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1.
Article in English | WPRIM | ID: wpr-812989

ABSTRACT

OBJECTIVES@#To analyze the clinical characteristics and prognosis of children with anti-N-methyl--aspartate receptor (NMDAR) encephalitis and to provide a basis for early clinical identification of this disease.@*METHODS@#The clinical data of 42 cases of anti-NMDAR encephalitis at Department of Pediatrics, Second Xiangya Hospital, Central South University from January 2015 to March 2018 were collected. The clinical features and followed-up outcomes were analyzed retrospectively.@*RESULTS@#There were 15 cases (35.7%) of males and 27 cases (64.3%) of females in 42 children, with a ratio of 1꞉1.8. They were aged from 4 months to 17 years, with an average of (9.20±4.66) years. The most common initial symptoms were seizures (47.6%, 20/42) and mental behavior disorder (35.7%, 15/42). During the course of the disease, 85.7% patients(36/42) had mental and behavior disorder, 85.7% patients (36/42) had epilepsy, 76.2% (32/42) had speech disorder, 66.7% patients (28/42) had dyskinesia, 66.7% patients (28/42) had the decreased level of consciousness, 61.9% patients (26/42) had autonomic instability, and 57.1% (24/42) patients had sleep disorder. All the children had positive antibody against NMDA receptor resistance encephalitis in cerebrospinal fluid. Head MRI showed the abnormal incidence was 50.0% (21/42), and the lesions involved in parietal lobe, frontal lobe, temporal lobe, occipital lobe, midbrain, thalamus, basal ganglia and optic nerve. There was a patient with optic nerve damage combined with myelin oligodendrocyte glycoprotein (MOG) antibody positive. Forty cases were examined by electroencephalogram (EEG), 92.5% cases (37/40) were abnormal, mainly showing diffuse slow waves, and δ brushes could be seen in severe cases. And there was 1 patient (2.4%) complicated with mesenteric teratoma. The mRS score (2.14±1.46) at discharge was significantly lower than the highest mRS score (3.88±1.38) during hospitalization (<0.05). After 3-39 months of follow-up, mRS score at 3 months after discharge was only 0.81±1.29, which was still improved compared with that at discharge, 76.2% cases (32/42) experienced complete or near-complete recovery (mRS score≤2), and 4.8% (2/42) cases relapsed. There was no mortality; the initial time of immunotherapy and the highest mRS score in the course of the disease were the factors affecting the prognosis. The earlier the starting time for immunotherapy and the lower mRS score in the course of the disease were, the better the prognosis was.@*CONCLUSIONS@#Seizures, mental and behavior disorder, dyskinesias, speech disorder and autonomic instability are common clinical manifestations of anti-NMDAR encephalitis in children. The effect of immunotherapy is significant, and the time to start immunotherapy and the severity of the disease are important factors affecting the prognosis. Anti-NMDAR encephalitis can be combined with other autoantibodies, but its clinical significance and mechanism need further study.


Subject(s)
Adolescent , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Autoantibodies , Child , Child, Preschool , Electroencephalography , Female , Humans , Infant , Magnetic Resonance Imaging , Male , Receptors, N-Methyl-D-Aspartate , Retrospective Studies
2.
Article in Chinese | WPRIM | ID: wpr-828539

ABSTRACT

Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a kind of autoimmune disease aiming at NR1 subunit of NMDA receptor. In the early stage, functional damage is the main cause. Electroencephalogram (EEG) can reflect the abnormal brain function by recording the changes of EEG signals. The common EEG patterns of anti NMDA receptor encephalitis are slow wave abnormality, epileptic discharge, a large number of β activity, extreme delta brush, etc. Here we review the waveform characteristics, origin, pathogenesis and clinical value of EEG in patients with NMDA receptor encephalitis.


Subject(s)
Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Electroencephalography , Humans , Research
3.
Article in Chinese | WPRIM | ID: wpr-826346

ABSTRACT

Cryptococcal encephalitis is a fatal central nervous system infectious disease,whereas anti-N-methyl-D-aspartate(NMDA)receptor encephalitis(NMDARE)is an autoimmune syndrome associated with psychological symptoms,behavioural abnormalities,seizures,and dyskinesias.Despite their distinct pathologies and pathogenic mechanisms,both of them can lead to cognitive dysfunction and abnormal behaviors,although anti-NMDARE can also have mood and mental disorders as its core manifestations.A patient with nephrotic syndrome accompanied by both cryptococcal encephalitis and anti-NMDARE was treated in our center,which for the first confirmed that these two conditions could coexist in one patient.The underlying mechanism may be similar to that of anti-NMDARE after other infections.


