-
Neuroradiology May 2024Autoimmune encephalitis is a relatively novel nosological entity characterized by an immune-mediated damage of the central nervous system. While originally described as... (Review)
Review
Autoimmune encephalitis is a relatively novel nosological entity characterized by an immune-mediated damage of the central nervous system. While originally described as a paraneoplastic inflammatory phenomenon affecting limbic structures, numerous instances of non-paraneoplastic pathogenesis, as well as extra-limbic involvement, have been characterized. Given the wide spectrum of insidious clinical presentations ranging from cognitive impairment to psychiatric symptoms or seizures, it is crucial to raise awareness about this disease category. In fact, an early diagnosis can be dramatically beneficial for the prognosis both to achieve an early therapeutic intervention and to detect a potential underlying malignancy. In this scenario, the radiologist can be the first to pose the hypothesis of autoimmune encephalitis and refer the patient to a comprehensive diagnostic work-up - including clinical, serological, and neurophysiological assessments.In this article, we illustrate the main radiological characteristics of autoimmune encephalitis and its subtypes, including the typical limbic presentation, the features of extra-limbic involvement, and also peculiar imaging findings. In addition, we review the most relevant alternative diagnoses that should be considered, ranging from other encephalitides to neoplasms, vascular conditions, and post-seizure alterations. Finally, we discuss the most appropriate imaging diagnostic work-up, also proposing a suggested MRI protocol.
Topics: Humans; Encephalitis; Hashimoto Disease; Autoantibodies; Seizures; Radiologists; Autoimmune Diseases of the Nervous System; Limbic Encephalitis
PubMed: 38507081
DOI: 10.1007/s00234-024-03318-x -
Arquivos de Neuro-psiquiatria Oct 2012Autoimmune encephalitis is an inflammatory disorder characterized by a subacute impairment of short-term memory, psychiatric features and seizures. It is often... (Review)
Review
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.
Topics: Autoimmune Diseases of the Nervous System; Diagnosis, Differential; Humans; Immunotherapy; Limbic Encephalitis; Prognosis
PubMed: 23060110
DOI: 10.1590/s0004-282x2012001000012 -
Clinical Medicine (London, England) Oct 2011Autoimmune limbic encephalitis is an increasingly recognised cause of cognitive decline and confusion. The typical presentation is with subacute cognitive decline,...
Autoimmune limbic encephalitis is an increasingly recognised cause of cognitive decline and confusion. The typical presentation is with subacute cognitive decline, behavioural disturbance and seizures. Magnetic resonance imaging may show characteristic changes in the medial temporal regions. The diagnosis is confirmed by identification of elevated voltage-gated potassium channel antibody (VGKC-Ab) titres. It is a highly treatable condition, often responding well to intravenous immunoglobulin or steroids. Recognition of autoimmune limbic encephalitis is sometimes delayed--usually because the diagnosis has not been considered--which can result in long-term neurological consequences.
Topics: Autoantibodies; Autoimmune Diseases of the Nervous System; Cognition Disorders; Diagnosis, Differential; Disease Progression; Epilepsy, Tonic-Clonic; Fatal Outcome; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Limbic Encephalitis; Magnetic Resonance Imaging; Male; Middle Aged; Potassium Channels, Voltage-Gated; Prognosis
PubMed: 22034711
DOI: 10.7861/clinmedicine.11-5-476 -
Frontiers in Immunology 2021The epidemiological characteristics of patients with antibody-medicated autoimmune encephalitis in China remain unclear, and a large-scale epidemiological survey is...
