Correlation between the MRI extent of silent cerebral infarcts(scis) in adult sickle-cell disease(scd) patients and the clinical severity of disease
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1 Correlation between the MRI extent of silent cerebral infarcts(scis) in adult sickle-cell disease(scd) patients and the clinical severity of disease Poster No.: C-0451 Congress: ECR 2013 Type: Authors: Scientific Exhibit E. Solomou 1, P. Kraniotis 2, A. Kourakli 2, T. Petsas 2 ; 1 Rion/GR, 2 Patras/GR Keywords: DOI: Haematologic diseases, Treatment effects, MR, Neuroradiology brain, Haematologic, CNS /ecr2013/C-0451 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 11
2 Purpose The main purpose of the study was to correlate the extent of silent cerebral infarcts (SCIs) on MRI with the clinical severity of disease in adult heterozygous (HbS/#-thal) sickle-cell disease(scd) patients. Page 2 of 11
3 Methods and Materials Twenty-four consecutive adult asymptomatic SCD patients (11 male and 13 female) with a mean age of 38.4 years (range 20-59) were submitted to brain MRI on a 1 Tesla Gyroscan Intera, Philips MR scanner with a dedicated head coil. All patients were heterozygous(hbs/#-thal) and had no evidence of focal neurologic signs. MRI protocol consisted of TSE T2-weighted (TR 5300ms, TE 120 ms) and FLAIR (TR 6000 ms, TE 110 ms, TI 2000ms) images in the axial and coronal planes. Imaging parameters included slice thickness of 5 mm and matrix size of 189 x 256. MRI readings were undertaken by two radiologists reaching consensus readings, which were blinded to the clinical results, for evidence of SCIs. The extent of SCIs was classified from 0-2 with 0 designating no lesions(figures 1-4). Patients with normal findings were scored as grade 0. Patients with small unifocal lesions <1cm in diameter or mild extent leukoencephalopathy with lesions measuring <3cm, were scored as grade 1. Patients with more severe/diffuse leukoencephalopathy and lesions>3cm were scored as grade 2. Clinical severity was graded as 0-2 by the hematologist, according to the frequency and severity of angio-occlusive crises. More specifically the clinical severity score was based upon frequency of pain episodes, severity of the symptoms experienced, number of hospitalisations and necessity of episodic blood transfusions, extent of organ involvement, and history of splenectomy. According to the above criteria patients were classified in qualitative terms in 3 groups as suffering either of mild, moderate or severe clinical disease. Page 3 of 11
4 Images for this section: Fig. 1: Sequential caudal to cranial axial T2-weighted images(figures 1-4), from the level of the posterior horns to the level of the bodies of the lateral ventricles. There are multiple lesions in the white matter of this 44-year-old patient, which were scored as grade 2. Magnetic Resonance Imaging Unit, Department of Radiology, University Hospital Patras - Rion/GR Page 4 of 11
5 Fig. 2: Sequential caudal to cranial axial T2-weighted images(figures 1-4), from the level of the posterior horns to the level of the bodies of the lateral ventricles. There are multiple lesions in the white matter of this 44-year-old patient, which were scored as grade 2. Magnetic Resonance Imaging Unit, Department of Radiology, University Hospital Patras - Rion/GR Page 5 of 11
6 Fig. 3: Sequential caudal to cranial axial T2-weighted images(figures 1-4), from the level of the posterior horns to the level of the bodies of the lateral ventricles. There are multiple lesions in the white matter of this 44-year-old patient, which were scored as grade 2. Magnetic Resonance Imaging Unit, Department of Radiology, University Hospital Patras - Rion/GR Page 6 of 11
7 Fig. 4: Sequential caudal to cranial axial T2-weighted images(figures 1-4), from the level of the posterior horns to the level of the bodies of the lateral ventricles. There are multiple lesions in the white matter of this 44-year-old patient, which were scored as grade 2. Magnetic Resonance Imaging Unit, Department of Radiology, University Hospital Patras - Rion/GR Page 7 of 11
8 Results According to clinical criteria 33.33% (8/24) of the patients had mild, 37.50% (9/24) moderate and 29.17% (7/24) severe clinical disease. Abnormalities were evident on MRI in 66.67% (16/24) of the patients. From the patients with abnormal MRI, 8.33% (2/24) were classified as score 1 and 58.33% (14/24) were classified as score 2. From the 8 patients with no abnormalities on the MRI, 25%(2/8) had mild, 37.50% (3/8) had moderate and 37.50%(3/8) had severe clinical disease. Both clinical severity and MRI score were investigated both through the Spearman's correlation coefficient (rho=-0.227, p=0.286) and with tests for independence (p=0.72, chi-square test). There was no statistically significant relation between the them. As the mean age of the patient population was 38.4 years, the patients were subcategorized into two groups: the younger group with age under 38.4 years and the older group aged over The difference in MRI scores between the two age groups was not statistically significant (p=0.216, chi-square test). Finally the MRI score was not correlated with hydroxyurea treatment (p=0.230). Page 8 of 11
9 Conclusion The extent of SCIs in heterozygous (HbS/#-thal) SCD patients is not age related and may be quite severe even in younger (<38.4 years) patients. However the extent of SCIs in MRI is not correlated with the severity of clinical disease. Patients on hydroxyurea do not have statistically significant differences in the extent of SCI lesions. Page 9 of 11
10 References 1. Debaun MR, Armstrong FD, McKinstry RC, Ware RE, Vichinsky E, Kirkham FJ. Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood 2012;119(20): Manfre L, Giarratano E, Maggio A, Banco A, Vaccaro G, Lagalla R. MR imaging of the brain: findings in asymptomatic patients with thalassemia intermedia and sickle cellthalassemia disease. AJR Am J Roentgenol 1999;173(6): Marouf R, Gupta R, Haider MZ, Adekile AD. Silent brain infarcts in adult Kuwaiti sickle cell disease patients. Am J Hematol 2003;73(4): Zafeiriou DI, Prengler M, Gombakis N, Kouskouras K, Economou M, Kardoulas A, Tsantali C, Dimitriadis A, Athanasiou M, Kirkham FJ. Central nervous system abnormalities in asymptomatic young patients with Sbeta-thalassemia. Ann Neurol 2004;55(6): Moran CJ, Siegel MJ, DeBaun MR. Sickle cell disease: imaging of cerebrovascular complications. Radiology 1998;206(2): Steen RG, Hankins GM, Xiong X, Wang WC, Beil K, Langston JW, Helton KJ. Prospective brain imaging evaluation of children with sickle cell trait: initial observations. Radiology 2003;228(1): Vichinsky EP, Neumayr LD, Gold JI, Weiner MW, Rule RR, Truran D, Kasten J, Eggleston B, Kesler K, McMahon L, Orringer EP, Harrington T, Kalinyak K, De Castro LM, Kutlar A, Rutherford CJ, Johnson C, Bessman JD, Jordan LB, Armstrong FD. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA 2010;303(18): Page 10 of 11
11 Personal Information E. Solomou: Assoc. Prof. of Radiology, University Hospital of Patras, Greece. Page 11 of 11
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