Cognitive function and event related potentials in children with beta-thalassemia

Over the years, several reports have demonstrated the involvement of the nervous system in patients with betathalassemia. In most cases, neurological involvement does not initially present with relevant signs or symptoms (i.e., subclinical). This study was designed to assess P300 and cognitive function in a group of children, who were neurologically asymptomatic children with beta-thalassemia. Thirty children with beta-thalassemia were selected from the pediatric department, hematology unit at Assiut university hospital. Another 40 apparently healthy children of the same age, sex, body mass index and social state were chosen as control. Assessment of clinical, laboratory, cognitive functions using Stanford Binet and event related potential were done. The mean age of children was 9.97 +/- 3.24 years; the mean duration of illness was 8.53 +/- 3.44 years. We reported significant impairment in cognitive functions compared with control group. Also significant prolongation in P200, N2 and P300 latencies, while significant decreased in P300-N2 amplitude was reported. Splenectomy and infrequent blood transfusion act as additional factors for cognitive impairment. Children with beta-thalassemia have subclinical involvement of the central neural pathway. Cognitive function and event related potentials should be done as early as possible for early detection of neural pathway impairment.