ABSTRACT
Anti NMDA receptor encephalitis is autoimmune encephalitis where antibodies are directed against NMDA receptor subunit. It represents a new category of immune-mediated disorder that is often paraneoplastic, presenting with neuropsychiatric symptoms, which is treatable and can be diagnosed serologically affecting both children and adults. Patient can have variable clinical presentation ranging from prodromal illness, neuropsychiatric symptoms, seizures, autonomic instability, hyperkinesias, catatonia, hypoventilation and with or without an associated teratoma. A positive serum or CSF sample screening for antibodies to the NMDA receptor subunit is confirmative. Supportive findings include CSF study, EEG and MRI Brain. The first-line therapies includes IVIG, corticosteroids or plasma exchange. Second line immunotherapy is rituximab or cyclophosphamide or both. Given the high mortality rate (up to 25%), the likelihood of presentation across the age range and the potential for treatment, a high index of suspicion is warranted by clinicians. Authors report a case of a 5 year old child with anti NMDA receptor encephalitis who responded well to IVIG therapy.
ABSTRACT
Ovarian teratoma is agynecological disease,which has been found to be closely related to antiNMDA receptor encephalitis in recent years.Anti-NMDA receptor encephalitis is a kind of autoimmune encephalitis.Its etiology,pathogenesis,diagnosis and treatment are still not clear,and the disease has various clinical manifestations and special test methods,which are often ignored by clinicians.The prognosis is poor if the best treatment timing is missed,so early treatment is very important.At present,the main treatment methods include surgical treatment and immunotherapy.Most people can be cured,but the disease has a certain recurrence rate.The recurrence mechanism and treatment need further investigation.Reports on maternal anti-NMDAR encephalitis are rare,but we need to pay lots of attention to it as it is related to maternal and fetal outcomes.
ABSTRACT
OBJETIVOS: Relatar dois casos com apresentações diferentes de encefalite antirreceptor N-metil-D-aspartato (NMDA), uma doença autoimune recentemente identificada e caracterizada por alterações da consciência, déficit de memória, convulsões, disfunção autonômica e distúrbios do movimento. DESCRIÇÃO DOS CASOS: Criança de seis anos, sexo feminino, que se apresentou com movimentos distônicos e coreoatetósicos incapacitando a marcha. Adolescente de 17 anos, sexo masculino, com alteração do comportamento, amnésia retrógrada e convulsões. Ambos realizaram eletroencefalograma e ressonância magnética cerebral que não revelaram alterações relevantes. Os anticorpos antirreceptor NMDA foram positivos no líquido cefalorraquidiano em ambos os casos e no sangue no primeiro paciente. Foram administrados metilprednisolona, imunoglobulina e rituximab em diferentes intervalos. Ambos tiveram uma recidiva cerca de seis meses depois, com recuperação ao final de um ano e meio após o diagnóstico. CONCLUSÕES: A encefalite antirreceptor NMDA deve ser considerada quando estamos perante o início súbito de sintomas neuropsiquiátricos. O diagnóstico e terapêutica precoces são fatores prognósticos fundamentais.
AIMS: To report two cases with different presentations of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, a newly identified autoimmune disease characterized by consciousness changes, memory deficit, seizures, autonomic dysfunction and movement disorders. CASES DESCRIPTION: A six-year-old female, who presented with dystonic and choreoathetoid movements with refusal to walk. A 17-yearold male, presented with behavioral changes, retrograde amnesia and seizures. Electroencephalogram and brain magnetic resonance imaging did not show any significant findings. Anti-NMDAR antibodies were positive in cerebrospinal fluid in both cases and in serum in the first patient. Methylprednisolone, immunoglobulin and rituximab were given at different intervals. Both had a recurrence about six months later, with recovery at the end of one and a half year of the diagnosis. CONCLUSIONS: Anti-NMDAR encephalitis should be considered in patients with sudden onset of neuropsychiatric symptoms. Early diagnosis and treatment are major prognostic factors.
Subject(s)
Humans , Male , Female , Child , Adolescent , Autoimmune Diseases of the Nervous SystemABSTRACT
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.