Subject(s)
Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Antibodies , Humans , Receptors, N-Methyl-D-Aspartate
5.
Rev. MED ; 27(2): 113-120, jul.-dic. 2019. graf
Article in Spanish | LILACS | ID: biblio-1115233

ABSTRACT

Resumen: La encefalitis por anticuerpos contra el receptor de NMDA es un desorden grave, tratable y potencialmente reversible, caracterizado por la presencia de alteraciones en el comportamiento, convulsiones y trastornos del movimiento. La presencia de anticuerpos contra el receptor del glutamato (anti-NMDA) en plasma o líquido cefalorraquídeo es específico para el diagnóstico de la enferme -dad. El reconocimiento temprano de la enfermedad es vital para el pronóstico del paciente, dado que el manejo precoz facilita la recuperación y reducción de la morbimortalidad. El tratamiento consiste en la utilización de corticoides, inmunoglobulina intravenosa o plasmaféresis como primera línea de terapia; además de otros inmunomoduladores, como ciclofosfamida o rituximab, como segunda línea. La recuperación es lenta, pero presenta mejoría en la medida en que los títulos de anticuerpos disminuyen y llega a una recuperación completa hasta en 75 % de los pacientes.


Abstract: Anti-NMDA receptor encephalitis is a severe, treatable, and potentially reversible disorder characterized by the presence of behavioral disturbances, seizures, and movement disorders. The presence of antibodies against the glutamate receptor (anti-NMDA) in plasma or cerebrospinal fluid is specific to the diagnosis of the disease. Early recognition of the disease is vital for the patient's prognosis since early management facilitates recovery and reduction of morbidity and mortality. Treatment consists of corticosteroids, intravenous immunoglobulin, or plasmapheresis as the first line of therapy, in addition to other immunomodulators, such as cyclophosphamide or rituximab, as the second line. Recovery is slow but improves as antibody titers decrease. Complete recovery occurs in up to 75 % of patients.


Resumo: A encefalite causada por anticorpos contra o receptor NMDA é um distúrbio grave, tratável e potencialmente reversível, caracterizado pela presença de alterações comportamentais, convulsões e transtornos de movimento. A presença de anticorpos contra o receptor de glutamato (anti-NMDA) no plasma ou no líquido cefalorraquidiano é específica para o diagnóstico da doença. O reconhecimento precoce da doença é vital para o prognóstico do paciente, visto que o tratamento precoce facilita a recuperação e a redução da morbimortalidade. O tratamento consiste no uso de corticoides, imunoglobulina intravenosa ou plasmaférese como primeira linha de terapia. Além de outros imunomoduladores, como ciclofosfamida ou rituximabe, como segunda linha. A recuperação é lenta, mas apresenta melhora à medida que os títulos de anticorpos diminuem e a recuperação completa ocorre em até 75% dos pacientes.


Subject(s)
Humans , Female , Child , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Immunologic Factors , Encephalitis , Encephalitis/drug therapy
6.
Medicina (B.Aires) ; 79(supl.3): 54-59, set. 2019. tab
Article in Spanish | LILACS | ID: biblio-1040551

