Autoimmune encephalitis is characterized by subacute onset of the altered mental status that can rapidly progress to autonomic instability and refractory seizures requiring intensive care. It is mediated by autoantibodies that bind to synaptic surface proteins and alter their function. In contrast to many autoimmune CNS diseases, there is often little detectable inflammatory damage to the brain making it difficult to diagnose. Early engagement of a multidisciplinary team is essential to obtaining a complete diagnostic workup and instituting definitive therapy as early as possible to optimize outcomes. Diagnosis, treatment, and monitoring for this devastating condition continue to evolve. Pathogenesis, diagnosis and both current and emerging therapies are reviewed.
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http://dx.doi.org/10.1016/j.ccc.2021.11.007 | DOI Listing |
Ann Thorac Surg Short Rep
December 2024
Division of Cardiothoracic Surgery, University of California, San Diego, California.
Thymomas have been associated with the generation of paraneoplastic autoantibodies to neurogenic epitopes, collapsin-response-mediator protein-5 receptor (CRMP-5) and alpha-amino-3-hydroxyl-5methyl-4isoxazolepropionic acid receptor (AMPAR), in patients with acute viral infection. We report a patient with thymoma and myasthenia gravis, with SARS-CoV-2 infection, who became comatose secondary to autoimmune encephalitis. Plasmapheresis, high-dose steroids, pyridostigmine, eculizumab, and rituximab did not restore neurologic function.
View Article and Find Full Text PDFEur J Neurol
January 2025
Experimental and Clinical Research Center, Max Delbrück Center for Molecular Medicine Berlin and Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Background: Hyperreflective retinal foci (HRF) visualized by optical coherence tomography (OCT) potentially represent clusters of microglia. We compared HRF frequencies and their association with retinal neurodegeneration between people with clinically isolated syndrome (pwCIS), multiple sclerosis (pwMS), aquaporin 4-IgG positive neuromyelitis optica spectrum disorder (pwNMOSD), and healthy controls (HC)-as well as between eyes with (ONeyes) and without a history of optic neuritis (ONeyes).
Methods: Cross-sectional data of pwCIS, pwMS, and pwNMOSD with previous ON and HC were acquired at Charité-Universitätsmedizin Berlin.
Sci Rep
January 2025
Department of Clinical Pathology, Faculty of Medicine, Ain Shams University, Abassia, Cairo, Egypt.
Some patients with neuromyelitis optica spectrum disorder (NMOSD)-like symptoms test negative for anti-aquaporin-4 (anti-AQP4) antibodies. Among them, a subset has antibodies targeting myelin oligodendrocyte glycoprotein (MOG), a condition now termed MOG antibody-associated disease (MOGAD). MOGAD shares features with NMOSD, like optic neuritis and myelitis, but differs in pathophysiology, clinical presentation, imaging findings, and biomarkers.
View Article and Find Full Text PDFBMJ Open
January 2025
Department of Rheumatology and Physiotherapy, Third Faculty of Medicine, Charles University and Thomayer University Hospital, Prague, Czech Republic
Introduction: Upper limb (UL) impairment is common in people with multiple sclerosis (pwMS), and functional recovery of the UL is a key rehabilitation goal. Technology-based approaches, like virtual reality (VR), are increasingly promising. While most VR environments are task-oriented, our clinical approach integrates neuroproprioceptive 'facilitation and inhibition' (NFI) principles.
View Article and Find Full Text PDFZhonghua Nei Ke Za Zhi
January 2025
Department of Thoracic Surgery, Xuanwu Hospital, Capital Medical University, Beijing100053, China.
To explore the risk factors of delayed extubation after expanded thymectomy in patients with myasthenia gravis. Patients with myasthenia gravis who underwent expanded thymectomy from May 2021 to January 2024 and were admitted to Intensive Care Unit (ICU) after surgery were retrospectively analyzed. Patients were divided in to the delayed extubation and successful extubation according to the length of mechanical ventilation whether exceeding than 48 hours.
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