Subject(s)
Female , Humans , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Dyskinesias , Encephalitis , Encephalitis, Herpes Simplex , Herpes Simplex , Korea , Memory , Seizures , Simplexvirus , TeratomaABSTRACT
An extra-ovarian teratoma has been reported in a few cases of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. A 25-year-old woman presented with psychiatric symptoms. We did not find an ovarian teratoma on initial examination, and her initial simple chest X-ray was normal. We incidentally found an abnormality on follow-up simple chest X-ray and diagnosed an anterior mediastinal teratoma. Therefore, in patients with suspected anti-NMDAR encephalitis, even if simple chest X-ray is normal, chest computed tomography should be performed to investigate a hidden teratoma.
Subject(s)
Adult , Female , Humans , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Encephalitis , Follow-Up Studies , Teratoma , ThoraxABSTRACT
Objective@#To have a profound understanding of anti-N-methyl-D-aspartic receptor (anti-NMDAR) encephalitis, through the clinical analysis of 5 cases of anti-NMDAR encephalitis, and literature review.@*Methods@#This is a retrospective analysis. Five cases of anti-NMDA receptor encephalitis treated from May 2010 to June 2015, in the Department of Neurology, Beijing Friendship Hospital affiliated to Capital Medical University, were included in this study. The clinical data, including clinical manifestation, past history, radiological features, serum and cerebral spinal fluid examinations, treatment and prognosis, were analyzed.@*Results@#Among the 5 cases, 3 young female and 2 middle-to old-aged male. The clinical features of the onset was mental and behavior disorder, as well as seizure and extrapyramidal features, like facial and limbic involuntary movements or tremor. Coma and hypopnea was severe in 3 young female cases, needing assistance of mechanical ventilator, while the manifestation of 2 male patients was much mild, need not assisted respiration. 1 case had teratoma of ovary, 1 case had Vogt-Koyanagi-Harada syndrome. The anti-NMDA receptor antibody was positive in cerebraospinal fluid of all 5 cases, but in serum of 3 cases, serum and CSF Epstein-Barr virus (EBV) IgM antibody was positive in 1 case, while herpes simplex I virus (HSV-1) IgM antibody positive in another case, and anti-myelin oligodendrocyte glycoprotein (MOG) antibody was seen in serum and CSF in 1 case. The time interval from the onset to treatment was 10-37 d (18.8±9.8 d). IVIG was used in all of the 5 cases, glucocoticoid in 4 cases, and plasma exchange in 3 cases. One case with Vogt-Koyanagi-Harada syndrome, having a long time before diagnosis and treatment, died, while the other 4 cases had good prognosis, and had no relapse.@*Conclusions@#Mental and behavior disturbance is common at onset of anti-NMDAR encephalitis. The radiological and lab examination may be normal. It may be accompanied with HSV-1 or EBV infection, anti-MOG antibody may be positive in this disease. Active treatment is important.
ABSTRACT
Systemic lupus erythematosus (SLE)is an autoimmune disease,involving multiple organs and systems,and it is defined as neuropsychiatric systemic lupus erythematosus (NPSLE) when neuropsychiatric symptoms appear.The N-methyl-D-aspartate (NMDA) receptor is a kind of membrane-spanning ion channels' glutamate receptors,which mainly contains NR1 subunit and NR2 subunit.In recent years,the role of anti-NMDA-receptor antibodies in the pathogenesis of NPSLE has become a research hotspot,especially for anti-NR2 antibody.Therefore we review the research progress of the mechanism of the anti-NMDA-receptor antibodies in NPSLE in this paper.
ABSTRACT
Abstract Anti-N-methyl-D-aspartate receptor encephalitis is a recently described neurological disorder and an increasingly recognized cause of psychosis, movement disorders and autonomic dysfunction. We report 20-year-old Chinese female who presented with generalized tonic-clonic seizures, recent memory loss, visual hallucinations and abnormal behavior. Anti-N-methyl-D-aspartate receptor encephalitis was diagnosed and a computed tomography scan of abdomen reviewed a left adnexal tumor. We describe the first such case report of a patient with anti-N-methyl-D-aspartate receptor encephalitis who was given a bilateral transversus abdominis plane block as the sole anesthetic for removal of ovarian tumor. We also discuss the anesthetic issues associated with anti-N-methyl-D-aspartate receptor encephalitis. As discovery of tumor and its removal is the focus of initial treatment in this group of patients, anesthetists will encounter more such cases in the near future.
