Frequency of neurological manifestations in β-thalassaemic patients in Zagazig University Hospitals

Abstract

Background: Beta thalassemia syndrome is a hereditary disorder characterized by reduced or absent synthesis of thebeta chains of hemoglobin that disturbs the normal shape of red blood cells. Chronic hypoxia of the nerves resulting from severe anemia may contribute to the pathogenesis of the peripheral neuropathy in patients with β-thalassemia. The aim of this study was to find out the frequency of neurological manifestations in β- thalassemia patients and to determine the contributing factors that lead to these manifestations.
Patients and Methods: This study was prospective cross sectional study conducted during the period from June 2019 to December 2020. This study was carried out on 120 thalassemia patients (67 males and 53 females), with ages ranged from 11 to 22 years old with a mean age of 16.45+3.31 years.
Results: About 31.7% of the studied cases had neurological manifestations. Tingling and numbness were the main neurological symptoms among cases (24.1% and 23.3% respectively) followed by headache (21.7%), joint and muscle pain (20.8%) and tremors in hands (3.3%). Hypotonia was found in 25 cases (20.8%). Grade 4 muscle power was reported in 13 cases (10.8%) and normal (grade 5) muscle power was reported in 107 cases (89.1%). Deep tendon reflexes were normal in (89.2%) cases, while (10.8%) cases had brisk deep tendon reflexes.
Conclusion: Frequency of neurological manifestations in beta thalassemia patients was 31.7%. About 26.3% of them had abnormal nerve conduction study (NCS). Age >16 years old, short stature, prolonged duration of the disease, transfusion frequency >10 times/year, delayed puberty and jaundice were risk factors for neurological manifestations in our studied cases.