ABSTRACT
La encefalitis límbica (EL) autoinmune es una afección neurológica infrecuente de curso subagudo con manifestaciones neuropsicológicas. Actualmente el tratamiento inmunoterápico agudo o de mantenimiento es dirigido según el anticuerpo neural acompañante y la presencia o ausencia de cáncer. Presentamos el caso de una mujer de 52 años con hipotiroidismo autoinmune, síndrome de secreción inadecuada de hormona antidiurética (SIADH) e hiponatremia (hipoNa) persistente, con evolución progresiva de perdida de la memoria y crisis distónicas faciobraquiales (DFBC) a quien se le realiza un diagnóstico oportuno de encefalitis límbica. Recibió tratamiento intravenoso combinado en base a corticoides e inmunoglobulina con buena respuesta y morbilidad mínima neuropsicológica. El reconocimiento de esta patología permite un diagnóstico y tratamiento temprano, imprescindible para mejorar el pronóstico de estos pacientes.
Autoimmune limbic encephalitis is a rather unusual neurological condition with subacute progression and neuropsychological symptoms. Currently, acute or maintenance treatment with immunotherapy is targeted depending on the accompanying neural specific antibody and the presence or absence of cancer. The study presents the case of a 52-year-old woman suffering from autoimmune hypothyroidism, syndrome of inappropriate secretion of anti-diuretic hormone (SIADH) and persistent hyponatremia, with progressive evolution which involved memory loss and faciobrachial dystonic seizures (FBDS). She was timely diagnosed with limbic encephalitis and was treated with intravenous combined corticosteroids and immunoglobulin therapy. Response was good, with minimum neuropsychological. Recognizing this condition allows for early diagnosis and treatment, what is key to improve the prognosis of these patients.
A encefalite límbica (LE) autoimune é uma condição neurológica rara de curso subagudo com manifestações neuropsiquiátrica. Atualmente, o tratamento com imunoterapia aguda ou de manutenção é orientado de acordo com o anticorpo neural e a presença ou ausência de câncer. Apresentamos o caso de uma mulher de 52 anos com hipotireoidismo autoimune, síndrome de secreção inapropriada de hormônio antidiurético e hiponatremia persistente, com evolução progressiva da perda de memória e crises distônicas faciobraquiais que foi diagnosticada oportunamente como encefalite límbica. Recebeu tratamento endovenoso combinado à base de corticoide e imunoglobulina com boa resposta e morbidade neuropsiquiátrica mínima. O reconhecimento desta patologia permite um diagnóstico e tratamento precoces, essenciais para melhorar o prognóstico desses pacientes.
Subject(s)
Limbic Encephalitis/therapy , Hyponatremia , Inappropriate ADH SyndromeABSTRACT
La encefalitis límbica es una enfermedad infrecuente y potencialmente grave, que puede o no ser paraneoplásica y se caracteriza por déficit de la memoria reciente, alteraciones psiquiátricas y convulsiones. De origen autoinmunitario, está asociada a anticuerpos séricos e intratecales contra antígenos neuronales intracelulares y de superficie, con especial afectación de zonas límbicas. En este artículo se revisan aspectos históricos y epidemiológicos, patogenia, síndromes más frecuentes y mejor delimitados, histopatología y estudios complementarios. Se repasan también las dificultades del diagnóstico diferencial y la necesidad de descartar siempre un tumor subyacente. La detección de autoanticuerpos neuronales es importante para el diagnóstico, la planificación terapéutica y el pronóstico. La inmunoterapia y, si corresponde, el tratamiento de la neoplasia son cruciales para lograr una recuperación neurológica sustancial. La encefalitis límbica es una entidad probablemente subdiagnosticada, con un pronóstico más favorable si se trata de forma temprana. El actual conocimiento de su patogenia puede además aportar claridad para la mejor comprensión de otros síndromes neurológicos y psiquiátricos que puedan compartir mecanismos autoinmunitarios, como algunos trastornos psicóticos y epilepsias farmacorresistentes. (AU)
Limbic encephalitis is a rare and potentially serious disease, which may or may not be paraneoplastic and is characterized by recent memory deficits, psychiatric disturbances and seizures. Of autoimmune origin, it is associated with serum and intrathecal antibodies against intracellular and surface neuronal antigens, with special involvement of limbic areas. This article reviews historical and epidemiological aspects, pathogenesis, more frequent and better defined syndromes, histopathology and complementary studies. The difficulties of differential diagnosis and the need to always rule out an underlying tumor are also reviewed. Detection of neuronal autoantibodies is important for diagnosis, therapeutic planning and prognosis. Immunotherapy and, if appropriate, neoplasm treatment, are crucial to achieve substantial neurological recovery. Limbic encephalitis is probably an underdiagnosed entity, with a more favorable prognosis if treated early. The current knowledge of its pathogenesis may also provide clarity for a better understanding of other neurological and psychiatric syndromes that may share autoimmune mechanisms, such as some psychotic disorders and drug-resistant epilepsies. (AU)
Subject(s)
Humans , Autoantibodies/metabolism , Autoimmune Diseases/pathology , Paraneoplastic Syndromes, Nervous System/pathology , Limbic Encephalitis/pathology , Psychotic Disorders/diagnosis , Psychotic Disorders/etiology , Autoimmune Diseases/diagnosis , Autoimmune Diseases/etiology , Autoimmune Diseases/therapy , Review Literature as Topic , Paraneoplastic Syndromes, Nervous System/diagnosis , Paraneoplastic Syndromes, Nervous System/etiology , Paraneoplastic Syndromes, Nervous System/therapy , Limbic Encephalitis/diagnosis , Limbic Encephalitis/etiology , Limbic Encephalitis/history , Limbic Encephalitis/therapy , Epilepsy/diagnosis , Epilepsy/etiologyABSTRACT
Autoimmune encephalitis is an inflammatory disorder characterized by a subacute impairment of short-term memory, psychiatric features and seizures. It is often associated with a variety of other neurological symptoms, and its differential diagnosis is wide, leading to challenges in its recognition. It used to be regarded as a rare disease, usually paraneoplastic and with poor prognosis. However, with the recent recognition of membrane-surface directed antibodies, it is now known that in a substantial proportion of cases there is no association with any malignancy and there is a good prognosis if treated. Hence, early recognition and prompt initiation of immunotherapies are of great importance.
A encefalite autoimune é uma doença inflamatória caracterizada por envolvimento subagudo da memória de curto prazo, presença de sintomas psicóticos e crises epilépticas. Dada a diversidade de sintomas na apresentação, o diagnóstico diferencial é um verdadeiro desafio. Anteriormente, era considerada uma doença rara, de etiologia paraneoplásica e com mau prognóstico. No entanto, com a recente descoberta dos anticorpos dirigidos à superfície da membrana, é atualmente reconhecido que uma grande parte dos casos não tem uma neoplasia subjacente e apresenta um ótimo prognóstico. Assim, o diagnóstico e tratamento imunoterápico precoces são de extrema importância.