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Abstract Background Autoimmune encephalitis (AE) consists of a group of acquired diseases that affect the central nervous system. A myriad of phenotypes may be present at the onset. Due to the heterogeneity of clinical presentations, it is difficult to achieve uniformity for the diagnostic and therapeutic processes and follow-up strategies. Objective To describe a series of patients diagnosed with AE in a resource-limited public hospital in southern Brazil and to analyze therapeutics and outcomes. Methods We retrospectively reviewed the electronic medical records of patients diagnosed with AE at the Hospital de Clínicas de Porto Alegre from 2014 to 2022. Data collected included clinical presentation, neuroimaging, cerebrospinal fluid testings, electroencephalogram, autoantibodies, treatments, outcomes, follow-up time, degree of neurological impairment, and mortality. Results Data from 17 patients were retrieved. Eleven cases were classified as definite AE and 6 as possible AE. Autoantibodies were identified in 9 patients. Timing for diagnosis was impacted by the high costs associated with autoantibody testing. Most patients became functionally dependent (82.4%) and most survivors remained with autoimmune-associated epilepsy (75%). Five patients died during hospitalization, and one after a 26-month of follow-up. Conclusion In this resource-limited hospital, patients with AE had a worse clinical outcome than that previously described in the literature. Development of epilepsy during follow-up and mortality were greater, whilst functional outcome was inferior. Autoantibody testing was initially denied in most patients, which impacted the definitive diagnosis and the use of second-line therapies.
Resumo Antecedentes A encefalite autoimune (EA) consiste em um grupo de doenças adquiridas que afetam o sistema nervoso central. Objetivo Descrever uma série de pacientes diagnosticados com EA em um contexto de atenção terciária à saúde com recursos limitados e analisar a terapêutica e os resultados. Métodos Revisamos retrospectivamente os prontuários eletrônicos de pacientes diagnosticados com EA no Hospital de Clínicas de Porto Alegre de 2014 a 2022. Os dados coletados incluíram apresentação clínica, neuroimagem, exames de líquido cefalorraquidiano, eletroencefalograma, autoanticorpos, tratamentos, resultados, tempo de acompanhamento, grau de comprometimento neurológico e mortalidade. Resultados Dados de 17 pacientes foram coletados. Onze casos foram classificados como EA definitivo e seis como EA possível. Autoanticorpos foram identificados em nove pacientes. O tempo para o diagnóstico foi afetado pelos altos custos associados ao teste de autoanticorpos. A maioria dos pacientes tornou-se funcionalmente dependente (82,4%), e a maioria dos sobreviventes permaneceu com epilepsia autoimune associada (75%). Cinco pacientes faleceram durante a internação, e um após 26 meses de seguimento. Conclusão No hospital em questão, os pacientes com EA tiveram um desfecho clínico pior do que o previamente descrito na literatura. O desenvolvimento de epilepsia durante o acompanhamento e a mortalidade foram maiores, enquanto o desfecho funcional foi inferior. Os testes de autoanticorpos foram inicialmente negados para a maioria dos pacientes, o que impactou o diagnóstico definitivo e o uso de terapias de segunda linha.
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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.
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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
Objective:To investigate the clinical characteristics and outcome of patients with voltage-gated potassium channel complex (VGKCc) antibody associated clinical syndromes complicated with myasthenia gravis (MG) with thymoma.Methods:The clinical history, examinations and follow-up prognosis of 2 cases of VGKCc antibodies associated clinical syndromes with MG complicated with thymoma in Qilu Hospital (Qingdao), Cheeloo College of Medicine,Shandong University in September 2020 and December 2020 were reviewed. Related literatures were summarized at the same time.Results:Case 1, a 64-year-old female clinically presented with cognitive impairment, psychosis, and epilepsy seizures, whose serum autoimmune antibody testing showed positive leucine-rich glioma-inhibited 1 (LGI1) antibody, was diagnosed as anti-LGI1 encephalitis,and had history of MG with thymoma. Her symptoms were improved by immunotherapy. Case 2, a 67-year-old male, was diagnosed as MG, and developed cognitive impairment, myokymia and autonomic dysfunction later. His serum autoimmune antibody testing showed positive contactin associated protein-like 2 antibody. Therefore, Morvan syndrome complicated with MG with thymoma was definitely diagnosed. After admission, the patient was improved with immunotherapy and thymoma resection.Conclusions:Patients with VGKCc antibody-associated clinical syndromes complicated with MG have the clinical characteristics of the two diseases simultaneously, and there is also crossover. Immunotherapy and treatment for thymoma are generally effective.
