Neurophysiologic Evaluation of Children with Beta-Thalassemia Major

Abstract

Neural pathway involvement is considered to be a neurological complication in Beta-thalassemia major (BTM). This study aimed to detect possible involvement of central and peripheral neural pathways in a group of neurologically asymptomatic patients with BTM. It was performed on 30 patients diagnosed as having BTM (group I), and 10 healthy children who were matched for age, sex, and height (group II). Serum ferritin, iron, glucose tolerance test, intelligence quotient (IQ) test, and electroencephalogram (EEG) were assessed in BTM patients. Electrophysiologic study was assessed in both groups. The mean age of the patients was 10.45 ± 2.88 years. They were 14 males and 16 females. The results of the present study showed that 66.7% of patients had subclinical sensory peripheral neuropathy. Twenty percent of the patients had abnormal somatosensory evoked potentials. Low average and slow learner total IQ score were found in 63.3% of the studied patients. These findings did not correlate with age, disease duration, serum iron, and ferritin. Twenty-three patients had mature EEG background activity, while seven patients had immature background activity. The EEG background maturity had significant correlation with age, duration of desferrioxamine therapy, and serum iron. There was no correlation between serum ferritin level, EEG, and electrophysiologic findings. Regular electrophysiologic monitoring and intellectual evaluation are recommended to detect relevant abnormalities and apply appropriate management.