Neurocognitive function in patients with β-thalassemia major.

BACKGROUND: Children with β-thalassemia major (β-TM) have multiple risk factors for developing cognitive impairment. The aim of the present study was to evaluate cognitive function in patients with β-TM.

METHODS: Twenty children with β-TM were enrolled into the study and were compared with a control group consisting of 21 healthy children. All participants were evaluated with neuropsychological tests and event-related potentials (ERP).

RESULTS: All of the participants had normal IQ scores, but the patient group had significantly lower full-scale, performance, and verbal IQs compared with the control group (P < 0.05). The number of children with visuomotor dysfunction was higher in the patient group compared with the control group (P < 0.05). In the P300 test, the patient group had significantly prolonged N1, P2 and N2 latencies at the FZ, and a prolonged N1 latency at the Cz compared with the control group (P < 0.05). The patient group also had lower N1 and P3N4 amplitudes at the Fz, and lower N1, N1P2 and P3N4 amplitudes at the Cz when compared with the control group (P < 0.05). Mismatch negativity latency and duration were longer in the patient group (P < 0.05).

CONCLUSIONS: Neuropsychological tests are safe, and reliable for the diagnosis of cognitive impairment in β-TM patients, and the use of ERP may facilitate early diagnosis. The number of β-TM patients in the present study was limited, however, and larger numbers of patients are required in further studies.