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ABSTRACT Autoimmune encephalitis (AE) comprises a group of diseases mediated by antibodies against neuronal cell surface or synaptic antigens, such as ion channels or neurotransmitter receptors. New clinical syndromes and their associated antibodies were and are still being characterized over the last two decades. The fact that their main clinical features are interdisciplinary, - encompassing neuropsychiatric symptoms, cognitive dysfunction, epileptic seizures, movement and sleep disorders - has led to a surge of interest in this field. Some of these diseases present with a well-defined syndrome, being recognizable on clinical grounds. Correct diagnosis is important since AE are potentially treatable diseases, despite their severity. On the other hand, an increasing number of neuronal antibodies being described casts doubt upon the way we should utilize antibody testing and interpret results. In this article we review, summarize and update the current knowledge on antibody mediated encephalitis.
RESUMO As encefalites autoimunes compreendem um grupo de doenças mediadas por anticorpos contra antígenos de superfície neuronal ou sinapse, como canais iônicos ou receptores de neurotransmissores. Novas síndromes clínicas e os anticorpos a elas associados foram e ainda estão sendo caracterizados ao longo das últimas duas décadas. Dado que suas principais características clínicas são interdisciplinares, isto é, incluem -se sintomas neuropisquiátricos, disfunção cognitiva, crises epilépticas, distúrbio do movimento e do sono, há uma grande onda de interesse sobre esse campo de conhecimento. Algumas dessas doenças apresentam-se com uma síndrome bem definida, sendo possível reconhecê-las clinicamente. Diagnosticá-las corretamente é importante uma vez que se trata de doenças potencialmente tratáveis apesar da gravidade que lhes é característica. Por outro lado, o número crescente de anticorpos sendo descritos causam dúvida frequente sobre qual o melhor teste a solicitar e como interpretá-los. Nós aqui apresentamos uma revisão atualização resumida do conhecimento atual sobre as encefalites mediadas por anticorpos.
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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.
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A case of limbic encephalitis with positive anti-leucine-rich glioma inactivated 1 protein (LGI1) antibody and anti-myelin oligodendrocyte glycoprotein (MOG) antibody was reported. The patient was a middle-aged male with a history of retinal vein occlusion. The main symptoms were temporal lobe epilepsy, facial arm dystonia, autonomic nerve dysfunction. Magnetic resonance imaging showed long T 2 signal in the right hippocampus without enhancement and normal perfusion. Electroencephalogram showed paroxysmal slow wave and sharp slow wave in interictal period. Blood anti-MOG antibody, blood and cerebrospinal fluid anti-LGI1 antibody were double positive. The main diagnosis was limbic encephalitis. After treatment with hormone and gamma globulin, the symptoms were improved and double antibodies were turned negative. Anti-LGI1/MOG double positive cases are rare, and the clinical manifestations and imaging manifestations of double positive antibody cases are not completely consistent with those with each single antibody, with different characteristics. This report can help clinicians enhance awareness.
