Publications by authors named "Jeremias Motte"

Background: The short-chain fatty acid propionate has been shown to attenuate atherosclerosis, for example by modulating intestinal cholesterol metabolism. Beyond cholesterol metabolism, data from rodent models suggest a potential additional effect on blood pressure reduction.

Material And Methods: We conducted a prospective uncontrolled, exploratory, and observational study on 58 individuals receiving propionate supplementation (500 mg twice daily) without preexisting atherosclerotic cardiovascular disease, assessing peripheral and central blood pressure, arterial stiffness, and endothelial function.

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Collapse of self-tolerance toward peripheral nervous system antigens initiates chronic inflammatory demyelinating polyneuropathy. This breakdown likely recurs, driving disease onset and flare-ups, providing a window to predict progression before symptoms worsen, yet the mechanisms behind self-tolerance maintenance or disruption remain underexplored. Using a transgenic mouse model with Schwann cell-restricted ovalbumin expression and adoptive transfer of ovalbumin reactive CD8 T-cells, we demonstrate that maintenance of immune tolerance to peripheral nervous system antigen is linked to PD1-axis activity.

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Background: Autoimmune encephalitides are a heterogeneous group of autoantibody-associated central nervous system disorders. The clinical course of autoimmune encephalitides can be life threatening, and treatment can be challenging.

Objective: This report describes a case of treatment-refractory, anti-diacylglycerol lipase alpha (DAGLA) antibody-associated autoimmune encephalitis successfully treated with chimeric antigen receptor (CAR) T cells.

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Background: The novel criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) have established imaging with nerve ultrasound (NUS) and magnetic resonance neurography (MRN) as complementary methods for CIDP diagnosis.

Objectives: Our goal was to investigate the role of MRN and NUS for CIDP monitoring.

Methods And Design: We longitudinally examined 12 CIDP patients from 2016 to 2022 using NUS, MRN, nerve conduction studies (NCS), and clinical parameters (inflammatory neuropathy cause and treatment (INCAT)/overall disability sum score (ODSS)).

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Background And Objectives: The aim of this study was to characterize serum neurofilament light chain (sNFL) levels in a large cohort of patients with autoimmune neuropathies to provide every-day clinical practice recommendations.

Methods: In this retrospective cohort study, we recruited 191 patients with immune-mediated neuropathies from 2 referral centers. sNFL was measured using the Simoa NF-light kit (Quanterix), and age-corrected and BMI-corrected z-scores (zNFL) were calculated.

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Background: Short-chain fatty acids (SCFAs), including propionic acid (PA), are key in immunological research. Supplementing PA has shown benefits for autoimmune diseases. A comprehensive understanding of the PA pharmacokinetics is essential for the optimal design and execution of studies utilizing orally administered PA.

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Background: Diagnosing chronic inflammatory demyelinating polyneuropathy (CIDP) can be challenging, leading to delays in initiating therapy. As disability in CIDP is mainly dependent on axonal damage, the impact of delayed immunotherapy remains unclear. We multimodally investigated the clinical outcomes of patients with early CIDP regarding different treatment strategies and time points.

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Objective: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune neuropathy characterized by progressive or relapsing-remitting weakness and sensory deficits. This study aims to evaluate the utility of corneal confocal microscopy (CCM) in diagnosing and monitoring CIDP.

Methods: We analysed 100 CIDP patients and 31 healthy controls using CCM to measure corneal nerve fiber density (CNFD), length (CNFL), and branch density (CNBD).

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Background: Neurosarcoidosis is a rare entity, usually within the context of systematic sarcoidosis. Isolated neurosarcoidosis and especially a manifestation with pachymeningitis is a notable rarity.

Case Report: A 26-year-old patient presented to the emergency department with acute onset, recurrent episodes of occipital headaches spreading over the whole cranium and vomiting without food consumption, for three days.

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Article Synopsis
  • Vagal atrophy is a significant feature of Parkinson's disease and is linked to autonomic dysfunction, prompting a study on the vagus nerve in atypical Parkinsonian syndromes like multiple system atrophy (MSA) and progressive supranuclear palsy (PSP).
  • The study compared vagus nerve characteristics across MSA and PSP patients, Parkinson's disease patients, and healthy individuals to explore their potential as biomarkers for orthostatic dysregulation.
  • Results showed a high prevalence of abnormal autonomic responses in MSA and PSP, with a correlation between the size of the vagus nerve and autonomic function in these patients, highlighting differences in vagal atrophy compared to healthy controls.
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  • A study found that polyneuropathy (PNP) is common in patients with Parkinson's disease (PD), affecting around 65.85% of participants at the start and deteriorating in 21.95% over 2 years.
  • The research included comprehensive evaluations and nerve conduction studies to assess PNP, which was linked to older age and more severe PD symptoms.
  • The findings highlight the need for more extensive studies to understand the relationship between PD and PNP, particularly the progression and underlying mechanisms involved.
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Introduction: Ofatumumab (Kesimpta) is a subcutaneous CD20-targeting antibody approved in Germany in 2021 for the treatment of relapsing multiple sclerosis (RMS). After careful instruction, patients can administer the treatment themselves. We previously reported data of 101 patients (Klimas et al.

