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London, United Kingdom 2013 7 Small vessel stroke and white matter disease Cerebral microbleeds in Fabry disease I. Romani1, P. Nencini2, W. Borsini3, G.P. Giordano4, D. Inzitari5 on behalf of the Fabry Disease Centre of Careggi Hospital, Florence, Italy University of Florence, Florence, ITALY1, University of Florence, Florence, ITALY2, University of Florence, Florence, ITALY3, University of Florence, Florence, ITALY4, University of Florence, Florence, ITALY5 Background: Cerebral microbleeds (CMBs) are small perivascular deposits of hemosiderin rec-ognised by MRI techniques such as T2-weighted gradient-recalled echo (T2*-weighted MRI), and they are mainly related to small vessel diseases. There are few data about the occurrence of CMBs in Fabry disease. We aimed to determine the CMBs frequency in a series of patients referred to the Fabry Disease Centre of Careggi Hospital, Florence, Italy. Methods: In our referral centre, Fabry patients undergo a systematic follow-up, including neurological, cardiological, nephrological eval-uations, blood, and urine analysis every 6 months, and periodic instrumental investigations. From March 2011 to August 2012, a consecutive series of Fabry patients scheduled for the neuroradio-logical follow-up performed a standardized brain MRI protocol, including T2*-weighted images for CMBs detection. Clinical and imaging data were prospectively collected in a local database. Results: Twenty-nine patients (females 58.6%, mean age +/- SD: 46.9 +/- 13.7 years) performed T2*-weighted MRI. All patients had mutations of GLA gene of definite pathogenic significance (17 different genotypes). Ten (34.5%) patients had previous ischemic strokes (females 50%, mean age +/- SD 54.1 +/- 9.2 years). The TOAST pathogenesis was small vessel disease in all patients, ex-cept one (cardioembolism). CMBs were detected in 3 patients with 3 different genotypes (females 33.4%, mean age ± SD 60.1 ± 4.6 years) who suffered from ischemic strokes due to small vessel disease; no CMBs were detected in patients without strokes (p=0.01). CMBs were multiple and lo-cated in subcortical regions in all patients. Conclusions: In this series of Fabry patients, CMBs seem associated with ischemic strokes due to small vessel disease. Further studies are necessary to con-firm E-Poster Session Red Cerebrovasc Dis 2013; 35 (suppl 3)1-854 241 our findings. 6 Small vessel stroke and white matter disease tPA-activity predicts progression of periventricular white matter lesions in first-ever lacunar stroke patients E.C. van Overbeek1, J.E.A. Staals2, I.L.H. Knottnerus3, R.J. van Oostenbrugge4 Maastricht university medical centre, Maastricht, THE NETHERLANDS1, Maastricht uni-versity medical centre, Maastricht, THE NETHERLANDS2, Maastricht university medical centre, Maastricht, THE NETHERLANDS3, Maastricht university medical centre, Maastricht, THE NETH-ERLANDS4 Background – Tissue plasminogen activator (tPA) and plasminogen activator inhibitor type 1 anti-gen (PAI-1) are components of the fibrinolytic system and are seen as hemostasis-related markers of endothelial function. They are extensively present in small blood vessels of the cerebral white mat-ter. Earlier we showed that high levels of tPA-activity and low levels of PAI-1 were associated with extensive white matter lesions (WML) in first-ever lacunar stroke patients. The aim of this study was to determine whether tPA-activity and PAI-1 levels are associated with progression of WML. Methods – In 132 first-ever lacunar stroke patients in whom baseline brain MRI (FLAIR, T2) and levels of tPA-activity and PAI-1 were available, we obtained a follow-up MRI after 2 years. We as-sessed progression of periventricular WML (pWML) and deep WML (dWML) using the visual Rot-terdam WML change scale. We determined the relationship between levels of tPA-activity and PAI-1 and WML progression on MRI after 2 years. Results – 34 (25.8%) patients had pWML progression. tPA-activity was higher in patients with pro-gression compared to those without (median 0.93 IU/mL (0.45 – 1.47) vs. 0.57 IU/mL (0.34-0.92), p < 0.01). In logistic regression analyses with correction for age and sex, tPA was associated with pWML progression (OR 2.35, 95% CI 1.06 – 5.22). Although tPA-activity was increased in patients with dWML progression (0.70 vs. 0.60 IU/mL), and PAI-1 levels were lower in both patients with progression of pWML (median 23.1 vs. 37.4 ng/mL) and dWML (27.1 vs. 35.4 ng/mL), these results were not significant. Conclusion – We found higher baseline tPA-activity to be associated with progression of pWML af-ter 2-year follow-up. Our results suggest a causal relationship between tPA-activity and WML. This may be due to the direct involvement of tPA in mediating tissue damage within the cerebral white matter and marks endothelial dysfunction as underlying pathogenetic mechanism of small vessel disease.


Karger_ESC London_2013
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