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Objective:To analyse the clinical features of encephalitis patients with antibodies against the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR).Methods:Three anti-AMPAR encephalitis patients diagnosed in Tangdu Hospital, the Air Force Military Medical University between January 2020 and May 2021 were retrospectively reviewed. The clinical symptoms, supplementary examination, treatment options and outcomes with knowledge from literature were summarized in this study.Results:Three patients aging from 12 to 70 years presented with symptoms ranging from cognitive impairment, personality change to headache and paralysis. The lung occupying lesion was pathologically proved to be small cell lung cancer in case 1. Antibody to AMPAR (AMPAR-ab) was positive in both blood and cerebrospinal fluid of case 1, with coexisting antibodies against sex-determining region of Y chromosome-related high mobility group box 1 in blood, and the symptoms persisted but did not recur following therapy with corticosteroids. AMPAR-ab was detected only in serum in case 2, with the lesion located in both frontal and temporal lobes, centrum semiovale and lateral ventricle, combined with classic imaging features of intracranial hypotension, and the syndrome was partially improved following treatment with corticosteroids. The lesions were located in the pons and middle cerebellar peduncle, accompanied by cerebellar atrophy in case 3. Spinal cord magnetic resonance imaging showed long hyperintense lesions involving the cervical and thoracic cord, extending from C 2 to Th 10 level on T 2-weighted images. AMPAR-ab was positive in both serum and cerebrospinal fluid. And the symptoms improved significantly following treatment with corticosteroids and intravenous immunoglobulin. Conclusions:The clinical manifestations of anti-AMPAR encephalitis are highly heterogeneous, and brainstem and spinal cord can also be involved in addition to the limbic system, accompanied by brain atrophy. Combining with concurrent antibodies, especially the intracellular antibodies, malignancy needs to be closely monitored; the immunotherapy is effective and the presence of tumor superimposed with multiple antibodies may be associated with poor prognosis.
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Antibodies to glutamic acid decarboxylase (GAD) have been associated with several neurological syndromes, including stiff-person syndrome, cerebellar ataxia and epilepsy. This article critically reviews the main clinical characteristics and the evidence on the pathogenicity of GAD antibodies.
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Objective:To explore the clinical features, auxiliary examinations, therapies and prognoses of patients with antibodies targeting glutamic acid decarboxylase 65 (GAD65).Methods:The nine patients with anti-GAD65 neuroimmune disease, admitted to Xuanwu Hospital, Capital Medical University from October 2018 to October 2020, were analyzed, retrospectively.Results:The onset age of nine cases was 17-68 (43.6±20.5) years old, and six cases were female. Two cases had preceding infection. The data of initial symptoms were collected and analyzed, including epileptic onset in four cases, memory impairment in two cases, dizziness in two cases and limb stiffness in one case. As the disease continued to advance, one case developed cerebellar ataxia, one case presented with isolated epilepsy, five cases suffered from limbic encephalitis, one case had stiffman syndrome, one case had brainstem encephalitis. Five cases had antibodies against thyroid peroxidase. Brain magnetic resonance imaging scan showed abnormal signals of T 2/fluid attenuated inversion recovery sequences in four cases, mainly involved bilateral temporal lobes or hippocampus. Epileptiform discharges of frontal or temporal regions of electroencephalography were observed in six cases. All cases received immunotherapy and long-term follow-up was performed in seven cases. Four cases benefited from the immunotherapy. Among the four patients, one fully recovered and returned to work, the other three cases developed neurologic sequelae, including seizures (two cases), and short-term memory loss (one case). The remaining three patients were unresponsive to treatment. Conclusions:GAD65 antibody-mediated neuroimmune disease is a rare neurological disorder, presenting with various syndromes including limbic encephalitis or stiffman syndrome, which is more susceptible to young female. The clinical manifestations included epileptic onset, limb stiffness, cognitive impairment and cerebellar ataxia, etc. Detection of GAD65 antibody in serum or cerebrospinal fluid was gold standard. Early immunotherapy contributed to improving the prognosis of patients, especially for those patients with epileptic onset as the main symptom.
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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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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
Abstract Various neuropathologies produce hyperintense signals on T2-weighted or fluid-attenuated inversion recovery sequences of the temporal lobes. Recognition of the distribution pattern and associated findings may narrow the spectrum of differential diagnoses or suggest a specific disease. This pictorial essay aims to illustrate the relatively common diseases that affect the temporal lobe, such as herpes simplex encephalitis, neurosyphilis, limbic encephalitis, postictal edema, neoplasia, and multiple sclerosis, as well as those that are less common, such as myotonic dystrophy type 1, CADASIL, and CARASIL, together with the particularities of each entity.
