Publications by authors named "Motte J"

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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  • 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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Scanning Thermal Microscopy (SThM) has become an important measurement technique for characterizing the thermal properties of materials at the nanometer scale. This technique requires a SThM probe that combines an Atomic Force Microscopy (AFM) probe and a very sensitive resistive thermometer; the thermometer being located at the apex of the probe tip allows for the mapping of temperature or thermal properties of nanostructured materials with very high spatial resolution. The high interest of the SThM technique in the field of thermal nanoscience currently suffers from a low temperature sensitivity despite its high spatial resolution.

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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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  • The study aimed to assess the effectiveness of magnetic resonance neurography (MRN) for tracking changes in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) over time.
  • Researchers examined 12 CIDP patients in 2016 and again in 2022, comparing nerve metrics with healthy individuals and correlating findings with clinical tests.
  • Key findings showed that nerve cross-sectional area (CSA) decreased over 6 years in CIDP patients, with initial CSA levels possibly indicating the severity of the disease's course and treatment response.
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Background And Purpose: The role of high-resolution nerve ultrasound (HRUS) and corneal confocal microscopy (CCM) in the early detection of taxane-induced polyneuropathy (TIPN) is unclear. The present prospective longitudinal controlled observational pilot study estimates the role of HRUS and CCM in the early diagnosis of TIPN in breast cancer patients.

Methods: Fifteen breast cancer patients receiving paclitaxel and 15 healthy age matched controls were included.

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Microbeam radiation therapy (MRT) is a radiotherapy technique combining spatial fractionation of the dose distribution on a micrometric scale, X-rays in the 50-500 keV range and dose rates up to 16 × 10 Gy s. Nowadays, in vivo dosimetry remains a challenge due to the ultra-high radiation fluxes involved and the need for high-spatial-resolution detectors. The aim here was to develop a striped diamond portal detector enabling online microbeam monitoring during synchrotron MRT treatments.

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Background: Depression has a major impact on the disease burden of multiple sclerosis (MS). Analyses of overlapping MS and depression risk factors [smoking, vitamin D (25-OH-VD) and Epstein-Barr virus (EBV) infection] and sex, age, disease characteristics and neuroimaging features associated with depressive symptoms in early MS are scarce.

Objectives: To assess an association of MS risk factors with depressive symptoms within the German NationMS cohort.

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Introduction: Serum neurofilament light chain (sNfL) is a marker for axonal degeneration. Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) often report a fluctuation of symptoms throughout one treatment cycle with intravenous immunoglobulins (IVIG). The aim of this study was to determine whether sNfL is suitable to quantify patient-reported symptom fluctuations.

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