ABSTRACT
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.
Subject(s)
Autoimmune Diseases of the Nervous System/diagnosis , Autoimmune Diseases of the Nervous System/therapy , Limbic Encephalitis/diagnosis , Limbic Encephalitis/therapy , Diagnosis, Differential , Humans , Immunotherapy/methods , PrognosisABSTRACT
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.
Subject(s)
Humans , Autoimmune Diseases of the Nervous System/diagnosis , Autoimmune Diseases of the Nervous System/therapy , Limbic Encephalitis/diagnosis , Limbic Encephalitis/therapy , Diagnosis, Differential , Immunotherapy/methods , PrognosisABSTRACT
The autoimmune encephalopathies are a group of conditions that are associated with autoantibodies against surface neuronal proteins, which are likely to mediate the disease. They are established as a frequent cause of encephalitis. Characteristic clinical features in individual patients often allow the specificity of the underlying antibody to be confidently predicted. Antibodies against the VGKC-complex, mainly LGI1(leucine-rich glioma-inactivated 1), CASPR2 (contactin-associated protein 2), and contactin-2, and NMDA (N-methyl, D-aspartate) -receptor are the most frequently established serological associations. In the minority of cases, an underlying tumour can be responsible. Early administration of immunotherapies, and tumour removal, where it is relevant, offer the greatest chance of improvement. Prolonged courses of immunotherapies may be required, and clinical improvements often correlate well with the antibody levels. In the present article, we have summarised recent developments in the clinical and laboratory findings within this rapidly expanding field.
Subject(s)
Autoantibodies/immunology , Autoimmune Diseases/therapy , Encephalitis/therapy , Immunotherapy/methods , Autoimmune Diseases/immunology , Contactin 2/immunology , Encephalitis/classification , Encephalitis/immunology , Humans , Intracellular Signaling Peptides and Proteins , Membrane Proteins/immunology , Nerve Tissue Proteins/immunology , Proteins/immunology , Receptors, N-Methyl-D-Aspartate/immunologyABSTRACT
The autoimmune encephalopathies are a group of conditions that are associated with autoantibodies against surface neuronal proteins, which are likely to mediate the disease. They are established as a frequent cause of encephalitis. Characteristic clinical features in individual patients often allow the specificity of the underlying antibody to be confidently predicted. Antibodies against the VGKC-complex, mainly LGI1(leucine-rich glioma-inactivated 1), CASPR2 (contactin-associated protein 2), and contactin-2, and NMDA (N-methyl, D-aspartate) -receptor are the most frequently established serological associations. In the minority of cases, an underlying tumour can be responsible. Early administration of immunotherapies, and tumour removal, where it is relevant, offer the greatest chance of improvement. Prolonged courses of immunotherapies may be required, and clinical improvements often correlate well with the antibody levels. In the present article, we have summarised recent developments in the clinical and laboratory findings within this rapidly expanding field.
As encefalopatias autoimunes constituem um grupo de condições associadas à presença, no soro, de anticorpos contra proteínas de superfície neuronais. Acredita-se que esses anticorpos sejam mediadores da ocorrência da doença, sendo reconhecidos atualmente como causas frequentes de encefalite. Apresentações clínicas características permitem, muitas vezes, predizer o grupo específico de anticorpos subjacentes. Anticorpos contra o complexo VGKF, especialmente LGI1 (leucine-rich glioma-inactivated1), CASPR2 (contactin-associated protein 2) e contactina-2, e contra o receptor NMDA(N-methyl, D-aspartate) são as associações sorológicas mais frequentemente estabelecidas. Na minoria dos casos, pode ser detectado um tumor subjacente. As maiores chances de melhora estão relacionadas à administração precoce de imunoterapia e à remoção do tumor, quando presente. A duração da imunoterapia pode se prolongada e a melhora se correlaciona, muitas vezes, com os níveis séricos de anticorpos. Neste artigo, estão resumidos os avanços recentes nos achados clínicos e laboratoriais neste campo que está em tão rápida expansão.
Subject(s)
Humans , Autoantibodies/immunology , Autoimmune Diseases/therapy , Encephalitis/therapy , Immunotherapy/methods , Autoimmune Diseases/immunology , /immunology , Encephalitis/classification , Encephalitis/immunology , Membrane Proteins/immunology , Nerve Tissue Proteins/immunology , Proteins/immunology , Receptors, N-Methyl-D-Aspartate/immunologyABSTRACT
Anti-N-methyl-D-aspartate-receptor encephalitis is a recently identified autoimmune disorder. We report on a 4-year-old girl presenting with seizures after nonspecific viral-like symptoms, progressing to severe aphasia, upper limb dyskinesias, fluctuation in consciousness, and inability to walk. Anti-N-methyl-D-aspartate-receptor encephalitis should be included in the differential diagnosis of acute/subacute encephalitis in children.