Resumo Diversas neuropatologias apresentam hipersinal em T2 ou FLAIR nos lobos temporais, porém, o reconhecimento do padrão de distribuição e achados associados podem estreitar o espectro de diagnósticos diferenciais ou sugerir uma doença específica. Este ensaio iconográfico visa demonstrar doenças que acometem o lobo temporal e que são relativamente comuns no dia-a-dia dos radiologistas, como encefalite herpética, neurossífilis, encefalite límbica, edema pós-crise convulsiva, glioma e esclerose múltipla, e outras nem tão comuns como distrofia miotônica tipo I, CADASIL e CARASIL, atentando para as particularidades de cada entidade que auxiliam no diagnóstico.
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A encefalite límbica vem sendo descrita como um distúrbio neurológico raro, que afeta seletivamente as estruturas do sistema límbico. Clinicamente, é caracterizada como uma desordem neurológica debilitante, que se desenvolve como encefalopatia rapidamente progressiva, causada por inflamação encefálica. Objetivamos aqui relatar um caso de encefalite do sistema límbico de provável etiologia autoimune para melhor conhecimento da comunidade médica, bem como averiguar métodos diagnósticos deste quadro. Paciente do sexo masculino, 59 anos, admitido em nosso serviço com queixa de confusão mental. O exame clínico evidenciou desorientação, disartria, paresia e parestesia no hemicorpo esquerdo, dificuldade de marcha, desvio de rima e histórico de epilepsia há 2 anos. No estudo por ressonância magnética do crânio, foram observadas extensas lesões que acometiam a região mesial do lobo temporal direito, todo o hipocampo e giro para-hipocampal direito, estendendo-se pelo fórnix até a porção posterior do hipocampo esquerdo, substância branca do lobo frontal bilateral. Mediante os resultados da investigação complementar, o paciente foi tratado com pulsoterapia de metilpredinisolona por 5 dias, resultando na regressão parcial dos sintomas. Atualmente, o paciente se encontra em seguimento ambulatorial para acompanhamento. A encefalite límbica é uma doença rara, porém muito importante de ser investigada e diagnosticada precocemente, uma vez que a progressão da doença pode causar incapacidade e sequelas irreversíveis.
Limbic encephalitis has been described as a rare neurological disorder affecting the limbic system structures selectively. Clinically, it is characterized as a debilitating neurological syndrome that develops as a quickly progressive encephalopathy caused by brain inflammation. This paper reports a case of limbic encephalitis, probably of autoimmune etiology, aiming to improve the knowledge of the medical community, and to promote a debate on diagnosis methods for this pathology. The patient is male, 59 years old, and was admitted at our service complaining of mental confusion. The clinical examination showed disorientation, dysarthria, left hemiparesis and paresthesia, gait difficulties, light asymmetrical smile, and history of epilepsy 2 years ago. The magnetic resonance imaging of skull showed extensive lesions affecting the mesial region of the right temporal lobe, the entire hippocampus, and right parahippocampal gyrus, extending through the fornix to the posterior portion of the left hippocampus, white matter of bilateral frontal lobe. Based on the complementary investigation results, the patient was treated with intravenous methylprednisolone for five days. Currently, he is being followed in the outpatient's department. Although being rare, limbic encephalitis shall be investigated and diagnosed early because its progression can lead to disability and irreversible sequelae
Subject(s)
Humans , Male , Middle Aged , Autoimmunity , Limbic Encephalitis/diagnostic imaging , Paresis/etiology , Paresthesia , Carbamazepine/therapeutic use , Prednisone/therapeutic use , Magnetic Resonance Spectroscopy , Tomography, X-Ray Computed , Confusion/etiology , Limbic Encephalitis/complications , Limbic Encephalitis/immunology , Limbic Encephalitis/cerebrospinal fluid , Limbic Encephalitis/drug therapy , Limbic Encephalitis/blood , Limbic Encephalitis/virology , Dysarthria/etiology , Electroencephalography , Epilepsy/drug therapy , Hyponatremia , Anti-Inflammatory Agents/therapeutic use , Anticonvulsants/therapeutic use , Neurologic ExaminationABSTRACT