ABSTRACT

Las encefalitis autoinmunes (EA) se definen como síndromes neurológicos de instalación subaguda de compromiso de conciencia, alteración de la memoria de trabajo y trastornos psiquiátricos frecuentemente asociados a movimientos anormales y crisis epilépticas y que se producen por la acción de anticuerpos anti neuronales específicos que se fijan a receptores de neurotransmisores o proteínas de membrana. El anticuerpo anti NMDAR es el que origina la mayoría de los casos de EA en niños y adultos jóvenes, seguido por el anticuerpo anti LGI1 de presentación en el adulto. Las EA han aumentado en la última década, en la que se ha descrito un gran número de nuevos anticuerpos que producen en su mayoría síndromes neurológicos que involucran al sistema nervioso central, con predominio de signología psiquiátrica, excepto en niños en los que predominan movimientos anormales, crisis epilépticas y compromiso de conciencia. Se asocian frecuentemente a tumores en el adulto pero en los niños esta asociación es más rara. Todas las EA responden a terapia inmunomoduladora aunque en diferente medida según el tipo de anticuerpo involucrado. Generalmente la evolución a la mejoría es lenta y puede completarse en meses o incluso en un año o más. En esta revisión se destaca los principales cuadros de EA relacionados con anticuerpos específicos mencionando también los inmunofenotipos descubiertos recientemente.


Autoimmune encephalitis (AE) is defined as neurological syndromes of subacute installation of compromise of consciousness, alteration of working memory and psychiatric disorders associated with abnormal movements and epileptic seizures and that are produced by the action of anti-neuronal antibodies. They bind to neurotransmitter receptors or membrane proteins. Antibody to NMDAR is the origin of the majority of cases of AD in children and young adults, followed by anti-LGI1 antibody for presentation in adults. The AE has increased in the last decade, with a large number of new agents described that produce mostly neurological syndromes that involve the central nervous system, with predominance of psychiatric signaling, except in children and the predominant abnormal movements, epileptic seizures and compromise of conscience. They are frequently associated with tumors in adults but in children this association is more infrecuent. All AEs respond to immunomodulatory therapy although in different measures depending on the type of antibody involved. In general, the evolution to improvement is slow and can be completed in months or even in one year or more. In this review, the main EA clinical pictures related to specific antibodies are highlighted, also mentioning recently discovered immunophenotypes.


Subject(s)
Humans , Male , Female , Autoantibodies/adverse effects , Encephalitis/diagnosis , Encephalitis/etiology , Hashimoto Disease/diagnosis , Hashimoto Disease/etiology , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/immunology , Encephalitis/classification , Encephalitis/epidemiology , Hashimoto Disease/classification , Hashimoto Disease/epidemiology
7.
Rev. colomb. psiquiatr ; 48(2): 127-130, ene.-jun. 2019. tab
Article in Spanish | LILACS, COLNAL | ID: biblio-1042855

ABSTRACT

RESUMEN La encefalitis asociada a anticuerpos contra el receptor de N-metil-D-Aspartato (NMDAr) es una entidad clínica recientemente descrita con un número creciente de casos reporta dos. Los síntomas psiquiátricos en etapas tempranas de la enfermedad conforman un reto diagnóstico para el médico tratante. Presentamos dos casos clínicos: el caso clínico 1, un hombre de 26 arios y el caso clínico 2, un joven de 18 arios, ambos abordados como primer episodio de psicosis y hospitalizados en instituciones psiquiátricas. Posteriormente, ambos casos fueron diagnosticados como encefalitis anti-NMDAr. La alta prevalencia de síntomas psiquiátricos en la encefalis anti-NMDAr demanda a los médicos psiquiatras y neurólogos a tener un alto índice de sospecha en presencia de síntomas atípicos en pacientes evaluados por primer episodio de psicosis.


ABSTRACT Anti-N-methyl-D-Aspartate receptor (NMDAr) encephalitis is a recently described clinical entity with an increasing number of reported cases. Psychiatric symptoms in the early stages of the disease constitute a diagnostic challenge for the treating physician. We present two clinical cases: clinical case 1, a 26-year-old man, and clinical case 2, an 18-year-old man; both presented with a first episode of psychosis and were hospitalized as psychiatric disorders. Subsequently, both cases were diagnosed as anti-NMDAr encephalitis. The high prevalence of psychiatric symptoms in anti-NMDAr encephalitis forces psychiatrists and neurologists to have a high degree of suspicion in the presence of atypical symptoms in patients evaluated for the first episode of psychosis.