Resumo A encefalite antirreceptor de N-metil-D-aspartato (NMDA) é um distúrbio neurológico recentemente descrito e uma causa cada vez mais reconhecida de psicose, distúrbios do movimento e disfunção autonômica. Relatamos o caso de uma paciente de origem chinesa, de 20 anos, que se apresentou com crises tônico-clônicas generalizadas, perda de memória recente, alucinações visuais e comportamento anormal. Encefalite antirreceptor de NMDA foi diagnosticada e uma tomografia computadorizada de abdome revelou um tumor anexial à esquerda. Descrevemos o primeiro relato de caso de paciente com encefalite antirreceptor de NMDA submetida ao bloqueio de plano transverso abdominal (PTA) bilateral como única anestesia para remoção de tumor ovariano. Também discutimos as questões anestésicas associadas à encefalite antirreceptor de NMDA. Como a descoberta e a remoção do tumor são o foco do tratamento inicial nesse grupo de pacientes, os anestesiologistas encontrarão mais desses casos no futuro próximo.
Subject(s)
Humans , Male , Young Adult , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/complications , Anesthesia, General/standards , Ovarian Neoplasms/surgery , Ovarian Neoplasms/complications , Nerve BlockABSTRACT
We report a case of a young female patient with catatonic features who later turned out to be suffering from an anti-NMDA (N-methyl-D-aspartate)-receptor-antibody encephalitis. A previously healthy 21-year-old woman was admitted to psychiatric inpatient care presenting with acute psychotic behavior with catatonic features. Laboratory tests of serum and CSF (Cerebrospinal fluid), EEG (Electroencephalogram), brain MRI (Magnetic Resonance Imaging) were unremarkable except vague slow wave on EEG. However, subtle cognitive impairment at the bedside examination suggested further imaging studies to rule out possible organic etiology like autoimmune encephalitis. Brain PET (Positron Emission Tomography) and SPECT (Single Photon Emission Computed Tomography) suggested probable inflammation in the brain. In case of autoimmune encephalitis, given the severity of symptoms and worsening course, steroid pulse therapy was initiated promptly even though the diagnosis was not confirmed but presumed at that time. She recovered completely with steroid therapy. Later her disease turned out to be anti-NMDA-receptor-antibody encephalitis by the antibody test which was not available at the time of admission. Psychiatrists need to be aware of autoimmune encephalitis like anti-NMDA-receptor-antibody encephalitis in the differential diagnosis of acute psychosis with catatonic features. Subtle cognitive impairment which tends to be overlooked due to catatonic features might be a clue to suspect the organic etiology.
Subject(s)
Female , Humans , Young Adult , Brain , Catatonia , Cognition Disorders , Diagnosis , Diagnosis, Differential , Electroencephalography , Encephalitis , Glycogen Storage Disease Type VI , Inflammation , Inpatients , Magnetic Resonance Imaging , N-Methylaspartate , Psychiatry , Psychotic Disorders , Tomography, Emission-Computed, Single-PhotonABSTRACT
Resumen:La encefalitis por anticuerpos antirreceptor N-metil-D-Aspartato pertenece a un grupo de enfermedades que inicialmente se llamaban encefalitis límbicas, hoy conocidas como encefalitis autoinmunes. Se caracterizan por estar frecuentemente asociadas a algún tumor, responder a la inmunoterapia y provocar daños cerebrales variables que se manifiestan como cuadros clínicos polimorfos (desde la disfunción límbica hasta una encefalopatía multifocal o difusa). Algunos pacientes con condiciones psiquiátricas agudas podrían tener en realidad una encefalitis autoinmune, sin embargo, no es tan fácil sospecharlo con las manifestaciones iniciales. Su incidencia exacta no se conoce, pero cada vez hay más reportes de casos. Sus características clínicas están bien descritas y evolucionan en cinco fases. El diagnóstico definitivo se hace al obtener la confirmación serológica de los anticuerpos, pero hay algunas pruebas que pueden orientar la sospecha diagnóstica. El tratamiento se basa en esteroides y otros inmunosupresores.