ABSTRACT The association between memory loss and Hodgkin's lymphoma has been given the eponym of Ophelia syndrome, in memory of Shakespeare's character in The Tragedy of Hamlet, Prince of Denmark. Nevertheless, there are differences between the disease and the character. Objective: To review the origins and uses of the eponym through an original article by pathologist Ian Carr, its relation to the character Ophelia, and the related autoantibodies. Methods: Historical narrative review. Results: Besides an eloquent description in the original article, Carr presaged the presence of autoantibodies, before they had been thoroughly researched. Since then, five different autoantibodies (mGluR5, Hu, NMDAR, SOX, PCA2) have been associated with Hodgkin's disease. It is interesting to note the divergent outcomes of Shakespeare's character and the patient in the original description by Carr, the latter recovering to lead a normal life, and the former deceased. Conclusions: Although there is little relationship between the fictional character and the syndrome, both imply the unintentional trigger of self-harm (suicide in one case, autoimmunity in the other), thus remaining associated.
RESUMO El síndrome de Ofelia describe la asociación entre pérdida de memoria y enfermedad de Hodgkin, en memoria del personaje de La Tragedia de Hamlet, Príncipe de Dinamarca, de William Shakespeare. Sin embargo, existen diferencias entre ambos. Objetivo: Revisar los orígenes y usos del epónimo a través del artículo original, su relación con el personaje y los autoanticuerpos relacionados. Métodos: Revisión narrativa histórica. Resultados: Además de una descripción elocuente, el artículo original prefigura los autoanticuerpos, cuando no se buscaban de rutina. Desde entonces, cinco distintos (mGluR5, Hu, NMDAR, SOX, PCA2) han sido asociados. Cabe destacar, que el desenlace del personaje y del paciente fueron diametralmente opuestos, el primero falleció y el segundo se recuperó, llevando una vida normal. Conclusiones: A pesar de la poca relación entre el personaje y el síndrome, ambos implican el desencadenamiento no intencional de daño auto-inflingido (suicidio en un caso, autoinmunidad en el otro), manteniendo así la adecuacía.
Subject(s)
Humans , Male , Female , History, 20th Century , Hodgkin Disease/immunology , Medicine in Literature , Memory Disorders/immunology , Autoantibodies , Syndrome , Limbic EncephalitisABSTRACT
Objective: To study the clinical characteristics of eucine-rich glioma-inactivated 1 protein (LGI1) antibody positive limbic encephalitis (LE), and to provide the reference for its diagnosis and streatment. Methods: The clinical data of a patient with anti-LGI1 antibody positive LE who presented the hypothalamus symptoms was analyzed, the literature review was performed, and the diagnosis and treatment methods of the LE patients were summarized. Results: The patient showed the following symptoms 2 months before hospitalisation, such as sexual dysfunction, overeating, taciturnity, daytime hyposia, intermittent sweating, fever without inducement, hyponatremia, intermittent memory loss and faciobrachial dystonic seizure (FBDS), and the above symptoms progressively exacerbated. After hospitalization, the Mini-Mental State Examination (MMSE) score was 22; the patient could' t finish the evaluation of Montreal Cognitive Assessment Scale (MoCA); the serum anti-LGI1 antibody was positive (1 100), and the cerebrospinal fluid anti-LGI1 antibody was positive (1:10). The MRI results showed T1 low signal, T2, Flair high signal, DWI high signal in bilateral hippocampus, amygdala and medial temporal lobe, especially in the right side. The patient was diagnosed as anti-LGIl antibody positive LE, whose symptoms relieved rapidly after administration of immunoglobulin and steroid therapy. After 6- monthfollow-up, the patient recovered completely. Conclusion: The positive results of serum and cerebrospinal fluid anti-LGIl antibody in the patients combined with the MRI results of head can confirm the diagnosis of anti-LGIl antibody positive LE. The hypothalamus symptoms, memory loss and FBDS are improved rapidly after immunoglobulin and steroid therapy. The hypothalamic injury possibly exist in the early-stage of anti-LGIl antibody positive LE.
