ABSTRACT
La encefalitis autoinmune es un trastorno inmunomediado que compromete distintos territorios del parénquima cerebral, involucrando frecuentemente la materia gris profunda o la corteza, con o sin compromiso de la materia blanca, meninges o médula espinal. Se asocia frecuentemente con enfermedades autoinmunes o paraneoplásicas, y constituye un reto diagnóstico. Reportamos el caso de una mujer de 55 años con antecedente de síndrome de Sjögren que consultó a Emergencias por cefalea y confusión. El líquido cefalorraquídeo (LCR) presentaba leucocitosis con neutrofilia. En la resonancia magnética nuclear (RMN) cerebral se evidenciaron múltiples imágenes de comportamiento restrictivo, de señal hiperintensa en T2 y FLAIR, a predominio córtico-subcortical a nivel occipital bilateral, hemisferio cerebeloso derecho y parietal derecho. Se descartaron infecciones y neoplasias. El panel de anticuerpos para encefalitis autoinmune aquaporina-4 y anti-MOG en LCR fue negativo. Recibió metilprednisolona endovenosa con mejoría progresiva de los síntomas.
Autoimmune encephalitis is an immune-mediated disorder that affects different areas of the brain parenchyma, often involving deep gray matter or the cortex, with or without involvement of white matter, meninges, or spinal cord. It is frequently associated with autoimmune or paraneoplastic diseases and is a diagnostic challenge. We report the case of a 55-year-old woman with history of Sjögren's syndrome who presented to the emergency department with headache and episodes of confusion. Cerebrospinal fluid (CSF) analysis showed leukocytosis with neutrophilia. Brain MRI revealed multiple restricted diffusion lesions with hyperintense signal on T2 and FLAIR sequences, predominantly in the bilateral occipital region, right cerebellar hemisphere, and right parietal region. Infections and neoplasms were ruled out. The panel of antibodies for autoimmune encephalitis, including Aquaporin-4 and anti-MOG in CSF, was negative. She received intravenous methylprednisolone, leading to symptom improvement.
Subject(s)
Female , Central Nervous SystemABSTRACT
Resumen Introducción: Las encefalitis inmunomediadas son un desorden neurológico de origen autoinmune. Actual mente es escasa la descripción de las secuelas cognitivas crónicas. El objetivo del presente trabajo fue caracterizar la secuela cognitiva de diferentes tipos de encefalitis inmunomediadas en una cohorte de un centro único de Argentina. Métodos: Estudio prospectivo, observacional, trans versal, de pacientes en seguimiento en un hospital de la Ciudad de Buenos Aires, con diagnóstico de encefalitis inmunomediada probable y definitiva. Se evaluaron variables epidemiológicas, clínicas, paraclínicas y tra tamiento. Se determinó la secuela cognitiva a través de una evaluación neurocognitiva realizada a partir del año de la presentación clínica. Resultados: Fueron incluidos 15 pacientes, todos con resultado disminuido en al menos un test. La memoria fue el dominio más afectado. Aquellos que se encon traban bajo tratamiento inmunosupresor al momento de evaluarse presentaron menores resultados en el aprendizaje seriado (media -2.94; desvío estándar 1.54) versus los que se encontraban sin tratamiento (media -1.18; desvío estándar 1.40; p = 0.05) y en la prueba de reconocimiento (media -10.34; desvío estándar 8.02) ver sus sin tratamiento (media -1.39; desvío estándar 2.21; p = 0.003). Los pacientes con estatus epiléptico tuvieron resultados deficitarios en la prueba de reconocimiento (media -7.2; desvío estándar 7.91) en comparación a los que no lo tenían (media -1.47; desvío estándar 2.34; p = 0.05). Conclusión: Nuestros resultados demuestran que, a pesar del curso monofásico de la enfermedad, todos los pacientes presentan daño cognitivo persistente más allá del año del inicio del cuadro. Estudios prospectivos de mayor envergadura serían necesarios para confirmar nuestros hallazgos.
