Background: Neuromyelitis Optica (NMO) is an inflammatory demyelinating disease that mainly affects optic nerves and spinal cord. Besides, loss of motor and cognitive function has been reported as important symptoms of disease.
Objective: Here we investigated the mitochondrial dysfunction and metabolic alterations in NMO patients and evaluate their correlation with disease progress, disability and cognitive impairment.
Methods: The individuals (12 controls and 12 NMO) were assessed for disease severity by expanded disease status scale (EDSS), cognitive function via symbol digit modalities test (SDMT) and fine motor disability by 9-hole peg test (9-HPT). We have measured Sirtuin 1 (SIRT1), SIRT3, mitochondrial complex I, complex IV, aconitase and α-ketoglutarate dehydrogenase (α-KGD) activity in peripheral blood mononuclear cells (PBMCs). Furthermore, SIRT1, pyruvate, lactate and cytochrome c (Cyt c) were determined in plasma.
Results: Our results exhibited increased 9-HPT time in NMO patients. 9-HPT results correlated with EDSS; and SDMT negatively correlated with disease duration and number of attacks in patients. Investigation of PBMCs of NMO patients exhibited a decrease of mitochondrial complex I and IV activity that was significant for complex IV. Besides, complex I activity was negatively correlated with 9-HPT time in NMO group. In the plasma samples, a correlation between pyruvate to lactate ratio and EDSS in NMO patients was found and a negative correlation between Cyt c concentration and SDMT was detected.
Conclusion: Our data support the hypothesis that mitochondrial dysfunction occurred in the CNS and the peripheral blood may contribute to disease progress, disability level and the cognitive impairment in NMO patients.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7098571 | PMC |
| http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0230691 | PLOS |
Publication Analysis
Top Keywords
Similar Publications
The current troubled state of the global pathology workforce: a concise review.
Diagn Pathol
December 2024
Women's Health Research Group, Leeds Institute of Medical Research, St James's University Hospital, University of Leeds, Wellcome Trust Brenner Building, Beckett Street, Leeds, LS9 7TF, UK.
The histopathology workforce is a cornerstone of cancer diagnostics and is essential to the delivery of cancer services and patient care. The workforce has been subject to significant pressures over recent years, and this review considers them in the UK and internationally. These pressures include declining pathologist numbers, the increasing age of the workforce, and greater workload volume and complexity.
View Article and Find Full Text PDFThe epidemiology and clinical presentation of seropositive neuromyelitis optica spectrum disorder in a US population.
Ann Clin Transl Neurol
December 2024
Departments of Neurology and Ophthalmology, Programs in Neuroscience and Immunology, Anschutz Medical Campus, University of Colorado School of Medicine, Aurora, Colorado, USA.
Objective: To define the epidemiology and clinical presentation of seropositive neuromyelitis optica spectrum disorder (NMOSD) in a large US health system.
Methods: We completed a retrospective observational study of adult patients in the University of Colorado Health System from 1 January 2011 to 31 December 2020, using Health Data Compass (HDC), a data warehouse that combines electronic health information with claims and public health data in Colorado. We screened HDC for patients with either (1) an abnormal aquaporin-4 IgG test or (2) any G36 ICD-10 code.
De-escalating and discontinuing disease-modifying therapies in multiple sclerosis.
Brain
December 2024
Department of Neurology, Multiple sclerosis center, Pitié-Salpêtrière Hospital, AP-HP, 47 bd de l'Hôpital, 75013 Paris, France.
The development of disease-modifying therapies (DMTs) for the treatment of multiple sclerosis (MS) has been highly successful in recent decades. It is now widely accepted that early initiation of DMTs after disease onset is associated with a better long-term prognosis. However, the question of when and how to de-escalate or discontinue DMTs remains open and critical.
View Article and Find Full Text PDFMOG-IgG is rare in AQP4-IgG seronegative NMO phenotype in Brazil.
Mult Scler Relat Disord
December 2024
CIEM MS Research Center, Federal University of Minas Gerais Medical School, Belo Horizonte, MG, Brazil.
Background: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease most frequently characterized by a neuromyelitis optica (NMO) phenotype, comprising both simultaneous or sequential optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM). Symptoms of brainstem, diencephalic and cerebral involvement may also occur. While most NMOSD patients test positive for serum aquaporin-4 (AQP4) antibodies, some seronegative patients test positive for oligodendrocyte glycoprotein-IgG (MOG-IgG).
View Article and Find Full Text PDFQuantitative Contribution of Clinical Attacks to Residual Disability in Patients With AQP4-Antibody Neuromyelitis Optica Spectrum Disorder.
Neurology
January 2025
From the Nuffield Department of Clinical Neurosciences (B.C., A.F., R.G., M.I.S.L., J.P.), Oxford University Hospitals, United Kingdom; Department of Neurology (B.C.), Tongji Hospital of Tongji Medical College, Huazhong University of Science of Technology, Wuhan, China; University Hospitals Sussex National Health Service Foundation Trust (S.A.C.), Brighton; Centre for Preventive Neurology (R.D.), Wolfson Institute of Population Health, Queen Mary University of London; Queen Square Multiple Sclerosis Centre (Y.H.), UCL Institute of Neurology, Faculty of Brain Sciences, University College London; Department of Paediatric Neurology (Y.H.), Great Ormond Street Hospital for Children, London; Department of Neurology (C. Halfpenny), University Hospital Southampton NHS Foundation Trust; Department of Neurology (C. Hemingway), Great Ormond Street Hospital for Children, London and Institute of Neurology; Department of Neurology (J.C.H.), University of Plymouth Faculty of Health and University Hospitals; Department of Ophthalmology (E.O.S.), King's College Hospital NHS Foundation Trust, London; Department of Neurology (W.R.), St George's University Hospitals NHS Foundation Trust, London; Department of Neurology (R.J.M.), Gloucestershire Hospitals National Health Service Foundation Trust; Department of Neurology (V.W.), King's College Hospital NHS Foundation Trust, London; Department of Neurology (V.W.), Guy's and St Thomas' National Health Service Foundation Trust, London; Department of Paediatric Neurology (S.R.), John Radcliffe Hospital, Oxford; and Neurology Department (R.G.), Wexham Park Hospital, Frimley Foundation Health Trust, Slough, United Kingdom.
Article Synopsis
- The study investigates how clinical attacks contribute to ongoing disability in patients with aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD).
- A total of 165 patients were analyzed using disability scores recorded after at least six months post-attack, with findings showing a significant increase in disability scores correlating with the number and type of relapses.
- Results indicated that specific relapse types, particularly the combination of transverse myelitis and optic neuritis, had the most substantial impact on increasing residual disability.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!