Subject(s)
Humans , Male , Adult , Psychotic Disorders , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Psychiatry , N-Methylaspartate , Emblems and Insignia , Encephalitis , Neurologists , Mental Disorders , Antibodies
9.
Article in English | WPRIM | ID: wpr-764335

ABSTRACT

BACKGROUND AND PURPOSE: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune encephalitis. This study aimed to explore the possible factors affecting the response to first-line treatments in patients with anti-NMDAR encephalitis. METHODS: We enrolled 29 patients who were diagnosed as anti-NMDAR encephalitis between January 1, 2015, and June 30, 2018. They were divided into the remission and nonremission groups according to their response to first-line treatments. The demographics, clinical manifestations, main ancillary examinations, follow-up treatments, and prognosis of patients were recorded. The symptoms reported on in this study occurred before treatments or during the course of first-line treatments. RESULTS: There were 18 patients (62.07%) in the remission group and 11 patients (37.93%) in the nonremission group. Compared to the remission group, a higher proportion of the patients in the nonremission group exhibited involuntary movements, decreased consciousness, central hypoventilation, lung infection, and hypoalbuminemia. The nonremission group had a high incidence of increased intracranial pressure and significant elevations of the neutrophil-to-lymphocyte ratio in peripheral blood (NLR), aspartate aminotransferase, and fibrinogen. Six patients (54.55%) in the nonremission group received second-line immunotherapy. Only one patient (3.45%) died, which was due to multiple-organ failure. CONCLUSIONS: Anti-NMDAR-encephalitis patients with more symptoms—especially involuntary movements, disturbance of consciousness, central hypoventilation, and accompanying hypoalbuminemia and pulmonary infection—may respond poorly to first-line treatments. Positive second-line immunotherapy therefore needs to be considered. Admission to an intensive-care unit, increased cerebrospinal fluid pressure, and increased NLR might be the significant factors affecting the response to first-line treatments.


Subject(s)
Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Aspartate Aminotransferases , Cerebrospinal Fluid Pressure , Consciousness , Demography , Dyskinesias , Encephalitis , Fibrinogen , Follow-Up Studies , Humans , Hypoalbuminemia , Hypoventilation , Immunotherapy , Incidence , Intracranial Pressure , Lung , Prognosis
10.
Article in English | WPRIM | ID: wpr-766286

ABSTRACT

Many neurologic disorders manifest as psychiatric symptoms. Anti-N-Methyl-D-Aspartate (NMDA) receptor encephalitis is an autoimmune disease of the brain characterized by numerous neurological and psychiatric features. Despite being rare, its prevalence is rapidly increasing and early management is critical in ensuring successful and sustainable recovery. Therefore, the illness should be considered as a differential diagnosis when clinically assessing patients. This report presents a case of a female child who was hospitalized for acute psychiatric manifestations, which was later confirmed as anti-NMDA receptor encephalitis. She recovered relatively successfully after combined neurological and psychiatric treatment. This report provides information on the clinical course of early onset anti-NMDA receptor encephalitis, including treatment strategy and prognosis.


Subject(s)
Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Autoimmune Diseases , Brain , Child , Diagnosis, Differential , Encephalitis , Female , Humans , Nervous System Diseases , Prevalence , Prognosis , Psychotic Disorders , Receptors, N-Methyl-D-Aspartate , Rituximab , Teratoma
12.
Article in Chinese | WPRIM | ID: wpr-813268

ABSTRACT

To investigate the clinical features, auxiliary examination and characteristics for anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis and its concomitant seizure.
 Methods: A total of 20 patients diagnosed as anti-NMDAR encephalitis were enrolled from January 2016 to September 2018 in Xiangya Hospital. The data including the clinical features, auxiliary examination, characteristics of seizure, treatment and prognosis were collected. The discharged patients were followed up for half a year.
 Results: The initial symptom in patients with anti-NMDAR encephalitis were mainly psychiatric symptom and seizure. Most of the EEG result were diffused slow waves. The mainly type of seizure in patients with anti-NMDAR encephalitis showed generalized tonic-clonic seizure. Patients occurred consciousness during the onset of the disease. MRI showed that patients with temporal lobe were more inclined to occur seizure than patients with anti-NMDAR encephalitis (P<0.05). After standardized treatment, 20 patients showed a significant improvement in modified Rankin Scale (mRS) scores and the seizure was under control within half a year. 
 Conclusion: Patients with temporal lobe affected in MRI should pay attention to the possibility of seizure occurrence. Anti-epileptic drugs and immunotherapy should be used promptly in patient with seizure. After standardized treatment, the prognosis of patients will be mostly good.