Abstract:Anti NMDA-receptor encephalitis belongs to a group of diseases originally called limbic encephalitis, nowadays known as autoimmune encephalitis. These encephalitis are characterized by a frequent association with a tumor, a good response to immunotherapy, and by causing a broad range of brain injuries which manifest in polymorphous clinical pictures (from limbic dysfunction to multifocal or diffuse encephalopathy). Some patients diagnosed with acute psychiatry conditions may have instead autoimmune encephalitis. Nevertheless, it is not easy to discern one from the other in the initial stages. Its exact incidence is unknown, but there are increasingly more case reports. Its clinical characteristics are well described and evolve in five stages or phases. The final diagnosis is made when antibodies serologic confirmation is obtained, but some other tests may help guide the diagnosis. The treatment is based on steroids and other immunosuppressants.
Subject(s)
Humans , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/diagnosis , Encephalitis , Neuropsychiatry , Costa RicaABSTRACT
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.
A encefalite antirreceptor N-metil-D-aspartato (NMDA) é uma doença autoimmune recentemente descoberta, na qual anticorpos têm como alvo os receptors NMDA no cérebro, levando à sua remoção da sinapse. Os pacientes frequentemente desenvolvem psicose e distúrbios de humor proeminentes nas fases iniciais da doença (i.e: mania e depressão), o que explica o fato de que a maioria destes pacientes seja vista primeiramente por psiquiatras. Consequentemente, as encefalites autoimunes têm recebido atenção crescente pela psiquiatria, principalmente pela preocupação de erroneamente diagnosticar doenças imunomediadas e potencialmente tratáveis como doenças psiquiátricas primárias, como esquizofrenia ou transtorno depressivo maior. Embora a encefalite antirreceptor NMDA ocorra no contexto de sintomas psiquiátricos de início recente, há carência de informações sobre o diagnóstico diferencial e tratamento desta doença após o diagnóstico de doenças psiquiátricas funcionais de longa data. Aqui, nós apresentamos o caso de uma paciente com diagnóstico de transtorno afetivo bipolar de longa data evoluindo com encefalite antirreceptor NMDA, inicialmente diagnosticada erroneamente como uma psicose não-orgânica.
Subject(s)
Humans , Psychotic Disorders , Recurrence , Bipolar Disorder , N-Methylaspartate , Anti-N-Methyl-D-Aspartate Receptor EncephalitisABSTRACT
Objective To summarize and analysis the clinical features, diagnosis and treatment of the cases which were positive for anti-N-methyl-D-aspartic acid (NMDA) receptor antibodies by indirect immunofluorescence assay (IFA). Methods We analyzed the disease process, clinical characteristics, auxiliary examination , diagnosis, treat-ment, and prognosis of five cases positive for anti NMDA receptor antibodies in their serum and cerebrospinal fluid (CSF). Results Four of the five cases positive for anti-N-methyl-D-aspartic acid (NMDA) receptor antibodies were di-agnosed with anti-NMDA receptor encephalitis and one was diagnosed with Herpes Simplex Virus Encephalitis(HSE). The five cases had a similar disease presentation including prodromal flu-like symptoms in three cases and psychiatric symptoms at onset in three cases. Four cases developed epilepsy and respiratory failure during the disease course and received treatment in the NICU. Four cases had movement disorders during the late stage of isease..Electroencephalo-graphs and brain MRI showed abnormalities in most cases. The virus infection and dysimmunity test were positive in four cases. Patients with the anti-NMDA receptor encephalitis could have a good immediate prognosis after treatment with hormone and immune globulin. However, two cases developed cancer and one case died during one year fol-low-up. Conclusion Patients with HSE may also test positive for anti-NMDA receptor antibodies. Thus, diagnosis of anti-NMDA receptor encephalitis requires a thorough evaluation including patient’s history and disease course to avoid misdiagnosis.
ABSTRACT
La Encefalitis por Anticuerpos contra el Receptor N-metil D-aspartato (NMDAR) es un trastorno grave conuna presentación inicial puramente neuropsiquiátrica, que evoluciona hacia una encefalopatía severa, y es acompañadopor movimientos involuntarios, convulsiones e inestabilidad autonómica; el curso de la enfermedadusualmente se prolonga y necesita tratamiento inmunomodulador agresivo.Se describe el caso de un paciente de 5 años con diagnóstico de Encefalitis por Anticuerpos contra el ReceptorN-metil D-aspartato (NMDAR), manejado con tratamiento inmunomodulador, logrando así control y noprogreso de enfermedad...