Abstract Introduction: Autoimmune encephalitis represents a group of immune-mediated neurological disorders. At present, the description of the chronic cognitive sequela is scarce. The objective of this study was to characterize the cognitive after effects of different types of autoimmune encephalitis in a cohort from a single center in Argentina. Methods: Prospective, observational, cross-sectional study of patients under follow-up at a hospital in Buenos Aires city, with a diagnosis of probable and definitive immune-mediated encephalitis. Epidemiological, clini cal, paraclinical and treatment related variables were evaluated. Cognitive sequela was determined through a neurocognitive evaluation performed at least a year after the clinical presentation. Results: Fifteen patients were included. All had di minished results in at least one test. Memory was the most affected domain. Patients who were under im munosuppressive treatment at the time of evaluation presented lower results in serial learning (mean -2.94; standard deviation 1.54) versus those who weren't under treatment (mean -1.18; standard deviation 1.40; p = 0.05). The same pattern was observed on the recognition test of treatment group (mean -10.34; standard deviation 8.02) versus treatment-free group (mean -1.39; standard deviation 2.21; p =0.003). Patients with status epilepticus had poorer results in the recognition test (mean -7.2; standard deviation 7.91) compared to those without it (mean -1.47; standard deviation 2.34; p = 0.05). Conclusion: Our results show that, despite the mo nophasic course of this disease, all patients had persis tent cognitive damage beyond the year of onset. Larger prospective studies are required to confirm our findings.
ABSTRACT
Using the immune system to its advantage, Salmonella Typhi initially invades the gut followed by the reticuloendothelial system and finally the nervous system, involvement of which usually occurs around the second week of fever. In developing countries, delayed diagnosis is predominantly due to hesitation in seeking treatment. Our subject presented with fever since one week, altered mentation, headache and neck pain; she was diagnosed with enteric fever. Although her neurological abnormality could be a complication of the infection, it appeared when she became afebrile- hence we evaluated her for autoimmune conditions. Positive results hinted at autoimmune encephalitis triggered by the infection; further studies were inconclusive. Association of enteric fever with autoimmune encephalitis has not been reported. Three months later, presence of antinuclear antibodies (ANA) was rechecked- a negative report led to a retrospective diagnosis of transient ANA positivity in a nonautoimmune inflammatory disease, the case in point being enteric fever.
ABSTRACT
This paper elaborated a case of an elderly patient with anti-contactin-associated protein-like 2 （CASPR2） antibody-associated autoimmune encephalitis. It illustrated the common manifestations and diagnostic process of anti-CASPR2 antibody-associated encephalitis， and the analysis of clinical symptoms and ancillary findings had contributed to gaining insight into the diagnosis of autoimmune encephalitis in elderly patients characterized by distinct psycho-behavioral abnormalities and cognitive decline， as well as suggesting the possibility that autoimmune encephalitis in elderly may exacerbate the development of cerebrovascular disease.
ABSTRACT
OBJECTIVES@#To study the clinical features of children with autoimmune encephalitis (AE) secondary to epidemic encephalitis B (EEB).@*METHODS@#A retrospective analysis was performed on the medical data of five children with EEB with "bipolar course" who were treated in Children's Hospital Affiliated to Zhengzhou University from January 2020 to June 2022.@*RESULTS@#Among the five children, there were three boys and two girls, with a median age of onset of 7 years (range 3 years 9 months to 12 years) and a median time of 32 (range 25-37) days from the onset of EEB to the appearance of AE symptoms. The main symptoms in the AE stage included dyskinesia (5/5), low-grade fever (4/5), mental and behavioral disorders (4/5), convulsion (2/5), severe disturbance of consciousness (2/5), and limb weakness (1/5). Compared with the results of cranial MRI in the acute phase of EEB, the lesions were enlarged in 3 children and unchanged in 2 children showed on cranial MRI in the AE stage. In the AE stage, four children were positive for anti-N-methyl-D-aspartate receptor antibody (one was also positive for anti-γ-aminobutyric acid type B receptor antibody), and one was negative for all AE antibodies. All five children in the AE stage responded to immunotherapy and were followed up for 3 months, among whom one almost recovered and four still had neurological dysfunction.@*CONCLUSIONS@#EEB can induce AE, with anti-N-methyl-D-aspartate receptor encephalitis as the most common disease. The symptoms in the AE stage are similar to those of classical anti-N-methyl-D-aspartate receptor encephalitis. Immunotherapy is effective for children with AE secondary to EEB, and the prognosis might be related to neurological dysfunction in the acute phase of EEB.
