Sicle-cell disease and silent cerebral infarcts evaluated with magnetic resonance imaging

Transcription

1 Sicle-cell disease and silent cerebral infarcts evaluated with magnetic resonance imaging Poster No.: C-0243 Congress: ECR 2015 Type: Educational Exhibit Authors: E. Solomou, A. Rigopoulou, P. Kraniotis, T. Petsas; Patras/GR Keywords: Neuroradiology brain, MR, Screening, Hematologic diseases DOI: /ecr2015/C-0243 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 7

2 Learning objectives To correlate the extent of silent cerebral infarcts (SCIs) on magnetic resonance imaging (MRI) with the clinical severity of sickle cell disease (SCD). Background Sickle cell disease (SCD) is a genetic hemoglobinopathy. Cerebral infarction, either overt or silent, is a serious complication in SCD, developing in % of patients, with high morbidity and mortality, most often between 9 to 15 years of age. Cerebrovascular disease is probably related either to intimal hyperplasia or to thrombosis. Vaso-occlusion results in ischemia of the central nervous system. Small vessels in the arterial border zones are presumed to be the first to be affected. Stroke is caused by damage to either large or small cerebral vessels. SCIs are correlated with increased incidence of new stroke or new SCIs which could be even more extensive. Findings and procedure details 32 SCD patients (14 male and 18 female) with a mean age of 42.4 years were submitted to brain MRI. None of the patients had any neurological symptoms or neurologic deficits. The extent of SCIs was classified from 0-2 according to the literature. Lacunar infarcts were defined as focal (less than 1 cm) high intensity lesions on the T2- weighted or FLAIR images. Leukoencephalopathy was defined as multiple high intensity lesions over 1 cm on the T2-weighted or FLAIR images. Score 0 was attributed to patients with normal MRI. Score 1 was attributed to unifocal lesions <1 cm in diameter or mild extent leukoencephalopathy with lesions measuring <3 cm. Page 2 of 7

3 Score 2 was attributed to more severe/diffuse leukoencephalopathy with lesions >3 cm. Additional to their SCI score, patients were also evaluated for evidence of brain atrophy, with binary criteria either existing or non-existing. Atrophy was defined as lesser volume of brain tissue than the one expected in a healthy person of similar age. The extent of SCIs is not age related and may be quite severe in young patients and it is not correlated with the severity of clinical disease. Images for this section: Fig. 1: Coronal Fluid attenuated inversion recovery images. A 44 year old patient with multifocal lesions, scored as grade 2 Page 3 of 7

4 Fig. 2: Axial T2-WI. A 20-year old sickle cell disease patient with cerebral atrophy Page 4 of 7

5 Fig. 3: Coronal T2-WI. Cerebral atrophy in a young sickle cell disease patient Page 5 of 7

6 Conclusion SCD is associated with silent cerebral infarcts (SCIs) detected on brain MRI in the absence of clinical symptoms. SCIs affects mostly the borderline zones and this may suggest hemodynamic reasons implicated in its pathogenesis. MRI is proved to be highly sensitive in cases of identification of SCIs in asymptomatic patients. Personal information Associate Professor Magnetic Resonance Imaging Department University Hospital of Patras References Vichinsky EP, Neumayr LD, Gold JI, et al. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA 2010;303: Kwiatkowski JL, Zimmerman RA, Pollock AN, et al. Silent infarcts in young children with sickle cell disease. Br J Haematol 2009;146: Adams RJ, Ohene-Frempong K, Wang W. Sickle cell and the brain. Hematology Am Soc Hematol Educ Program 2001: Schnog JB, Duits AJ, Muskiet FA, et al. Sickle cell disease; a general overview. Neth J Med 2004;62: Page 6 of 7

7 Debaun MR, Armstrong FD, McKinstry RC, et al. Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood 2012;119: Bernaudin F, Verlhac S, Freard F, et al.multicenter prospective study of children with sickle cell disease: radiographic and psychometric correlation. J Child Neurol 2000;15: Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood 1998;91: Page 7 of 7