Anti-NMDA receptor encephalitis is a severe disorder with a dramatic clinical presentation, often showing apurely initial neuropsychiatric phase, and evolving into a severe encephalopathy accompanied by involuntarymovements, seizures and autonomic instability; the disease course is typically prolonged and needs intensivecare treatment.We report the case of a 5-year-old child, diagnosed with Anti-NMDA receptor encephalitis with immunemodulatory therapy...
Subject(s)
Humans , Antibodies , Encephalitis , N-MethylaspartateABSTRACT
Anti-NMDA receptor encephalitis is a severe but treatable autoimmune disease of the CNS. However, the use of immunotherapy and long-term outcomes have yet to be defined for this disease. We describe a case of an 18-year-old male diagnosed with anti-NMDAR encephalitis not associated with tumor, which did not respond to initial treatment with immunoglobulin, followed by corticosteroids, cyclophosphamide and evolved with significant clinical improvement after a prolonged course of plasmapheresis. Although it is not possible to affirm the good outcome was due solely to the prolonged plasmapheresis regimen, recently published data shows that improvement may take weeks or months to occur. This case discloses another therapeutic possibility for patients with refractory disease who fail to respond to recommended first-line and second-line therapy.
A encefalite antirreceptor de NMDA é uma grave doença autoimune do sistema nervoso central, mas responsiva a tratamento. No entanto o uso da imunoterapia e o prognóstico a longo prazo ainda não foram estabelecidos para esta doença. Aqui nós descrevemos o caso de um homem de 18 anos de idade diagnosticado com encefalite antirreceptor de NMDA, não associada a tumor, o qual provavelmente não respondeu à terapia inicial com corticosteroides, ciclofosfamida e, o qual evoluiu com melhora clínica importante após um curso prolongado de plasmaférese. Embora não seja possível atribuir o desfecho favorável apenas ao regime de plasmaférese prolongado, uma vez que publicações recentes demonstram que a resposta à imunoterapia pode levar semanas à meses, nosso caso abre uma possibilidade terapêutica nova para casos com doença refratária, não responsivos ao tratamento de primeira e segunda-linha preconizados
Subject(s)
Humans , Autoimmune Diseases , Plasmapheresis , Encephalitis , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Mental DisordersABSTRACT
Los síndromes paraneoplásicos se caracterizan por la presencia de signos y síntomas que ocurren lejos del sitio del tumor o de sus metástasis. Muchos de estos síndromes son mediados por hormonas, citoquinas, u otras sustancias secretadas por células tumorales, así como por una respuesta inmune contra el tumor. La presencia de anticuerpos generados para combatir el tumor puede resultar en un síndrome neurológico si las células del sistema nervioso son afectadas. Las primeras descripciones de tales casos datan más de 20 años, cuando los anticuerpos anti-Hu y anti-Yo fueron descritos. Existe una variedad de síndromes paraneoplásicos, incluyendo cuadros neurológicos clásicos y diversos. En este artículo de revisión hemos querido enfatizar un cuadro neuropsiquiátrico que corresponde a un síndrome paraneoplásico descrito recientemente, aunque también puede ocurrir en pacientes sin presencia tumoral aparente. Se trata de la encefalitis asociada a anticuerpos contra los receptores N-metil-D-aspartato (NMDA). Nuestro interés en dicho síndrome, como especialistas en trastornos del movimiento, se basa en la presencia común de movimientos anormales en esta enfermedad. Sin embargo los pacientes afectados, la mayoría mujeres con teratoma ovárico, presentan un cuadro mixto, psiquiátrico inicialmente, seguido por epilepsia, empeoramiento de conciencia, disautonomía y distonía orofacial y truncal. Como resultado, la relevancia de la encefalitis asociada a anticuerpos contra los receptores NMDA abarca una variedad de especialidades incluyendo psiquiatría, neurología, oncología, inmunología, radiología, medicina general y cirugía.
As early as the late 1990s, a severe and potentially fatal multi-faceted disease, occurring predominantly in women, was reported in Japan. The earliest patients presented with psychosis or memory problems which rapidly progressed into numerous neurological complications which required intensive care. There were many different descriptions and labels attributed to the disease, thus delaying its classification as a paraneoplastic syndrome. In early 2007, this disease was characterized as an acute form of encephalitis-termed anti-N-methyl-D-aspartate (NMDA) encephalitis.