Subject(s)
Male , Female , Humans , Child , Infant, Newborn , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Retrospective Studies , Hashimoto Disease/therapy , Encephalitis, ArbovirusABSTRACT
Las encefalitis autoinmunes son una condición emergente, caracterizada por la aparición repentina de síntomas psicóticos o depresivos "de novo", crisis convulsivas o estatus epiléptico refractario, o demencia rápidamente progresiva. Las encefalitis autoinmunes están asociadas a diversos fenómenos desencadenantes, como infecciones virales previas entre las más comunes, y se asocian con la presencia de anticuerpos antineuronales y/o onconeuronales, que deben estudiarse ante la sospecha de esta entidad. Es muy importante desarrollar un diagnóstico presuntivo y precoz, ya que la terapia con inmunosupresores como los corticoides -iniciados en el momento oportuno-, puede cambiar su evolución hacia la mejoría clínica. Presentamos un paciente con encefalitis autoinmunes y anticuerpos anti-Titina positivos (habitualmente presentes en timoma y miastenia gravis), no asociados a neoplasia conocida y con buena respuesta a esteroides.
Autoimmune Encephalitis, are an emerging condition, characterized by the sudden onset of psychotic or depressive symptoms "de novo", refractory seizures or epilepsy, or rapidly progressive dementias. The autoimmune encephalitis are associated to various triggered phenomena as a previous viral infections among others; it's related to the presence of antineuronal and/or onconeuronal antibodies, and there must be studied when autoimmune encephalitis is suspected. It is very important to develop a presumptive and early diagnosis, since steroid therapy -on opportunity time- can change its evolution towards clinical improvement. We present a patient with autoimmune encephalitis, and positive anti-Titin antibodies (usually presents in thymoma and myasthenia gravis) not associated with known neoplasia, and with a good response to steroids.
ABSTRACT
La encefalitis autoinmune es un cuadro con una expresión neuropsiquiátrica especialmente en pediatría, aunque existen diversas opciones de tratamiento, otras alternativas terapéuticas se relacionan con procedimientos que pueden tener un mayor beneficio para el paciente, como es la plasmaféresis. Este procedimiento, representa una de las terapias de primera línea en este padecimiento. Hace poco se cuenta con la disponibilidad de estos equipos, motivo por el cual describimos este primer procedimiento en pediatría a nivel Bolivia describiendo el manejo en un paciente con encefalitis autoinmune.
Autoimmune encephalitis is a condition with a neuropsychiatric expression, especially in pediatrics, although there are various treatment options, other therapeutic alternatives are related to procedures that may have a greater benefit for the patient, such as plasmapheresis. This procedure represents one of the first line therapies in this condition. This equipment has recently become available, which is why we describe this first procedure in pediatrics at the Bolivian level, describing the management of a patient with autoimmune encephalitis.
Subject(s)
Therapeutics , Encephalitis , Pediatrics , PlasmapheresisABSTRACT
This article reported the clinical features of a rare patient with anti-metabotropic glutamate receptor 5 （mGluR5） encephalitis with mental disorders as the initial symptom， so as to provide references for clinical diagnosis and treatment. The patient was a 38-year-old male， developed pharyngeal pain as prodromal symptoms， and the main clinical manifestations included rapidly progressive memory loss， anxiety and depression， and psychomotor excitement symptoms including irritability and impulsive behaviors. The disease had a progressive deterioration. In the most severe state， the patient became unconscious in a shallow coma， with further cognitive decline， hallucinations and delusions， and lack of self-awareness. Both cerebrospinal fluid and serum anti-mGluR5 antibody were strongly positive （1∶100）. After two sessions of hormone shock therapy， the patient showed significantly improvement in consciousness， cognitive， emotional and psychiatric dimensions.
