P1462: neurocognitive function and psychological assessment in transfusion-dependent beta-thalassemia patients: relation to brain iron content

Topic: 27. Thalassemias Background: Neurological complications in β-thalassemia patients remain subclinical and detected only during neuroimaging evaluation. Cognitive impairment, abnormal findings on evoked potentials and cerebrovascular disease comprise the broad spectrum of neurological involvement. Aims: This study assessed association between central iron overload and neurocognitive function in transfusion-dependent β-thalassemia patients. Methods: Thirty children and adolescents with β-thalassemia were enrolled and compared with 30 age- and sex-matched healthy controls. Patients were studied stressing on transfusion history, chelation therapy, markers of hemolysis and serum ferritin. Brain magnetic resonance imaging (MRI) was performed and brain R2* values of both caudate and thalamic regions were calculated. Neurocognitive functions were assessed using Stanford-Binet Test. Psychological evaluation was done using Taylor Manifest Anxiety and Deck Depression Inventory. Results: Seventeen (56.7%) β-thalassemia patients had under-threshold intelligence quotient (IQ), 36.7% had anxiety and 63.3% had moderate to severe depression. The total IQ was significantly lower among transfusion-dependent β-thalassemia patients compared with healthy control. Right and left thalamus R2* as well as right and left caudate R2* were significantly higher in β-thalassemia patients while T2* values were decreased compared with controls. β-thalassemia patients with borderline and low average IQ had higher right and left thalamus R2* as well as right and left caudate R2* values. Right and left thalamus R2* as well as right and left caudate R2* were negatively correlated to total IQ. Right and left caudate R2* were positively correlated to transfusion index, duration of chelation and liver iron concentration (LIC). Summary/Conclusion: Children and adolescents with β-thalassemia have impaired cognitive functions which may be related to the presence of subclinical levels of brain hemosiderosis. Cognitive function in those patients and brain imaging should be done as early as possible for early detection of neurological complications. Keywords: CNS, Children, beta thalassemia