Publications by authors named "Franziska Thaler"
Introduction: Very rarely, adult NMDAR antibody-associated encephalitis (NMDAR-E) leads to persistent cerebellar atrophy and ataxia. Transient cerebellar ataxia is common in pediatric NMDAR-E. Immune-mediated cerebellar ataxia may be associated with myelin oligodendrocyte glycoprotein (MOG), aquaporin-4 (AQP-4), kelch-like family member 11 (KLHL11), and glutamate kainate receptor subunit 2 (GluK2) antibodies, all of which may co-occur in NMDAR-E.
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- The BAFF-APRIL system is important in systemic lupus erythematosus (SLE) as it helps B cells survive and contribute to autoimmunity; this study examined BCMA expression in B cell subsets in SLE patients and healthy controls.
- SLE patients showed higher BCMA expression on B cells compared to healthy controls, with notable increases in memory B cells and a correlation between BCMA levels and disease markers like anti-dsDNA antibodies.
- Belimumab treatment reduced BCMA expression and other components of the BAFF-APRIL system, indicating its potential as both a treatment strategy and a biomarker for monitoring disease activity in SLE.
Article Synopsis
- - Autoantibodies against Caspr2 are linked to limbic autoimmune encephalitis and pain, with 36% of patients in a study experiencing pain, which is often severe and sometimes the main symptom.
- - Two main pain phenotypes were identified: distal-symmetric burning pain and widespread pain with myalgia/cramps, highlighting variability among patients.
- - There is a correlation between anti-Caspr2 autoantibodies and pre-existing chronic pain risk factors, suggesting that these autoantibodies could indicate decreased pain sensitivity, warranting testing in patients with different types of pain.
Early Neuroaxonal Damage in Neurologic Disorders Associated With GAD65 Antibodies.
Neurol Neuroimmunol Neuroinflamm
January 2024
Article Synopsis
- Neurodegeneration is a significant factor in neurologic disorders linked to GAD65 antibodies, and this study explores neuroaxonal damage's relationship with disease duration and clinical symptoms.
- In a study involving 50 patients, serum neurofilament light chain (sNfL) levels were measured and correlated with disease progression and clinical presentations, using MRI to assess brain structure changes.
- The findings show elevated sNfL levels early in the disease, especially in patients with cerebellar ataxia and limbic encephalitis, alongside atrophy in specific brain regions, highlighting the importance of early intervention in these conditions.
Article Synopsis
- Anti-septin-5 encephalitis is a rare neurological condition characterized by symptoms like cerebellar ataxia and oculomotor issues, with limited treatment options available.
- A case study of a 54-year-old woman shows the diagnostic process, treatment methods, and follow-up results, including severe symptoms and positive anti-septin-5 IgG tests in her CSF and serum.
- The patient improved with treatments like corticosteroids and plasma exchange, although she experienced relapses; ultimately, bortezomib provided moderate and sustained improvement, highlighting its potential effectiveness.
The BAFF/APRIL-system with the two cytokines BAFF and APRIL and their three receptors, transmembrane activator and CAML interactor (TACI), BAFF receptor, and B-cell maturation Ag, is important for B cell maintenance. The BAFF/APRIL system is a therapeutic target in B cell-derived malignancies and autoimmune diseases. However, unexpected outcomes of clinical trials with atacicept (TACI-Fc) underline our incomplete understanding of this system.
View Article and Find Full Text PDFBackground And Objectives: Antibodies (Abs) against the cytoplasmic protein glutamic acid decarboxylase 65 (GAD65) are detected in patients with neurologic syndromes together referred to as GAD65-Ab spectrum disorders. The response of some of these patients to plasma exchange or immunoglobulins indicates that GAD65-Abs could contribute to disease pathogenesis at least at some stages of disease. However, the involvement of GAD65-reactive B cells in the CNS is incompletely understood.
View Article and Find Full Text PDFOcrelizumab Treatment Modulates B-Cell Regulating Factors in Multiple Sclerosis.