ABSTRACT
The clinical data of 5 patients with autoimmune encephalitis admitted to the psychiatric department of the 904th Hospital of the Joint Logistics Service Force from January 2016 to June 2020 were retrospectively analyzed. Among 5 patients, 4 had stress psychological events within one month before the onset, and 3 had precursor symptoms such as fever and vomiting. They were all characterized by rapid progress of atypical mental and behavioral abnormalities and cognitive impairment. In terms of neurological symptoms, 1 case had faciobrachial dystonic seizures (FBDS), 3 cases had seizures, 2 cases had involuntary movement, and 4 cases had autonomic dysfunction, including central hypopnea, arrhythmia, blood pressure instability and paroxysmal facial flushing. Most neurological symptoms occur within 1 month of the onset. MRI revealed abnormalities in cerebral cortex, thalamus, temporal lobe and insular lobe in 4 cases; EEG demonstrated bilateral short-range medium amplitude θ wave in 2 cases. Abnormal cerebrospinal fluid (CSF) pressure was detected in 4 cases and 2 cases had abnormal cell number CSF. Three patients had positive anti-N-methyl-D-aspartate receptor (NMDAR) antibody, one patient had positive anti-LGI1 antibody, and one patient had positive anti-γ-aminobutyric acid B receptor (GABA BR) antibody. One case was discharged automatically, the remaining 4 patients were treated with glucocorticoid or combined with gamma globulin and cyclophosphamide, antiepileptic drugs, antipsychotic drugs and other symptomatic treatment, and their symptoms were relieved. Patients were followed up for six months, there was slightly slow residual reaction in 2 cases and personality change in 1 case. Autoimmune encephalitis characterized by mental symptoms is likely to be misdiagnosed as mental disorders. Clinicians should identify symptoms different from mental disorders, taking into account of the possibility of autoimmune encephalitis, to make early diagnosis and treatment.
ABSTRACT
Autoimmune encephalitis is a type of autoimmune disease affecting the central nervous system, which is mediated or associated with anti-neural antibodies. The pathogenesis and etiology are complex. Acute or subacute psycho-behavioral abnormalities, seizures, and cognitive impairment are the main clinical manifestations. Early diagnosis and aggressive immunotherapy are helpful for prognosis. This article reviews the progress in the pathogenesis, etiology, diagnosis and treatment of autoimmune encephalitis.
ABSTRACT
Immunoadsorption can selectively remove pathogenic antibodies and has recently achieved good results in neuroimmune diseases. The type and titer of pathogenic antibodies play an important role in the clinical symptoms and severity of patients with autoimmune encephalitis. First-line immunotherapy for autoimmune encephalitis includes steroids, intravenous immunoglobulin, plasma exchange or immunoadsorption. Immunoadsorption selectively and rapidly eliminates autoantibodies and can be an effective therapeutic option as part of multimodal immunotherapy.
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La presencia de manifestaciones neuropsiquiátricas en pacientes reumatológicos trae consigo un gran desafío diagnóstico que exige una mirada amplia, desde las bases de la medicina interna, a fin de poder orientar un estudio adecuado y el tratamiento oportuno. Junto con ello, el permanente diálogo e intercambio de miradas clínicas con otras especialidades permite tener un enfoque multidisciplinario que enriquece el abordaje de estas presentaciones complejas.
The presence of neuropsychiatric manifestations in rheumatological patients brings with it a great diagnostic challenge that requires a broad view, from the foundations of internal medicine, in order to guide the appropriate study and timely treatment of these patients. Along with this, the permanent dialogue and exchange of clinical views with other specialties allows for a multidisciplinary approach that enriches the approach to these complex presentations.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Lupus Vasculitis, Central Nervous System/therapy , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/therapy , Azathioprine , Cyclophosphamide/therapeutic use , Immunosuppressive AgentsABSTRACT
Sleep disorders are common diseases of nervous system, while sleep disorders in autoimmune encephalitis have received little attention. Sleep involves in the complex function of nerve centers and brain networks. Some autoimmune encephalitis with specific antibodies has sleep disorders as the core symptoms, or sleep patterns change as disease evolution. Sleep disorders often occur in autoimmune encephalitis and usually persist beyond the acute stage of disease,which seriously affect the recovery and life quality of patients. The study of sleep disorders in autoimmune encephalitis is rarely recognized and studied. The characteristics and possible mechanisms of sleep disorders in autoimmune encephalitis were reviewed here to improve the recognition and attention of clinicians.
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A case of limbic encephalitis with positive anti-zinc finger protein 4 (ZIC4) antibody and anti-Hu antibody was reported. A middle-aged female was admitted to hospital for two months because of memory loss and unstable walking. The main manifestations were cognitive decline, ataxia and sensory disturbance of both lower extremities. The main diagnosis was limbic encephalitis, complicated with subacute cerebellar degeneration and subacute sensory neuron disease, which was consistent with paraneoplastic nervous system syndrome. Magnetic resonance imaging showed abnormal signals in bilateral temporal lobe and hippocampus, electromyography showed sensory nerve damage, blood and cerebrospinal fluid anti-ZIC4 antibody and Hu antibody were both positive, and no tumor was found. It is speculated that there may be potential tumors and need to be followed up and monitored. This rare case is reported to attract the attention of clinicians.
