{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T02:51:14Z","timestamp":1786935074244,"version":"build-2736575974"},"reference-count":62,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2020,11,1]],"date-time":"2020-11-01T00:00:00Z","timestamp":1604188800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Department of Health\u2019s NIHR Biomedical Research Centres"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Cerebral white matter pathology is a common CNS manifestation of Fabry disease, visualized as white matter hyperintensities on MRI in 42\u201381% of patients. Diffusion tensor imaging (DTI) MRI is a sensitive technique to quantify microstructural damage within the white matter with potential value as a disease biomarker. We evaluated the pattern of DTI abnormalities in Fabry disease, and their correlations with cognitive impairment, mood, anxiety, disease severity and plasma lyso-Gb3 levels in 31 patients with genetically proven Fabry disease and 19 age-matched healthy control subjects. We obtained average values of fractional anisotropy and mean diffusivity within the white matter and performed voxelwise analysis with tract-based spatial statistics. Using a standardized neuropsychological test battery, we assessed processing speed, executive function, anxiety, depression and disease severity. The mean age (% male) was 44.1 (45%) for patients with Fabry disease and 37.4 (53%) for the healthy control group. In patients with Fabry disease, compared to healthy controls the mean average white matter fractional anisotropy was lower in [0.423 (standard deviation, SD 0.023) versus 0.446 (SD 0.016), P\u2009=\u20090.002] while mean average white matter mean diffusivity was higher (749\u2009\u00d7\u200910\u22126 mm2\/s (SD 32\u2009\u00d7\u200910\u22126) versus 720\u2009\u00d7\u200910\u22126 mm2\/s (SD 21\u2009\u00d7\u200910\u22126), P\u2009=\u20090.004]. Voxelwise statistics showed that the diffusion abnormalities for both fractional anisotropy and mean diffusivity were anatomically widespread. A lesion probability map showed that white matter hyperintensities also had a wide anatomical distribution with a predilection for the posterior centrum semiovale. However, diffusion abnormalities in Fabry disease were not restricted to lesional tissue; compared to healthy controls, the normal appearing white matter in patients with Fabry disease had reduced fractional anisotropy [0.422 (SD 0.022) versus 0.443 (SD 0.017) P\u2009=\u20090.003] and increased mean diffusivity [747\u2009\u00d7\u200910\u22126 mm2\/s (SD 26\u2009\u00d7\u200910\u22126) versus 723\u2009\u00d7\u200910\u22126 mm2\/s (SD 22\u2009\u00d7\u200910\u22126), P\u2009=\u20090.008]. Within patients, average white matter fractional anisotropy and white matter lesion volume showed statistically significant correlations with Digit Symbol Coding Test score (r\u2009=\u20090.558, P\u2009=\u20090.001; and r = \u22120.633, P\u2009\u2264\u20090.001, respectively). Average white matter fractional anisotropy correlated with the overall Mainz Severity Score Index (r = \u22120.661, P\u2009\u2264\u20090.001), while average white matter mean diffusivity showed a strong correlation with plasma lyso-Gb3 levels (r\u2009=\u20090.559, P\u2009=\u20090.001). Our findings using DTI confirm widespread areas of microstructural white matter disruption in Fabry disease, extending beyond white matter hyperintensities seen on conventional MRI. Moreover, diffusion measures show strong correlations with cognition (processing speed), clinical disease severity and a putative plasma biomarker of disease activity, making them promising quantitative biomarkers for monitoring Fabry disease severity and progression.<\/jats:p>","DOI":"10.1093\/brain\/awaa282","type":"journal-article","created":{"date-parts":[[2020,8,12]],"date-time":"2020-08-12T19:29:14Z","timestamp":1597260554000},"page":"3331-3342","source":"Crossref","is-referenced-by-count":31,"title":["White matter integrity correlates with cognition and disease severity in Fabry disease"],"prefix":"10.1093","volume":"143","author":[{"given":"Leonardo","family":"Ulivi","sequence":"first","affiliation":[{"name":"Stroke Research Centre, Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London WC1B 5EH, UK"},{"name":"Department of Experimental and Clinical Medicine, Neurological Clinic, Pisa University, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Baris","family":"Kanber","sequence":"additional","affiliation":[{"name":"Department of Neuroinflammation, UCL Queen Square Institute of Neurology, London WC1B 5EH, UK"},{"name":"Centre for Medical Image Computing (CMIC), Department of Medical Physics and Biomedical Engineering, University College London, London, WC1V 6LJ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ferran","family":"Prados","sequence":"additional","affiliation":[{"name":"Department of Neuroinflammation, UCL Queen Square Institute of Neurology, London WC1B 5EH, UK"},{"name":"Centre for Medical Image Computing (CMIC), Department of Medical Physics and Biomedical Engineering, University College London, London, WC1V 6LJ, UK"},{"name":"e-Health Centre, Universitat Oberta de Catalunya, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Indran","family":"Davagnanam","sequence":"additional","affiliation":[{"name":"Stroke Research Centre, Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London WC1B 5EH, UK"},{"name":"Academic Department of Neuroradiology, Department of Brain Repair and Rehabilitation,\u00a0UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aine","family":"Merwick","sequence":"additional","affiliation":[{"name":"Stroke Research Centre, Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London WC1B 5EH, UK"},{"name":"Cork University Hospital, University College Cork, Wilton, Cork, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edgar","family":"Chan","sequence":"additional","affiliation":[{"name":"Department of Neuropsychology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fay","family":"Williams","sequence":"additional","affiliation":[{"name":"Department of Neuropsychology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK"},{"name":"Charles Dent Metabolic Unit, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derralynn","family":"Hughes","sequence":"additional","affiliation":[{"name":"Lysosomal Storage Disorders Unit, Royal Free Hospital, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elaine","family":"Murphy","sequence":"additional","affiliation":[{"name":"Charles Dent Metabolic Unit, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R H","family":"Lachmann","sequence":"additional","affiliation":[{"name":"Charles Dent Metabolic Unit, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia A M Gandini","family":"Wheeler-Kingshott","sequence":"additional","affiliation":[{"name":"Department of Neuroinflammation, UCL Queen Square Institute of Neurology, London WC1B 5EH, UK"},{"name":"Brain MRI 3T Research Centre, IRCCS Mondino Foundation, Pavia, Italy"},{"name":"Department of Brain and Behavioural Sciences, University of Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Cipolotti","sequence":"additional","affiliation":[{"name":"Department of Neuropsychology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David J","family":"Werring","sequence":"additional","affiliation":[{"name":"Stroke Research Centre, Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London WC1B 5EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,11,3]]},"reference":[{"key":"2021012813551937400_awaa282-B1","doi-asserted-by":"crossref","first-page":"964","DOI":"10.1136\/jnnp.2006.112987","article-title":"Voxel based analyses of diffusion tensor imaging in Fabry disease","volume":"78","author":"Albrecht","year":"2007","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"2021012813551937400_awaa282-B2","doi-asserted-by":"crossref","first-page":"2148","DOI":"10.1212\/01.WNL.0000166000.24321.4F","article-title":"Effect of genetic modifiers on cerebral lesions in Fabry 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