Neurol Neuroimmunol Neuroinflamm
March 2023
Article Synopsis
- Ocrelizumab, an anti-CD20 treatment, effectively reduces B cell populations in multiple sclerosis (MS) patients but has complex effects on immune systems, particularly related to the BAFF-APRIL system.* -
- The research analyzed immune cell changes over 2.5 years, revealing that ocrelizumab led to increased BAFF levels and decreased free soluble receptors like sTACI in both serum and cerebrospinal fluid (CSF).* -
- The reduction of sTACI, which normally acts as a decoy for APRIL, may enhance APRIL's activity, potentially affecting the regulation of immune responses in MS and promoting certain plasma cell types.*
Article Synopsis
- High levels of antibodies against glutamic acid decarboxylase (GAD-abs) are linked to various autoimmune neurological syndromes, while the significance of low serum levels is still debated.
- A study analyzed data from 101 patients suspected of having GAD-ab-associated neurological issues, revealing that most presented with conditions like epilepsy and cerebellar ataxia but had mixed outcomes with immunotherapy.
- The research found that low GAD-ab serum levels didn't significantly affect clinical characteristics or outcomes, suggesting that detecting unmatched oligoclonal bands in the cerebrospinal fluid may be more important than GAD-ab levels.
Seizure Semiology in Antibody-Associated Autoimmune Encephalitis.
Neurol Neuroimmunol Neuroinflamm
November 2022
Article Synopsis
- - The study evaluates seizure characteristics in patients with antibody-associated autoimmune encephalitis (ab + AE) focusing on the three most common antibodies: NMDAR, LGI1, and GAD, involving 320 patients across multiple centers in Germany.
- - Seizures were prevalent in these patients, with frequencies of 60% in NMDAR+, 78% in LGI1+, and 65% in GAD+, and certain types of seizures such as faciobrachial dystonic seizures and status epilepticus presented uniquely or more frequently in specific antibody groups.
- - The findings suggest that seizure types can help in diagnosis, with distinct patterns observed among different antibodies, indicating that NMDAR+ patients tend to have
Article Synopsis
- Autoimmune neurological syndromes (AINS) associated with anti-GAD65 autoantibodies lead to various neurological symptoms, including seizures and cerebellitis, and also have a connection to autoimmune diabetes.
- A genome-wide association study (GWAS) in a German cohort revealed 16 significant genetic loci linked to susceptibility to AINS, with a notable variant in the HLA class I region.
- Over 40% of identified genetic variants affect the expression of genes in immune and neural cells, emphasizing the relationship between immune response and neurological function through specific pathways.
Anti-IgLON5 disease is a newly defined clinical entity characterized by a progressive course with high disability and mortality rate. While precise pathogenetic mechanisms remain unclear, features characteristic of both autoimmune and neurodegenerative diseases were reported. Data on immunotherapy are limited, and its efficacy remains controversial.
View Article and Find Full Text PDFCSF Findings in Acute NMDAR and LGI1 Antibody-Associated Autoimmune Encephalitis.
Neurol Neuroimmunol Neuroinflamm
November 2021
Article Synopsis
- * Researchers analyzed CSF from 82 patients with NMDAR-E and 36 with LGI1-E, looking at various parameters like white blood cells and immunoglobulin levels, finding abnormalities in 94% of NMDAR-E patients compared to 36% of LGI1-E patients.
- * Key findings include that high levels of immunoglobulin synthesis and CSF leukocytes were characteristic of NMDAR-E, while these traits were absent in LG
Article Synopsis
- A study analyzed the use of rituximab in patients with autoimmune encephalitis (AE), particularly focusing on those with NMDA receptor (NMDAR) and other related conditions, to see how it correlates with long-term outcomes.
- Out of 358 patients, 163 received rituximab, with treatment starting significantly earlier for NMDAR and LGI1-AE, showing improved functional outcomes and reducing relapse rates compared to those who didn't receive the drug.