Subject(s)
Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Humans , Immunotherapy , Magnetic Resonance Imaging , Receptors, N-Methyl-D-Aspartate , Seizures
13.
Autops. Case Rep ; 8(2): e2018019, Apr.-May 2018. ilus
Article in English | LILACS | ID: biblio-905584

ABSTRACT

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently described auto-immune and paraneoplastic encephalitis with prominent neuropsychiatric manifestations affecting young adults with ovarian teratoma. The availability of a novel assay to measure these antibodies might suggest an etiology for this potentially life-threatening disease, which if early recognized can be treated promptly with surgery with chances of a good clinical outcome. Reported prognostic indicators for a good outcome depend on the presence of a tumor, prompt treatment and no admission to an intensive care unit. However, due to the rarity and unawareness of this disease, the diagnosis may be delayed as primary psychiatric disorders, and infective encephalitis is taken more into consideration and ruled out first. Here we report a case of anti-NMDAR encephalitis in a 22-year-old female prompted by an ovarian teratoma with a gradual and complete resolution of symptoms after surgical excision of the teratoma and immunomodulating therapies.


Subject(s)
Humans , Female , Adult , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/complications , Ovarian Neoplasms/complications , Teratoma/complications , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/diagnosis
14.
Arq. neuropsiquiatr ; 76(1): 41-49, Jan. 2018. tab, graf
Article in English | LILACS | ID: biblio-888343

ABSTRACT

ABSTRACT Autoimmune encephalitis (AIE) is one of the most common causes of noninfectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. The neurological manifestations can be either acute or subacute and usually develop within six weeks. There are a variety of clinical manifestations including behavioral and psychiatric symptoms, autonomic disturbances, movement disorders, and seizures. We reviewed common forms of AIE and discuss their diagnostic approach and treatment.


RESUMO As encefalites autoimunes (EAI) são a principal causa de encefalite não-infecciosa. As manifestações neurológicas são variadas, incluindo alterações comportamentais ou psiquiátricas, disautonomia, transtornos do movimento e epilepsia. Habitualmente a instalação dos sintomas ocorre em até 6 semanas, de forma aguda ou subaguda. As EAI podem ser desencadeadas por tumores, quadros infecciosos virais ou ainda apresentar etiologia criptogênica. Este artigo revisa as principais EAI, estratégias de diagnóstico e tratamento.


Subject(s)
Humans , Male , Female , Encephalitis/diagnosis , Encephalitis/therapy , Hashimoto Disease/diagnosis , Hashimoto Disease/therapy , Diagnosis, Differential , Encephalitis/etiology , Encephalitis/physiopathology , Hashimoto Disease/etiology , Hashimoto Disease/physiopathology , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/complications , Immunotherapy
15.
Arq. neuropsiquiatr ; 76(1): 2-5, Jan. 2018. tab
Article in English | LILACS | ID: biblio-888341

ABSTRACT

ABSTRACT Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a form of autoimmune encephalopathy that presents with a wide variety of symptoms, including neuropsychiatric manifestations. The authors' aim for this study was to analyze the results of paraclinical studies of patients with a diagnosis of anti-NMDAR encephalitis and the association between symptom onset and diagnosis, and start of immunotherapy. Retrospective data of 29 patients with anti-NMDAR encephalitis were gathered and analyzed. Abnormal EEG was found in 27 patients (93.1%), whereas MRI was abnormal in 19 patients (65.5%). In contrast, an inflammatory pattern on CSF analysis was found in only 13 patients (44.8%). The absence of pleocytosis or increased proteins in the CSF was associated with a longer time from symptom onset to diagnosis and treatment (p = 0.003). The authors conclude that noninflammatory CSF may delay the correct diagnosis and start of immunotherapy in anti-NMDAR encephalitis. In the presence of suggestive clinical features, extensive studies including EEG are recommended.