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Paraneoplastic neurological syndromes (PNS) are a rare group of immune-mediated disorders that affect the central and peripheral neuromuscular system in association with cancer.If the limbal lobe system of the brain is involved,it will show paraneoplastic limbal encephalitis(PLE).The discern of patients with PNS is challenging since tumors causing paraneoplastic neurologic disorders are often asymptomatic and sometimes occult.We report a case of PLE with double positive anti-Hu and Yo antibodies,and further analyze and discuss it in conmbination with relevant literature to improve the understanding of the disease.
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Anti-Ma2-associated encephalitis is one of the paraneoplastic limbic and brainstem encephalitis characterized by decreased consciousness, parkinsonism and the limitation of vertical eye movement. It is usually associated with non-small cell lung cancer in male and female or germ cell tumor in male. Herein, we report a case of atypical anti-Ma2-associated encephalitis which presented with axonal sensorimotor polyneuropathy.
Subject(s)
Female , Humans , Male , Autoantibodies , Axons , Brain Stem , Carcinoma, Non-Small-Cell Lung , Consciousness , Encephalitis , Eye Movements , Limbic Encephalitis , Neoplasms, Germ Cell and Embryonal , Paraneoplastic Syndromes , Parkinsonian Disorders , PolyneuropathiesABSTRACT
Leucine-rich glioma-inactivated protein 1(LGI1)antibody-associated encephalitis is an autoimmune brain disease mainly seen in mid-aged and elderly people.Its main clinical manifestations include abnormal mental behaviors,facial-arm dystonia,hyponatremia,and hypokalemia.Immunotherapy with gamma globulin and/or hormone is effective.Two patients with LGT1 antibody-associated encephalitis were diagnosed in our center between January 2018 and October 2018,with typical clinical findings.The disease was cursed after immunoglobulin and hormone treatments.
Subject(s)
Humans , Autoantibodies , Allergy and Immunology , Encephalitis , Diagnosis , Therapeutics , Proteins , Allergy and ImmunologyABSTRACT
BACKGROUND@#The aim of this study was to investigate the clinical features of paraneoplastic limbic encephalitis (PLE) with small cell lung cancer (SCLC) and improve clinical diagnosis and treatment.@*METHODS@#The clinical data of 15 patients with SCLC combined with PLE from January 1980 to May 2017 were collected from Beijing Union Hospital. Their symptoms and laboratory data were analyzed and the prognosis of the patients was followed.@*RESULTS@#PLE is a rare disease, the incidence rate in SCLC is about 0.842%. The data may be underestimated because of misdiagnose or missed diagnosis; High incidence crowd of the disease is the middle-aged male smoker, the tumor-node-metastasis (TNM) stages of them are later than others; Typical neurological symptoms include varying degrees of short-term memory loss, seizures and varying degrees of mental disorders; neurological symptoms usually occur before the onset of cancer or respiratory symptoms appear, an average of about 2 months be taken from onset to diagnosis; Serum antibody (anti-Hu, GABA-R-Ab), cerebrospinal fluid, head magnetic resonance imaging (MRI) and electroencephalogram (EEG) of the patients has abnormalities; Videography, especially computed tomography (CT) is a good means of screening the primary tumor, pathology diagnosis mainly rely on bronchoscopy; The treatment of primary tumors can be more effective in alleviating the nervous system symptoms than immunotherapy.@*CONCLUSIONS@#Paraneoplastic limbic encephalitis is a rare paraneoplastic syndrome in nervous system caused by malignant neoplasms often characterized by facial neurological symptoms. The disease are usually associated with lung cancer (especially SCLC). Its nervous system symptoms occur earlier than the tumor diagnosis. Early diagnosis and treatment for primary tumors will increase the benefit.