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Anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis is an autoimmune disorder mediated by anti-NMDAR antibody.It can be found in both adults and children.Anti-NMDAR encephalitis in children is different from adults in inducement, clinical manifestation, diagnosis process, treatment, follow-up strategy, and prognosis.Although the clinical manifestation of the disease is serious, timely and effective immunotherapy can make the vast majority of children get a good prognosis.Therefore, the review summarized and analyzed the treatment progress, in order to improve the standard diagnosis and treatment of pediatric anti-NMDAR encephalitis in China, and reduce the morbidity and mortality.
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Objective:To summarize the clinical features, imaging changes, treatment, and prognosis of children with severe autoimmune encephalitis (AE).Methods:A retrospective study was conducted on patients with severe AE admitted to PICU of Shanghai Children’s Hospital from June 2017 to May 2020. Clinical features, treatment protocols and follow-up data were collected.Results:A total of 27 children were included, among which 18 cases (66.7%) were girls. The on-set age was (7.9±3.2) years. Eighteen cases were diagnosed with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Fever (77.8%), headache (40.7%) and vomiting (44.4%) were most of prodromal symptoms in children with severe AE. Patients’ neurological symptoms showed seizures (88.9%), mental behavior abnormalities (81.5%), speech disorders (70.4%) and dyskinesia (70.4%). Moreover, epileptic discharge and slow wave activity were critical feature of electroencephalogram (EEG) abnormalities, and the abnormal signal changes on T2-weighted and FLAIR sequence of head MRI were in the posterior horn of the lateral ventricle. In addition, the main comorbidities included refractory status epilepticus (RSE), cardiovascular dysfunction, central hypoventilation syndrome and acute intracranial hypertension syndrome. For patients with central respiratory failure, the median duration of mechanical ventilation was 19.8 (14.8, 29.1) days. According to treatment protocol, the first-line immune treatment included the combination therapies of methylprednisolone, intravenous immunoglobulin (IVIG) and therapeutic plasma exchange (TPE). Eighteen cases were given with methylprednisolone [10-30 mg/(kg. d), 3-5 d] + IVIG (2 g/kg, within 2 d) + TPE, 1 case was treated with methylprednisolone [10-30 mg/(kg·d), 3-5 d] + TPE and 8 cases were given with[10-30 mg/(kg·d), 3-5 d] + IVIG (2 g/kg, within 2 d). Sequential therapy was given with methylprednisolone (1-2 mg/kg), gradually reduced from 3 to 6 months. Finally, 16 children (59.3%) had neurological damages at the first discharge, among which 8 cases (29.6%) were with dyskinesia, 5 cases (18.5%) were with speech disturbance, and 5 cases (18.5%) were with abnormal mental behaviors.Conclusions:The most of first clinical symptom is epileptic seizures in pediatric severe AE, and most of these patients are diagnosed with Anti-NMDA receptor encephalitis. RSE, cardiovascular dysfunction, central respiratory and acute intracranial hypertension syndrome constitute to main organ dysfunctions.
ABSTRACT
@#N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis is recognized as an autoimmune encephalitis, which is due to autoantibodies against synaptic NMDAR. This disorder affects individuals aged 23 months to 76 years and has a wide range of presentations. In Malaysia, more than 20 cases have been reported. Timely diagnosis and definitive immunotherapy are vital in optimizing functional recovery and prognosis. However, early diagnosis of the condition is often missed due to low awareness among clinicians in Malaysia. This article gathered the medical literature from Malaysia and highlights the aetio-pathophysiology, clinical presentation and management of the disease.
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 therapyABSTRACT
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/epidemiologyABSTRACT
Six children with anti-NMDAR encephalitis were followed-up for 6to 24 months. They presented with seizures, neuropsychiatricsymptoms and movement disorder, particularly orofacialdyskinesia and choreoathetosis. Immunosuppressive therapyresulted in varying degrees of improvement; none relapsed.Expressive aphasia was the last symptom to regress