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  • Multiple sclerosis is a disease where the immune system attacks the nervous system, making it hard to find effective treatments to fully fix it.
  • Scientists are studying how diet, especially high-fiber foods, can help by producing beneficial substances like propionic acid, which might help protect nerve cells.
  • In experiments, they found that propionic and butyric acid helped damaged nerve cells recover better, which could lead to new ways to help people with multiple sclerosis.
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  • Autologous chimeric antigen receptor (CAR) T cell therapy has shown promise in treating neuroimmunological disorders like myasthenia gravis, and this study details a case of its successful application for stiff-person syndrome (SPS).
  • A 69-year-old woman with severe, treatment-resistant SPS underwent an infusion of anti-CD19 CAR T cells, leading to significant improvements in her symptoms, including reduced leg stiffness and increased walking capacity.
  • The treatment was well tolerated with minimal side effects, encouraging further exploration of CAR T cell therapy for SPS and other autoimmune disorders involving B cells.
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Introduction: The value of a sural nerve biopsy for the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is controversial. Evidence-based recommendations for its implementation are lacking. We investigated factors leading to biopsy and analyzed biopsy outcomes and consequences, assessed the predictability of biopsy outcomes through clinical parameters to avoid unnecessary biopsies, and compared results with electrophysiological and clinical severity to determine their prognostic value.

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  • * Two female patients with both MG and LEMS saw little improvement from various immunotherapy options but experienced rapid recovery after receiving anti-CD19 CAR T cell therapy.
  • * Following the treatment, both patients regained full mobility and returned to normal activities within two months, showcasing the potential of anti-CD19 CAR T cells in effectively treating complex neuroimmunological diseases.
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Background: The consideration of patient preference for a certain drug route of administration (RoA) plays an important role in promoting patient adherence in chronic diseases. Natalizumab is an established treatment for relapsing-remitting multiple sclerosis (RRMS) and can be administered as intravenous (IV) infusion or subcutaneous (SC) injection developed to enable a shorter and easier administration IV RoA.

Study Objectives: Primary objective is to compare patients' preference for RoA and satisfaction with SC IV natalizumab at baseline and subsequent visits up to 12 months.

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Purpose: Corneal confocal microscopy is a noninvasive imaging technique to analyze corneal nerve fibers and corneal inflammatory cells (CICs). The amount of CICs is a potential biomarker of disease activity in chronic autoinflammatory diseases. To date, there are no standardized criteria for the morphological characterization of CICs.

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  • Managing multiple sclerosis is challenging due to the varying symptoms and disease progressions in patients, leading to difficulty in individualized treatment selection.
  • Researchers identified three unique blood immune profiles (endophenotypes) in early multiple sclerosis patients using advanced techniques, which correspond to different disease progression patterns—one focusing on inflammation and another on early structural damage.
  • The study suggests that understanding a patient's specific immune profile before starting treatment could help predict disease progression and support more personalized treatment strategies, as certain therapies may be less effective for some endophenotypes.
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Experimental autoimmune neuritis is a common animal model for acute human immune-mediated polyneuropathies. Although already established in 1955, a number of pathophysiological mechanisms remain unknown. In this study, we extensively characterize experimental autoimmune neuritis progression in Lewis rats, including new insights into the integrity of small nerve fibres, neuropathic pain and macrophage activation.

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Introduction: Blood-cerebrospinal fluid (CSF) barrier dysfunction is pivotal for diagnosing immune-mediated neuropathies, especially in spinal nerve root inflammation. Typically, either total CSF protein or the CSF to serum albumin ratio (Q) is measured. Total CSF protein measurements have limitations, notably its fixed reference value regardless of age, in contrast to the age-dependent reference for Q.

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Background And Purpose: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease with humoral and cellular autoimmunity causing demyelination of peripheral nerves, commonly treated with intravenous immunoglobulins (IVIg). The neonatal Fc receptor (FcRn), encoded by the FCGRT gene, prevents the degradation of immunoglobulin G (IgG) by recycling circulating IgG. A variable number of tandem repeat (VNTR) polymorphism in the promoter region of the FCGRT gene is associated with different expression levels of mRNA and protein.

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