AI Article Synopsis
Article Abstract
Background And Aim: It is unclear which patients with Fabry disease (FD) are at risk for progression of white matter lesions (WMLs) and brain infarctions and whether enzyme replacement therapy (ERT) changes this risk. The aim of this study was to determine the effect of ERT and clinical characteristics on progression of WMLs and infarctions on MRI in patients with FD.
Methods: MRIs were assessed for WMLs (Fazekas scale), infarctions and basilar artery diameter (BAD). The effect of clinical characteristics (renal and cardiac involvement, cardiovascular risk factors, cardiac complications, BAD) and ERT on WML and infarction progression was evaluated using mixed models.
Results: One hundred forty-nine patients were included (median age: 39 years, 38% men, 79% classical phenotype). Median follow-up time was 7 years (range: 0-13 years) with a median number of MRIs per patient of 5 (range: 1-14), resulting in a total of 852 scans. Variables independently associated with WML and infarction progression were age, male sex and a classical phenotype. Progression of WMLs and infarctions was not affected by adding ERT to the model, neither for the whole group, nor for early treated patients. Progression was highly variable among patients which could not be explained by other known variables such as hypertension, cholesterol, atrial fibrillation and changes in kidney function, left ventricular mass or BAD.
Conclusion: Progression of WMLs and cerebral infarctions in FD is mainly related to age, sex and phenotype. Additional effects of established cardiovascular risk factors, organ involvement and treatment with ERT are probably small to negligible.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1136/jnnp-2019-322268 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
White matter lesions contribute to motor and non-motor disorders in Parkinson's disease: a critical review.
Geroscience
November 2024
State Key Laboratory of Bioactive Substance and Function of Natural Medicines, Department of Pharmacology, Institute of Materia Medica, Chinese Academy of Medical Sciences & Peking Union Medical College, 1 Xian Nong Tan Street, Beijing, 100050, People's Republic of China.
Parkinson's disease (PD) is a prevalent neurodegenerative disease, characterized by movement disorders and non-motor symptoms like cognitive impairment and depression. Degeneration of dopaminergic neurons in the substantia nigra and Lewy bodies have long been considered as main neuropathological changes. However, recent magnetic resonance imaging (MRI) studies have shown that white matter lesions (WMLs) were present in PD patients.
View Article and Find Full Text PDFMicroglial activation without peripheral immune cell infiltration characterises mouse and human cerebral small vessel disease.
Neuropathol Appl Neurobiol
December 2024
Institute of Physiological Chemistry and Pathobiochemistry and Cells-in-Motion Interfaculty Centre (CiMIC), University of Muenster, Muenster, Germany.
Aims: Cerebral small vessel diseases (SVDs) involve diverse pathologies of the brain's small blood vessels, leading to cognitive deficits. Cerebral magnetic resonance imaging (MRI) reveals white matter hyperintensities (WMHs), lacunes, microbleeds and enlarged perivascular spaces in SVD patients. Although correlations of MRI and histopathology help to understand the pathogenesis of SVD, they do not explain disease progression.
View Article and Find Full Text PDFWhite Matter Lesion Volumes on 3-T MRI in People With MS Who Had Followed a Diet and Lifestyle Program for More Than 10 Years.
Mult Scler Int
November 2024
Faculty of Health and Wellness Sciences, Cape Peninsula University of Technology, Cape Town, South Africa.
Article Synopsis
- Cerebral white matter lesions (WMLs) in people with multiple sclerosis (pwMS) are linked to the loss of myelin-producing cells, and current treatments aim to reduce these lesions and associated disabilities.
- A new genetic testing program identified risk factors for WML formation and suggested personalized lifestyle changes and nutrient optimization as possible preventive measures.
- A 10-year pilot study found that pwMS who followed these lifestyle interventions exhibited significantly smaller WML volumes compared to those who did not, highlighting the potential of dietary and lifestyle changes in managing MS, though further research is necessary.*
Quantifying Remyelination Using χ-Separation in White Matter and Cortical Multiple Sclerosis Lesions.
Neurology
September 2024
From the Translational Imaging in Neurology (ThINk) Basel (J.M., P.-J.L., A.C., E.R., M.O.-P., X.C., C.T., M.B., R.G., M.W., S.A.S., L.K., C.G.), Department of Biomedical Engineering, Faculty of Medicine, and Neurologic Clinic and Policlinic, MS Center and Research Center for Clinical Neuroimmunology and Neuroscience Basel (RC2NB) (J.M., P.-J.L., A.C., E.R., M.O.-P., X.C., C.T., M.B., R.G., M.W., L.K., J.K., C.G.), University Hospital Basel and University of Basel, Switzerland; Department of Health Sciences (A.C.), University of Genova, Italy; Laboratory for Imaging Science and Technology (H.-G.S., J.L.), Department of Electrical and Computer Engineering, Seoul National University, South Korea; Division of Radiological Physics (M.W.), Department of Radiology, University Hospital Basel; Department of Clinical Research (S.A.S.), Clinical Trial Unit, University Hospital Basel, Switzerland; and Department of Radiology (Y.W., T.D.N., P.S.), Weill Medical College of Cornell University, New York, NY.
Background And Objectives: Myelin and iron play essential roles in remyelination processes of multiple sclerosis (MS) lesions. χ-separation, a novel biophysical model applied to multiecho T2*-data and T2-data, estimates the contribution of myelin and iron to the obtained susceptibility signal. We used this method to investigate myelin and iron levels in lesion and nonlesion brain areas in patients with MS and healthy individuals.
View Article and Find Full Text PDFHippocampal atrophy and white matter lesions characteristics can predict evolution to dementia in patients with vascular mild cognitive impairment.
J Neurol Sci
September 2024
Department of Medicine, Surgery and Neuroscience, University of Siena, 53100 Siena, Italy.
Background: Vascular mild cognitive impairment (VMCI) is a transitional condition that may evolve into Vascular Dementia(VaD). Hippocampal volume (HV) is suggested as an early marker for VaD, the role of white matter lesions (WMLs) in neurodegeneration remains debated.
Objectives: Evaluate HV and WMLs as predictive markers of VaD in VMCI patients by assessing: (i)baseline differences in HV and WMLs between converters to VaD and non-converters, (ii) predictive power of HV and WMLs for VaD, (iii) associations between HV, WMLs, and cognitive decline, (iv)the role of WMLs on HV.
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!