The epidemiological characteristics of patients with antibody-medicated autoimmune encephalitis in China remain unclear, and a large-scale epidemiological survey is necessary. A multiple-center retrospective study was performed. We collected 1,047 patients with suspected autoimmune encephalitis and ultimately enrolled 778 defined patients across centers in China. All patients were positive for serum [or cerebrospinal fluid (CSF)] antibodies. Demographic information and clinical data from January 2014 to January 2019 from 22 centers in China were reviewed. A total of 778 patients with autoimmune encephalitis were enrolled in the study. In general, the ratio of males to females was ~1.2:1. The main subtypes of autoimmune encephalitis were NMDAR-AE (61.35%), LGI-1-AE (20.61%), and GABAbR-AE (12.40%). According to the characteristics of age of onset, the incidence of autoimmune encephalitis showed a "double peak" distribution entailing a 20-year-old age group and a 60-year-old age group. We next analyzed the proportion of patients with tumors in this cohort. More specifically, there were 34 patients with tumors and 85 with tumor marker positivity. Relapse occurred in 81 patients within at least 1 year's follow up study: 52 with NMDAR-AE (18.2%); 19 with LGI-1-AE (16.8%); 5 with GABAbR-AE (9%); and 3 with CASPR2-AE. Due to the vast differences in demographic features, the incidence of cancer and the genetic characteristics between the populations in China and Western countries, the demographics, sex distribution, concomitant tumor rate, clinical features, and relapse characteristics associated with autoimmune encephalitis in China shows a similar profile with Western countries with some minor differences.
Topics: Adolescent; Adult; Age Factors; Aged; Aged, 80 and over; Child; Child, Preschool; China; Female; Follow-Up Studies; Humans; Infant; Intracellular Signaling Peptides and Proteins; Limbic Encephalitis; Male; Middle Aged; Neoplasms; Receptors, GABA-B; Receptors, N-Methyl-D-Aspartate; Retrospective Studies; Sex Factors; Treatment Outcome; Young Adult
PubMed: 33679765
DOI: 10.3389/fimmu.2021.621599 -
Journal of Clinical Tuberculosis and... Feb 2020Limbic encephalitis represents an autoimmune disorder that is commonly associated with malignancies. It is also seen in association with infectious or systemic...
INTRODUCTION
Limbic encephalitis represents an autoimmune disorder that is commonly associated with malignancies. It is also seen in association with infectious or systemic autoimmune diseases. The literature reports two case reports of limbic encephalitis associated with tuberculosis.
CASE REPORT
We report the case of a 42 year-old male referred to our clinic for a non-resolving pneumonia. He was found to have a limbic encephalitis associated with mediastinal tuberculous lymphadenitis. The diagnosis was made on a needle aspirate from a mediastinal lymph node obtained through endobronchial ultrasound. A paradoxical radiological progression was noted during therapy. He was successfully treated by anti-tuberculous drugs with clinical and radiological improvement.
CONCLUSION
Limbic encephalitis is associated with tuberculosis and should be included as part of the central nervous system involvement with tuberculosis. Endobronchial ultrasound has been shown to be useful in the diagnosis of mediastinal tuberculous lymphadenitis.
PubMed: 31956698
DOI: 10.1016/j.jctube.2019.100129 -
Cureus May 2022The limbic system (LS) coordinates an important role in memory generation, creating an emotional response to stress, and helping regulate autonomic and endocrine...
The limbic system (LS) coordinates an important role in memory generation, creating an emotional response to stress, and helping regulate autonomic and endocrine functions. Dysfunction of the limbic system can present secondary to many pathologies including autoimmune, infectious, paraneoplastic, etc. Lesions to the limbic system can also lead to varying symptoms which can be challenging for physicians to correctly identify and treat. Here we report a 59-year-old male with aggressive mood changes and acute onset of auditory and visual hallucinations. The cerebrospinal fluid (CSF) and serum immunological antibody panel confirmed the presence of voltage-gated potassium channel (VGKC) antibodies. Significant radiographic findings included an MRI revealing T2 hyperintensities in the bilateral hippocampus. Paraneoplastic screening with testicular ultrasound and chest CT was completed and was negative. A primary diagnosis of voltage-gated potassium channel limbic encephalitis (VGKC-LE) was made. Management included five days of intravenous immunoglobulin (IVIG) with subsequent resolution of symptoms. The limbic system is an intricate network of neurons that generates and relays key information to other parts of the brain. Its function and, in this case, its dysfunction remain an area of continued research. This case aimed to highlight the importance of recognizing the clinical presentation and objective findings of a rare type of autoimmune encephalitis and identifies the significance of paraneoplastic screening.
PubMed: 35747055
DOI: 10.7759/cureus.25186 -
Neurology(R) Neuroimmunology &... Jul 2023Limbic encephalitis (LE) is an autoimmune disease often associated with temporal lobe epilepsy and subacute memory deficits. It is categorized into serologic subgroups,...