RESUMEN La encefalitis por receptor anti-N-metil-D-aspartato (anti-NMDAR) es una encefalopatía autoinmune con una amplia variedad de síntomas, incluyendo manifestaciones neuropsiquiátricas. Nuestro objetivo en este estudio fue analizar los resultados paraclínicos de pacientes diagnosticados con encefalitis anti-NMDAR y la asociación entre inicio de sintomatología, el diagnóstico y el inicio de inmunoterapia. Encontramos un EEG anormal en 27 pacientes (93.1%), así como IRM anormal en 19 de ellos (65.5%). En contraste, el análisis de LCR mostró un patrón inflamatorio en tan solo 13 pacientes (44.8%). La ausencia de pleocitosis o proteínas incrementadas en el LCR se asoció con un mayor tiempo desde el inicio de la sintomatología hasta el inicio del tratamiento (p=0.003). Concluimos que el LCR no inflamatorio puede retrasar el diagnóstico correcto y el inicio de tratamiento en encefalitis anti-NMDAR, por lo que se recomienda la realización de estudios exhaustivos, incluyendo EEG, ante la presencia de indicadores clínicos sugerentes del padecimiento.


Subject(s)
Humans , Male , Female , Adolescent , Adult , Young Adult , Cerebrospinal Fluid Proteins/cerebrospinal fluid , Delayed Diagnosis , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/diagnosis , Magnetic Resonance Imaging , Retrospective Studies , Electroencephalography , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/cerebrospinal fluid , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/therapy , Immunotherapy , Leukocytosis/cerebrospinal fluid
16.
Article in Korean | WPRIM | ID: wpr-715168

ABSTRACT

Anti-N-methyl-D-aspartate (anti-NMDA) encephalitis, which is relatively common in autoimmune encephalitis, is often associated with ovarian teratoma in young women. It has a variety of symptoms including psychiatric symptom, abnormal behavior or movements, seizures, and autonomic instability. Therefore, it was often misdiagnosed as psychiatric problems such as schizophrenia in the early stage of symptoms, and antipsychotic drug was often administered. Here, we describe a 37-year-old woman who has shown abnormal behavior, psychiatric symptoms, and memory impairment and been diagnosed as ovarian teratoma associated anti-NMDA receptor encephalitis. The patient improved significantly after oophorectomy and immunotherapy.


Subject(s)
Adult , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Encephalitis , Female , Humans , Immunotherapy , Memory , Ovariectomy , Schizophrenia , Seizures , Teratoma
17.
Article in Korean | WPRIM | ID: wpr-766650

ABSTRACT

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)
Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Dyskinesias , Encephalitis , Encephalitis, Herpes Simplex , Female , Herpes Simplex , Humans , Korea , Memory , Seizures , Simplexvirus , Teratoma
18.
Article in Korean | WPRIM | ID: wpr-766647

ABSTRACT

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 , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Encephalitis , Female , Follow-Up Studies , Humans , Teratoma , Thorax
19.
Hosp. Aeronáut. Cent ; 13(2): 134-138, 2018.
Article in Spanish | LILACS, BINACIS | ID: biblio-1021142

ABSTRACT

Introducción: En la encefalitis por anticuerpos contra receptores N-Metil-D-Aspartato (NMDA) se genera una disfunción neuronal del ácido gamma-aminobutírico (GABA), con desregulación del glutamato y la dopamina. Los teratomas y las infecciones virales se presumen causales de la respuesta autoinmunitaria. La clínica evoluciona por etapas con un pródromo similar a una infección viral con posteriores manifestaciones psiquiátricas y convulsiones, seguidas de disfunción motora, cognitiva y autonómica. El diagnóstico se basa en la clínica y la presencia de anticuerpos del receptor NMDA. El tratamiento incluye inmunoterapia y eventual eliminación del tumor. La enfermedad puede ser letal o provocar daño irreversible en regiones corticales. Objetivo: Destacar la importancia del diagnóstico precoz en los casos de encefalitis autoinmune para una mayor efectividad de los tratamientos postulados. Reporte de caso: Paciente femenina de 13 años de edad, con diagnóstico de diabetes tipo I; presenta dolor y pérdida de fuerza con movimientos involuntarios en miembro superior izquierdo con dificultad en la deambulación, más episodio convulsivo tónico clónico generalizado seguido de manifestaciones neuropsiquiátricas. Se sospecha encefalitis autoinmune, se dosan anticuerpos anti ácido glutámico descarboxilasa (GAD) y anti NMDA, con resultados positivos. Recibe corticoterapia, inmunoglobulina endovenosa, rituximab y plasmaferesis. Presenta escasa mejoría clínica, con persistencia de síntomas secuelares psiquiátricos y neurológicos. Discusión: Es importante sospechar esta entidad aunque las manifestaciones clínicas iniciales sugieran otras etiologías. El tratamiento inmunosupresor agresivo no debería demorarse aun cuando no se haya confirmado la positividad de los anticuerpos NMDA. El buen pronóstico depende del inicio precoz del tratamiento.


