Magnetic resonance spectroscopy as marker for neurodegeneration in X-linked adrenoleukodystrophy
| Autor: | Matthan W.A. Caan, Petra J.W. Pouwels, Marc Engelen, Irene C. Huffnagel, Wouter J.C. van Ballegoij, Anouk Schrantee, Stephanie I.W. van de Stadt |
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| Přispěvatelé: | Graduate School, Amsterdam Neuroscience - Cellular & Molecular Mechanisms, Amsterdam Gastroenterology Endocrinology Metabolism, Radiology and Nuclear Medicine, APH - Personalized Medicine, Amsterdam Neuroscience - Compulsivity, Impulsivity & Attention, Biomedical Engineering and Physics, ACS - Atherosclerosis & ischemic syndromes, ACS - Microcirculation, Amsterdam Neuroscience - Brain Imaging, Neurology, Paediatric Neurology, APH - Mental Health, APH - Methodology, APH - Aging & Later Life, ACS - Diabetes & metabolism, Radiology and nuclear medicine, Pediatric surgery |
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Adult
Male Pathology medicine.medical_specialty Magnetic Resonance Spectroscopy Imaging biomarker Cognitive Neuroscience Myelopathy Computer applications to medicine. Medical informatics R858-859.7 Creatine Choline White matter chemistry.chemical_compound medicine Humans Radiology Nuclear Medicine and imaging Prospective Studies X-linked adrenoleukodystrophy Neurodegeneration Prospective cohort study Adrenoleukodystrophy RC346-429 Aspartic Acid business.industry T-cell receptor Magnetic resonance spectroscopic imaging Brain Regular Article medicine.disease Magnetic Resonance Imaging medicine.anatomical_structure Neurology chemistry Female Neurology (clinical) Neurology. Diseases of the nervous system business Biomarkers MRI |
| Zdroj: | van de Stadt, S I W, Schrantee, A, Huffnagel, I C, van Ballegoij, W J C, Caan, M W A, Pouwels, P J W & Engelen, M 2021, ' Magnetic resonance spectroscopy as marker for neurodegeneration in X-linked adrenoleukodystrophy ', NeuroImage: Clinical, vol. 32, 102793 . https://doi.org/10.1016/j.nicl.2021.102793 NeuroImage: Clinical, Vol 32, Iss, Pp 102793-(2021) NeuroImage : Clinical NeuroImage: Clinical, 32:102793. Elsevier BV |
| ISSN: | 2213-1582 |
| DOI: | 10.1016/j.nicl.2021.102793 |
| Popis: | Highlights • Spectroscopy provides valuable biomarker information for X-linked adrenoleukodystrophy. • Reduced metabolite levels indicate axonal damage in the brain of patients with ALD. • Two methods for spectroscopy, acquired at different field strengths, provide similar results. X-linked adrenoleukodsytrophy (ALD) is a genetic neuro-metabolic disorder, causing a slowly progressive myelopathy in adult male and female patients. New disease modifying therapies for myelopathy are under development. This calls for new (imaging) markers able to measure disease severity and progression in clinical trials. In this prospective cohort study, we measured cerebral metabolite levels with Magnetic Resonance Spectroscopy (MRS), and evaluated their potential as biomarkers for disease severity and neurodegeneration in ALD. We used a comprehensive protocol of 3T Magnetic Resonance Spectroscopic Imaging (MRSI) and 7T Single Voxel Spectroscopy (SVS) in a large cohort of adult ALD males without cerebral demyelination. One hundred seven baseline scans – 59 obtained in ALD patients (42 3T MRSI and 17 7T SVS) and 48 obtained in healthy male controls (32 3T MRSI and 16 7T SVS) – and 82 one and two-year follow-up scans (66 3T MRSI and 16 7T SVS) of ALD patients were included. Both protocols showed significantly lower concentration ratios of N-acetylaspartate/creatine (tNAA/tCr) and Glx (glutamine + glutamate)/tCr in the grey and white matter of patients, compared to controls. A novel finding is the higher level of inositol (Ins)/tCr and choline containing compounds (tCho)/tCr in ALD patients without cerebral demyelination. Furthermore, tNAA/tCr correlated strongly with clinical measures of severity of myelopathy. There was no detectable change in metabolite ratios after one-year or two-year follow-up. Our results imply that cerebral metabolite levels – and more specifically the tNAA/tCr ratio – measured with MRS, have potential value as (imaging) biomarkers in ALD. |
| Databáze: | OpenAIRE |
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