BACKGROUND AND OBJECTIVES
Limbic encephalitis (LE) is an autoimmune disease often associated with temporal lobe epilepsy and subacute memory deficits. It is categorized into serologic subgroups, which differ in clinical progress, therapy response, and prognosis. Using longitudinal MRI analysis, we hypothesized that mesiotemporal and cortical atrophy rates would reveal serotype-specific patterns and reflect disease severity.
METHODS
In this longitudinal case-control study, all individuals with antibody-positive (glutamic acid decarboxylase 65 [GAD], leucine-rich glioma-inactivated protein 1 [LGI1], contactin-associated protein 2 [CASPR2], and -methyl-d-aspartate receptor [NMDAR]) nonparaneoplastic LE according to Graus' diagnostic criteria treated between 2005 and 2019 at the University Hospital Bonn were enrolled. A longitudinal healthy cohort was included as the control group. Subcortical segmentation and cortical reconstruction of T1-weighted MRI were performed using the longitudinal framework in FreeSurfer. We applied linear mixed models to examine mesiotemporal volumes and cortical thickness longitudinally.
RESULTS
Two hundred fifty-seven MRI scans from 59 individuals with LE (34 female, age at disease onset [mean ± SD] 42.5 ± 20.4 years; GAD: n = 30, 135 scans; LGI1: n = 15, 55 scans; CASPR2: n = 9, 37 scans; and NMDAR: n = 5, 30 scans) were included. The healthy control group consisted of 128 scans from 41 individuals (22 female, age at first scan [mean ± SD] 37.7 ± 14.6 years). The amygdalar volume at disease onset was significantly higher in individuals with LE ( 0.048 for all antibody subgroups) compared with that in healthy controls and decreased over time in all antibody subgroups, except in the GAD subgroup. We observed a significantly higher hippocampal atrophy rate in all antibody subgroups compared with that in healthy controls (all 0.002), except in the GAD subgroup. Cortical atrophy rates exceeded normal aging in individuals with impaired verbal memory, while those who were not impaired did not differ significantly from healthy controls.
DISCUSSION
Our data depict higher mesiotemporal volumes in the early disease stage, most likely due to edematous swelling, followed by volume regression and atrophy/hippocampal sclerosis in the late disease stage. Our study reveals a continuous and pathophysiologically meaningful trajectory of mesiotemporal volumetry across all serogroups and provides evidence that LE should be considered a network disorder in which extratemporal involvement is an important determinant of disease severity.
Topics: Humans; Female; Young Adult; Adult; Middle Aged; Limbic Encephalitis; Case-Control Studies; Antibodies; Magnetic Resonance Imaging; Glutamate Decarboxylase; Memory Disorders
PubMed: 37230543
DOI: 10.1212/NXI.0000000000200125 -
Seizure Mar 2013In people with suspected inflammatory CNS disease, cerebrospinal fluid (CSF) is commonly analyzed. Antibody-associated limbic encephalitis (ab-LE) and... (Review)
Review
PURPOSE
In people with suspected inflammatory CNS disease, cerebrospinal fluid (CSF) is commonly analyzed. Antibody-associated limbic encephalitis (ab-LE) and anti-NMDAR-encephalitis are recognized as two major syndromes of autoimmune epilepsies. Here, we investigated the diagnostic value of CSF findings in these two entities.
METHODS
We reviewed patients from our tertiary epilepsy centre with ab-LE and anti-NMDAR-encephalitis in whom CSF examination including oligoclonal bands (OCB) was performed. Ab-LE patients were subdivided according to antibodies (voltage-gated potassium channels, VGKC; glutamic acid decarboxylase, GAD) or presence of onconeural antibodies/presence of tumour into three groups: VGKC-LE, GAD-LE or paraneoplastic LE (PLE). As controls, patients with CSF investigations in whom autoimmune origin was initially assumed but not confirmed later on were included. In addition, a review of published ab-LE and anti-NMDAR-encephalitis cases with reported CSF data was performed.