- The findings suggest that early initiation of rituximab therapy is potentially effective and safe for treating several types of AE, highlighting its benefits especially for NMDAR and LGI1 cases.
Background And Objectives: Neurotropic viruses are suspected to play a role in the pathogenesis of autoimmune diseases of the CNS such as the association between the Epstein-Barr virus (EBV) and multiple sclerosis (MS). A group of autoimmune encephalitis (AE) is linked to antibodies against neuronal cell surface proteins. Because CNS infection with the herpes simplex virus can trigger anti-NMDA receptor (NMDAR) encephalitis, a similar mechanism for EBV and other neurotropic viruses could be postulated.
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- Anti-IgLON5 disease is a newly identified neurological disorder characterized by autoimmunity and neurodegeneration, with a substantial prevalence of abnormal movements that have been largely overlooked.
- In a study of 72 patients, 87% exhibited at least one movement disorder, with gait and balance issues being the most common, followed by chorea and bradykinesia.
- The majority of patients experienced additional symptoms such as sleep disturbances, bulbar dysfunction, and cognitive impairment, and many showed significant improvement in movement disorders after receiving immunotherapy treatment.
Article Synopsis
- Autoimmune disorders of the central nervous system (CNS) are diverse, and understanding the specific autoantigens is crucial for improving therapy and understanding disease causes.
- Researchers studied oligodendrocyte myelin glycoprotein (OMGP) as a potential autoimmune target and found autoantibodies to OMGP in a small percentage of multiple sclerosis patients, a child with encephalomyelitis, and one patient with psychosis, but not in healthy controls.
- The presence of OMGP-specific T cells in an animal model led to a new type of experimental autoimmune encephalomyelitis characterized by unusual inflammation, emphasizing the role of OMGP in patient diagnostic and therapeutic stratification.
Article Synopsis
- The study focused on patients with GAD-antibody associated neurological disorders, investigating the presence of other autoimmune disorders in those patients.
- Researchers analyzed variations in 33 candidate genes using next-generation sequencing, observing significant genetic alterations in certain genes related to autoimmunity, such as CTLA4.
- Findings revealed that many patients had additional autoimmune disorders and suggested the presence of genetic risk factors linked to GAD-ab-associated neurological conditions.
Identification of circulating MOG-specific B cells in patients with MOG antibodies.
Neurol Neuroimmunol Neuroinflamm
November 2019
Article Synopsis
- The study aimed to identify circulating MOG-specific B cells in the blood of patients with MOG antibodies and investigate their relationship with serum anti-MOG antibody levels and epitope specificity.
- Researchers analyzed blood samples from 21 MOG-antibody-positive patients and 26 controls, finding that a higher frequency of MOG-specific B cells was present in patients, but only about 60% of them tested positive for these B cells.
- The findings suggest that the quantity of circulating MOG-specific B cells varies significantly among patients and does not correlate with serum MOG antibody levels, indicating potential differences in the sources of these antibodies, which could inform future patient-specific treatments.
High levels of antibodies against glutamic acid decarboxylase (GAD) are observed in patients with different neurological disorders, but cells producing these autoantibodies are largely unexplored. We detect circulating GAD-reactive B cells in peripheral blood that readily differentiate into antibody-producing cells. These cells are highly elevated in most patients with GAD-antibody-associated disorders (n = 15) compared to controls (n = 19).
View Article and Find Full Text PDFManagement and prognostic markers in patients with autoimmune encephalitis requiring ICU treatment.
Neurol Neuroimmunol Neuroinflamm
January 2019
Article Synopsis
- The study aimed to evaluate complications in ICU patients with autoimmune encephalitis (AE), their management, and factors affecting recovery outcomes.
- It involved 120 patients across various hospitals, finding that many experienced severe issues like disorders of consciousness and sepsis, with mechanical ventilation being a major risk factor for poor recovery.
- The findings suggest that common ICU complications are better predictors of outcomes than specific types of AE, highlighting the need for effective management and further research in this area.