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Effectiveness and Safety of IVIG for the Treatment of HMGCR Immune-Mediated Necrotizing Myopathy.
Muscle Nerve
December 2024
Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Introduction/aims: Immune-mediated necrotizing myopathy (IMNM) is an autoimmune myopathy. We aimed to compare clinical outcomes in patients with antibodies against 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) treated on immunotherapy regimens with and without maintenance intravenous immunoglobulin (IVIG). The secondary aim was to assess outcomes in a subset that received IVIG monotherapy.
View Article and Find Full Text PDFCo-Existent Central and Peripheral Demyelination: Related or Coincidental?
Neurol Int
December 2024
Department of Neurology, University of Texas Medical Branch, Galveston, TX 77555, USA.
Background: Hereditary Sensory Motor Neuropathy (HSMN) 1A and Multiple Sclerosis (MS) are distinct demyelinating disorders affecting the peripheral and central nervous systems, respectively. We present a case of simultaneous occurrence of both conditions, exploring the clinical presentation, diagnostic workup, and potential interplay between these diseases. Case presentation and clinical approach: A 49-year-old male with a history of optic neuritis presented with progressive numbness, weakness, and sensory loss in all extremities over four years.
View Article and Find Full Text PDF[Treatment Strategies for Chronic Inflammatory Demyelinating Polyneuropathy].
Brain Nerve
January 2025
Department of Neurology, Dokkyo Medical University.
Three main treatments are available for management of chronic inflammatory demyelinating polyneuropathy (CIDP). Both induction and maintenance therapies should be considered for treatment of CIDP. Plasma exchange and intravenous immunoglobulin therapy are effective as induction treatments for CIDP, and corticosteroid administration, such as daily oral or pulse therapies, may be effective for both induction and maintenance.
View Article and Find Full Text PDF[Updated Diagnosis and Treatment for Chronic Inflammatory Demyelinating Polyradiculoneuropathy Based on the EAN/PNS Guideline 2021].
Brain Nerve
January 2025
Department of Neurology, Graduate School of Medicine, Chiba University.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) includes a number of clinical subtypes. The major phenotype is "typical CIDP," which is characterized by symmetric and "proximal and distal" muscle weakness. Due to historical changes in the concept of CIDP, multifocal motor neuropathy, anti-myelin-associated glycoprotein (anti-MAG) neuropathy, and autoimmune nodopathy were excluded.
View Article and Find Full Text PDFVectorized Human Antibody-Mediated Anti-Eosinophil Gene Therapy.
Hum Gene Ther
December 2024
Department of Genetic Medicine, Weill Cornell Medical College, New York, New York, USA.
Chronic hypereosinophilia, defined as persistent elevated blood levels of eosinophils ≥1,500/μL, is associated with tissue infiltration of eosinophils and consequent organ damage by eosinophil release of toxic mediators. The current therapies for chronic hypereosinophilia have limited success, require repetitive administration, and are associated with a variety of adverse effects. As a novel approach to treat chronic hypereosinophilia, we hypothesized that adeno-associated virus (AAV)-mediated delivery of an anti-human eosinophil antibody would provide one-time therapy that would mediate persistent suppression of blood eosinophil levels.
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