RESULTS
55 ab-LE (23 VGKC-LE, 25 GAD-LE, 7 PLE) and 14 anti-NMDAR-encephalitis patients were identified at our centre. OCB were significantly more frequent in ab-LE and anti-NMDAR-encephalitis than in controls. Literature review identified 150 ab-LE and 95 NMDAR cases. Analysis of pooled data confirmed that presence of OCB was significantly more frequent in ab-LE and anti-NMDAR-encephalitis (especially in people with GAD-LE and anti-NMDAR encephalitis) as compared to controls. Sensitivity and specificity of OCB in the pooled ab-LE and anti-NMDAR-encephalitis patients was 34% and 96%, respectively. In patients with ab-LE and anti-NMDAR-encephalitis, the likelihood of OCB in CSF was 8.5-fold higher as compared to controls. Furthermore, in the pooled ab-LE and anti-NMDAR-encephalitis patients, cell counts in CSF were more frequently elevated (especially in those with anti-NMDAR encephalitis) than in controls, whereas protein content of CSF was not different between the groups.
CONCLUSION
OCB, and to a lesser extent cell counts in CSF, appear to be helpful additional CSF markers in the diagnostic evaluation of people presenting with a constellation suggestive for GAD-LE, PLE and anti-NMDAR-encephalitis, prompting subsequent analysis of specific antibodies.
Topics: Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Autoantibodies; Biomarkers; Humans; Limbic Encephalitis; Nerve Tissue Proteins; Receptors, N-Methyl-D-Aspartate; Retrospective Studies
PubMed: 23318046
DOI: 10.1016/j.seizure.2012.12.013 -
Brain and Behavior Mar 2020AMPA receptor (AMPAR) and CRMP5 antibodies are relatively uncommon in limbic encephalitis, and patients with both antibodies are rare. We recently treated such a... (Review)
Review
AIMS
AMPA receptor (AMPAR) and CRMP5 antibodies are relatively uncommon in limbic encephalitis, and patients with both antibodies are rare. We recently treated such a patient, but the patient died after active treatment. To further understand this disease, we conducted a case report and literature review.
DISCUSSIONS
To date, five encephalitis patients, including our patient, have been found to be positive for AMPAR and CRMP5 antibodies. The male-to-female ratio of the reported cases is 4:1, and the age range is 26 and 62 years old. All five patients presented with various neuropsychiatric symptoms, including insomnia, abnormal behavior, seizures, extrapyramidal symptoms, and autonomic dysfunction. Four patients had tumors (three invasive thymomas and one suspected lymphoma), and three cases died within a short period of time. No tumor was detected in one of the patients during the follow-up period; however, after active treatment, the outcome was poor, and the patient developed cachexia. One patient had good response to immunotherapy and tumor therapy and successfully returned to work.
CONCLUSIONS
The prognosis of encephalitis associated with AMPAR and CRMP5 antibodies is worse than that of the encephalitis associated with AMPAR antibodies alone. The most likely cause is that this encephalitis is more likely to be accompanied by malignant tumors, leading to a poor prognosis. In addition, it may also be due to some synergistic mechanisms between the two antibodies. Further studies aimed at the prognosis of this type of encephalitis are warranted.
Topics: Adult; Autoantibodies; Female; Humans; Hydrolases; Limbic Encephalitis; Male; Microtubule-Associated Proteins; Middle Aged; Prognosis; Receptors, AMPA; Seizures
PubMed: 31991060
DOI: 10.1002/brb3.1528 -
AJNR. American Journal of Neuroradiology Dec 2015Limbic encephalitis is far more common than previously thought. It is not always associated with cancer, and it is potentially treatable. Autoantibodies against various... (Review)
Review
Limbic encephalitis is far more common than previously thought. It is not always associated with cancer, and it is potentially treatable. Autoantibodies against various neuronal cell antigens may arise independently or in association with cancer and cause autoimmune damage to the limbic system. Neuroimaging plays a key role in the management of patients with suspected limbic encephalitis by supporting diagnosis and excluding differential possibilities. This article describes the main types of autoimmune limbic encephalitis and its mimic disorders, and emphasizes their major imaging features.
Topics: Autoantibodies; Autoimmune Diseases; Diagnosis, Differential; Encephalitis; Hashimoto Disease; Humans; Limbic Encephalitis; Neuroimaging
PubMed: 26381566
DOI: 10.3174/ajnr.A4408