ABSTRACT
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.
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
Background: Limbic encephalitis is a subacute syndrome characterized by memory impairment, confusion, seizures, hypothalamic dysfunction and psychiatric symptoms. It has been associated to tumors located outside of the central nervous system. In 2007, anti-N-methyl-D-aspartate receptors (NMDAr) antibodies were found in serum and CSF of patients with this particular type of encephalitis. We report a 25-year-old female who, following upper respiratory tract symptoms, developed serious behavioral and consciousness impairment that progressed to coma. Cerebrospinal fluid (CSF) analysis showed a lymphocyte pleocytosis, the electroencephalogram was altered with a slow encephalopathic rhythm and a brain magnetic resonance imaging was normal. Infectious etiologies were ruled out. CSF and serum anti NMDA receptors antibodies were positive.
Subject(s)
Adult , Female , Humans , Antibodies/cerebrospinal fluid , Limbic Encephalitis/diagnosis , N-Methylaspartate/immunology , Receptors, N-Methyl-D-Aspartate/immunology , Limbic Encephalitis/physiopathologyABSTRACT
Limbic encephalitis (LE) can be associated to cancer, viral infection or be idiopathic. One form is associated to voltage dependent potassium channel (VKC) antibodies. The clinical presentation includes impairment of consciousness, amnesia and temporal lobe seizures; typical abnormalities are also found in brain magnetic resonance. We report a 68 year-old male who had LE associated to VKC antibodies. The patient was treated with steroids with a partial response. At the moment of the report he is asymptomatic and continues with prednisone treatment.
Subject(s)
Aged , Humans , Male , Autoantibodies/blood , Limbic Encephalitis/immunology , Potassium Channels, Voltage-Gated/immunology , Electroencephalography , Glucocorticoids/therapeutic use , Limbic Encephalitis/diagnosis , Limbic Encephalitis/drug therapy , Magnetic Resonance Imaging , Piracetam/analogs & derivatives , Piracetam/therapeutic use , Prednisone/therapeutic use , Tomography, X-Ray Computed , Valproic Acid/therapeutic useABSTRACT
The high signal intensities in bilateral mesiotemporal lobes on T2-weighted images are typical findings of herpes encephalitis or paraneoplastic limbic encephalitis. We report a case of neurosyphilis with mesiotemporal involvement on MRI. Positive antibodies in the cerebrospinal fluid confirmed the diagnosis. The results suggest that neurosyphilis should be considered when MRI results indicate mesiotemporal abnormalities.
Subject(s)
Adult , Humans , Male , Anti-Bacterial Agents/administration & dosage , Biopsy, Needle , Chancre/diagnosis , Diagnosis, Differential , Encephalitis, Herpes Simplex/diagnosis , Follow-Up Studies , Limbic Encephalitis/diagnosis , Magnetic Resonance Imaging/methods , Neurosyphilis/diagnosis , Penicillins/administration & dosage , Rare Diseases , Temporal Lobe/pathology , Tongue/pathologyABSTRACT
A 46 years, nondiabetic, nonhypertensive woman presented with headache, vomiting, low grade intermittent fever, behavioral abnormality and seizures for last three months. Clinically she had meningism with bilateral papilloedema. Based on CSF analysis, normal CT scan of brain and suspicious lesion in X-ray chest, she was put on anti-tuberculosis therapy. As the patient further deteriorated clinically, MRI of brain was carried out and revealed bilateral increased signal intensities in both medial temporal lobes in T2 and flair sequences. Inj. acyclovir was added considering the diagnosis of herpes simplex encephalitis. In spite of receiving treatment patient gradually became more drowsy and repeat X-ray chest with CT guided FNAC showed picture of adenocarcinoma of lung. So finally, we concluded it to be a case of limbic encephalitis.