Introduction: The encephalitis by antibodies against NMDA receptors, a neuronal dysfunction of gamma-aminobutyric acid (GABA) is generated, with deregulation of glutamate and dopamine. Teratomas and viral infections are presumed to be the cause to the autoimmune response. The clinic evolves in stages with a prodrome similar to a viral infection with subsequent psychiatric manifestations and seizure, followed by motor, cognitive and autonomic dysfunction. Diagnosis is based on clinical symptoms and the presence of NMDA receptor antibodies. The treatment includes immunotherapy and, eventually, elimination of the tumor. The disease can be lethal or cause irreversible damage in cortical regions. Objective: Highlight the importance of early diagnosis in cases of autoimmune encephalitis for greater effectiveness of postulated treatments. Case report.:13 year old female patient diagnosed with type I diabetes; presents pain, loss of strength and involuntary movements of the upper left limb and ambulation difficulties, associeted with a generalized tonic-clonic seizure episode followed by neuropsychiatric manifestations. Autoinmune encefalitis was suspected so antiglutamic acid decarboxylase (GAD) and anti-NMDA antibodies were dosed, which throw a positive result. The patient receives corticotherapy, intravenous immunoglobulin, rituximab and plasmapheresis. Presenting little clinical improvement, with persistence of psychiatric and neurological sequelae symptoms. Discussion: It is important to suspect this entity although the initial clinical manifestations suggests other etiologies. Aggressive immunosuppressive therapy should not be delayed even when the positivity of NMDA antibodies has not been confirmed. The good prognosis depends on the early start of the treatment.


Subject(s)
Humans , Female , Adolescent , Early Diagnosis , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/prevention & control , Receptors, N-Methyl-D-Aspartate , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/diagnosis , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/therapy
20.
Buenos Aires; s.n; 2018. 50 p. graf..
Non-conventional in Spanish | LILACS | ID: biblio-1009917

ABSTRACT

Ateneo de los residentes de Psicopedagogía del Hospital de Niños Dr Ricardo Gutiérrez, de la Ciudad de Buenos Aires, donde a partir de la práctica psicopedagógica con niños y adolescentes con patología orgánica se desarrollan cuatro ejes temáticos para describir algunas de las patologías o condiciones con las cuales se han ido encontrando en su labor cotidiana. Se relatan algunas viñetas clínicas que surgen de la experiencia en el Consultorio Interdisciplinario de Espina Bífida, con el fin de reflejar la intervención y los aportes de la psicopedagogía en ese campo. Se presenta el caso de una paciente que presentó un cuadro de Encefalitis Autoinmune por anticuerpos contra el receptor NMDAR, patología que despertó un gran interés al interior del equipo y que debido a su creciente recurrencia en los últimos tiempos, convoca a ampliar el conocimiento en ese campo, que aún se encuentra poco investigado. A continuación, se caracterizan dos patologías genéticas a través de casos de Agustín y Matías, considerando la importante incidencia de diversas condiciones genéticas en la población con la cual trabajamos. A partir de los casos clínicos seleccionados, no solo perseguimos el fin de describir y caracterizar algunos cuadros específicos, sino que a través de los mismos buscaremos plasmar los pilares fundamentales sobre los cuales asentamos nuestra mirada e intervención frente a pacientes que exigen un abordaje complejo, integral e interdisciplinario.


Subject(s)
Congenital Abnormalities/psychology , Congenital Abnormalities/rehabilitation , Congenital Abnormalities/therapy , Spinal Dysraphism/therapy , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/therapy , Mandibulofacial Dysostosis/therapy , Noonan Syndrome/therapy , Ambulatory Care Facilities , Internship, Nonmedical
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