab, the patient’s neurologic status did not improve. Bilateral ovariectomy was then conducted and intrathecal injection of methotrexate (MTX) and dexamethasone (DXM) was given. The patient’s neurological symptoms improved dramatically， and she achieved a good prognosis after 23 months. Conclusions: For refractory cases of anti-NMDA receptor encephalitis, intrathecal injection of MTX and DXM may be beneficial. More research is required to elucidate the mechanisms of intrathecal treatment.

scholarly journals A refractory anti-NMDA receptor encephalitis successfully treated by bilateral salpingo-oophorectomy and intrathecal injection of methotrexate and dexamethasone: a case report

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052092566 ◽  
Author(s):  
Dongmei Wang ◽  
Yongming Wu ◽  
Zhong Ji ◽  
Shengnan Wang ◽  
Yunqi Xu ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Intrathecal Injection ◽  
Young Female ◽  
Good Prognosis ◽  
Ovarian Teratoma ◽  
Intravenous Methylprednisolone ◽  
Intravenous Cyclophosphamide ◽  
Case Presentation ◽  
Nmda Receptor Encephalitis ◽  
Intravenous Methylprednisolone Pulse

Introduction Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is an autoimmune-mediated disease that is common in young female patients with ovarian teratomas. With appropriate immunotherapy, most patients achieve a good prognosis. Nevertheless, some patients may be refractory to first- and second-line immunotherapy, thus alternative treatments are required for these patients. Case presentation: We present a case of anti-NMDA receptor encephalitis with ovarian teratoma. After the prompt removal of the teratoma and intense immunotherapy was administered, including an intravenous methylprednisolone pulse, intravenous immunoglobin, plasmapheresis, immunoadsorption, intravenous cyclophosphamide, and rituximab, the patient’s neurologic status did not improve. Bilateral salpingo-oophorectomy was then conducted, and intrathecal injection of methotrexate (MTX) and dexamethasone (DXM) was performed. The patient’s neurological symptoms improved dramatically, and she achieved a good prognosis after 23 months. Conclusions Intrathecal injection of MTX and DXM may be beneficial for treatment of refractory cases of anti-NMDA receptor encephalitis. Additional research is required to elucidate the mechanisms of intrathecal treatment with this therapy.

scholarly journals Case Presentation of Anti-NMDA Receptor Encephalitis in a 4-Year-Old Boy

2017 ◽  
Vol 64 (4) ◽  
pp. 352-354
Author(s):  
Daniel A Gbadero ◽  
Emmanuel O Adegbite ◽  
Jean-Baptist LePichon ◽  
Tina M Slusher
Keyword(s):  
Nmda Receptor ◽  
Case Presentation ◽  
Nmda Receptor Encephalitis

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

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Maria Stavrou ◽  
Jing Ming Yeo ◽  
Alexander David Slater ◽  
Oliver Koch ◽  
Sarosh Irani ◽  
...  
Keyword(s):  
Case Report ◽  
Nmda Receptor ◽  
Rapid Development ◽  
Clonic Seizure ◽  
Ovarian Teratoma ◽  
Caucasian Woman ◽  
Case Presentation ◽  
Nmda Receptor Encephalitis ◽  
Autonomic Instability ◽  
Lymphocytic Meningitis

Abstract Background Meningitis 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 presentation This 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. Conclusion Clinicians 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.

scholarly journals A case report of complement C4B deficiency in a patient with steroid and IVIG-refractory anti-NMDA receptor encephalitis

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Gilbert T. Chua ◽  
Danlei Zhou ◽  
Alvin Chi Chung Ho ◽  
Sophelia Hoi Shan Chan ◽  
Chack Yung Yu ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Intravenous Immunoglobulin ◽  
Clinical Improvement ◽  
Immune Complexes ◽  
Index Patient ◽  
Behavioral Changes ◽  
Case Presentation ◽  
Pulse Methylprednisolone ◽  
Nmda Receptor Encephalitis ◽  
Low Levels

Abstract Background Complement C4A or C4B deficiency has never been reported in autoantibody-associated encephalitides patient. Here we present a case of anti-N-methyl- D-aspartate (NMDA) receptor encephalitis associated with homozygous C4B deficiency, who did not respond to intravenous immunoglobulin and pulse methylprednisolone but plasmapheresis and rituximab. Case presentation A fourteen-year-old boy presented to our unit with subacute onset of behavioral changes and confusion, and was later confirmed to be anti-NMDA receptor encephalitis. He was initially managed with intravenous immunoglobulin (IVIG) and pulse methylprednisolone but did not achieve any clinical improvement. Seven sessions of plasmapheresis was commenced with remarkable improvement after the second session, and was followed by four doses of rituximab. His neurological and cognitive functioning gradually returned to baseline. Immunological investigations demonstrated persistently low C4 levels below 8 mg/dL. A more in-depth complement analysis of the patient and his family showed that he has homozygous C4B deficiency. Genetic analysis revealed that the index patient has homozygous deficiency in complement C4B and he carries one non-functioning mutant C4B gene inherited from his mother. Conclusions Low levels of serum C4 correlate with reduced functions of the classical and lectin pathways, leading to the impairment of immune-complexes removal. Plasmapheresis ameliorates complement deficiency and removes the offending immune-complexes leading to clinical improvement that was not achieved by IVIG and steroids. We postulate that serum C4 levels may serve as a biomarker for the need of plasmapheresis upfront rather than only after non-response to steroid and IVIG in treating anti-NMDA-receptor encephalitis.

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.

Sign in / Sign up

Export Citation Format

Share Document