UCL logo

UCL Discovery

UCL home » Library Services » Electronic resources » UCL Discovery

Central nervous system abnormalities in asymptomatic young patients with S beta-thalassemia

Zafeiriou, DI; Prengler, M; Gombakis, N; Kouskouras, K; Economou, M; Kardoulas, A; Tsantali, C; ... Kirkham, FJ; + view all (2004) Central nervous system abnormalities in asymptomatic young patients with S beta-thalassemia. ANN NEUROL , 55 (6) 835 - 839. 10.1002/ana.20104.

Full text not available from this repository.

Abstract

Twenty-one children and young adults with sickle/beta-thalassemia without overt stroke were examined with magnetic resonance imaging and angiography (MRA), transcranial Doppler (TCD), visual (VEP) and median nerve somatosensory (SEP)-evoked potential recordings, and neuropsychological testing (Wechsler Intelligence Scale [WISC-III]). Eight (38%) had silent infarction in the parietooccipital cortex, deep white matter, or basal ganglia, including two of three with previous seizures. Of 17 undergoing TCD, none had maximum middle cerebral artery (MCA) velocities greater than 126cm/sec, but 9 were abnormal, with low velocities and difficulty in tracking the MCA and/or asymmetry. Three patients had abnormal MRA, one of whom also had silent infarction. One patient had pathological VEP recordings, whereas all SEP recordings were normal. WISC-III was performed in all 11 children, 4 with silent infarction: all but 1 had IQ scores greater than 85 (mean, 97.7; standard deviation, 14.2). We conclude that Greek children and young adults with Sbeta-thalassemia and no history of clinical stroke have TCD abnormalities and silent infarction similar to those reported in children and adolescents with sickle cell anemia, but cognitive function is not necessarily compromised. International collaboration is needed to establish the risk factors for central nervous system sequelae in patients with sickle cell disease, including Sbeta-thalassemia, leading to evidence-based prevention.

Type: Article
Title: Central nervous system abnormalities in asymptomatic young patients with S beta-thalassemia
DOI: 10.1002/ana.20104
Keywords: SICKLE-CELL-DISEASE, TRANSCRANIAL DOPPLER ULTRASONOGRAPHY, SILENT CEREBRAL INFARCTS, RISK-FACTORS, STROKE RISK, CHILDREN, ANEMIA, BRAIN, INTELLIGENCE, ANGIOGRAPHY
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > UCL GOS Institute of Child Health > ICH Developmental Neurosciences Prog
URI: http://discovery.ucl.ac.uk/id/eprint/7977
Downloads since deposit
0Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item