Neurological manifestations in Sickle Cell Disease (SCD) in children

Dr Chavan Ravindra; Dr Tiple Nishikant; Dr Sangeeta Chavan

Volume : V, Issue : X, October - 2015

Neurological manifestations in Sickle Cell Disease (SCD) in children

Dr Chavan Ravindra, Dr Tiple Nishikant, Dr Sangeeta Chavan

Abstract :

Introduction: Sickle cell disease (SCD) is autosomal recessive inherited condition characterized by presence of anomalous haemoglobin ‘S’ in the erythrocytes. Patients with SCD inherit an abnormal haemoglobin which becomes insoluble when deoxygenated and so distorts the red cells and cause tissue infarction1,2,3. The organs mainly affected are spleen, the bones, the kidney, the lung and the skin. But any organ may be involved and the ain is not exemption. Hence the study is taken up to find out neurological manifestations in SCD in children. Aims & Objectives: To find out prevalence, nature and outcome of neurological manifestations in SCD in children. Material and Methods: This prospective study was conducted in pediatrics department of tertiary care hospital from Oct 2000 to April 2002. The study group includes SCD patients admitted in pediatrics ward and patients attending SCD speciality clinics, who were electrophoretically confirmed for diagnosis of sickle cell haemoglobinopathy. A detail history, clinical examination and routine investigation were done in each case. Observation and Results: A total of 204 case of SCD who were electrophoretically confirmed for diagnosis of sickle cell haemoglobinopathy were enrolled during study period, out of which 120(58.82%) patients were homozygous “SS” and 84(41.17%) patients were heterozygous “AS” for SCD. Out of 120 patients of haemoglobin “SS” 16 patients had neurological symptoms while no patients of haemoglobin “AS” had any neurological symptoms. The mean age of presentation of neurological manifestations in the present study is 6.5 years, out of which 7 were boys and 9 were girls. The convulsion was most common symptom in 9(56.25%) followed by hemiparesis in 5(31.25%), monoparesis in 1(6.25%) and 1(6.25%) patient had quadriparesis. Out of 16 patients with neurological manifestation 12 patients (75%) had complete recovery, 1 patient (6.25%) had neurodeficit, 1 patient had transient ischaemic attack and 2 patients (12.5%) died. Conclusion: The neurological manifestations were more common in Homozygous “SS” patients compared to Heterozygous “AS” patients. The mean age of presentation of neurological manifestations in the present study is 6.5 years. The most common neurological presentation in SCD found in our study was convulsion followed by cranial nerve palsies, hemiparesis, monoparesis and quadriparesis.