Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a distinct pathologic entity of unknown etiology. Here, we describe the clinical and radiologic presentation of myelin oligodendrocyte glycoprotein associated disease (MOG-AD) with features mimicking CLIPPERS. Three patients met the 2017 CLIPPERS diagnostic criteria, while one patient had a single lesion in the pons that mimicked CLIPPERS lesions. All had an excellent response to steroids, but the three who met the CLIPPERS criteria had a relapsing course. When CLIPPERS is observed, it is crucial to test for mimickers. The ever-expanding spectrum of MOG-AD calls for further research into the immunopathogenesis of its several phenotypes.
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http://dx.doi.org/10.1016/j.msard.2021.102874 | DOI Listing |
Neurol Neuroimmunol Neuroinflamm
March 2025
Hospices Civils de Lyon, Service de Neurologie, Sclérose en Plaques, Pathologies de la Myéline et Neuro-Inflammation-Hôpital Neurologique Pierre Wertheimer, Bron Cedex.
Objectives: To characterize the serum cytokine profile in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) at onset and during follow-up and assess their utility for predicting relapses and disability.
Methods: This retrospective multicentric cohort study included patients aged 16 years and older meeting MOGAD 2023 criteria, with serum samples collected at baseline (≤3 months from disease onset) and follow-up (≥6 months from the baseline), and age-matched and time to sampling-matched patients with multiple sclerosis (MS). Eleven cytokines were assessed using the ELLA system.
Neurochem Res
January 2025
Department of Neurology, Affiliated Hospital of Zunyi Medical University, Zunyi, China.
Alzheimer's disease (AD) is a central nervous system degenerative disease with a stealthy onset and a progressive course characterized by memory loss, cognitive dysfunction, and abnormal psychological and behavioral symptoms. However, the pathogenesis of AD remains elusive. An increasing number of studies have shown that oligodendrocyte progenitor cells (OPCs) and oligodendroglial lineage cells (OLGs), especially OPCs and mature oligodendrocytes (OLGs), which are derived from OPCs, play important roles in the pathogenesis of AD.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
University of Florida, Gainesville, FL, USA.
Background: Severe systemic infections can trigger cognitive decline, but the underlying mechanisms and their impact on the manifestation and progression of Alzheimer's disease and other neurodegenerative diseases are poorly understood. The current COVID-19 pandemic has brought a surge of severe viral illness and highlights the importance of understanding the impact of acute infections on cognition and the manifestation of neurodegenerative disease in survivors. A wealth of observational and clinical data suggests major short- and long-term effects of severe infections on cognition, but detailed and systematic analyses of neuropathological changes after acute infections are scarce.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
Institute for Memory Impairments and Neurological Disorders (MIND), Irvine, CA, USA.
Background: Late-onset Alzheimer's disease (LOAD) represents the majority of human AD cases, yet the availability of animal models that accurately reflect LOAD progression and pathology is limited. Traditional transgenic mouse models including 3xTg-AD and 5xFAD rely on supraphysiological overexpression of familial AD risk genes, failing to adequately replicate the disease progression observed in LOAD. Here, we present the first characterization of MODEL-AD1 (MAD1), a platform mouse developed by the Model Organism Development and Evaluation for Late-onset Alzheimer's Disease (MODEL-AD) Consortium.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
Brain and Mind Research Institute, new york, NY, USA.
Background: DAP12 (DNAX-activation protein 12 or TYROBP) functions as a pivotal adaptor, facilitating signal transmission from surface immune receptors on microglia, including TREM2-a known risk gene for Alzheimer's disease (AD). Previous studies showed that DAP12-deficient mice exhibit resistance to tau toxicity in a tauopathy model, manifesting reduced brain inflammation and improved cognition, despite increased tau pathology. However, the precise mechanism underlying how DAP12 deficiency enhances resilience to tauopathy remains elusive.
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