International Journal of Hematology

, Volume 96, Issue 6, pp 710–718 | Cite as

Adverse neurological outcomes in Nigerian children with sickle cell disease

  • I. A. Lagunju
  • B. J. Brown
Original Article


Sickle cell disease (SCD) is reported to be the most common genetic disorder affecting Nigerians. Children with SCD are at a high risk of neurological morbidity. The main objective of this study was to determine the pattern of adverse neurological outcomes among a cohort of Nigerian children with SCD. All children with SCD seen in the Department of Paediatrics, University College Hospital, Ibadan, Nigeria, over a period of 2 years were carefully evaluated for symptoms and signs of neurological complications, defined as clinical outcomes referable to the central nervous system. Of the 214 children evaluated, 187 were diagnosed with Hb SS disease and 27 with Hb SC disease. Neurological complications were identified in 78 (36.4 %) of the cases. The most common complications were headache (17.8 %), seizure (9.3 %) and stroke (8.4 %). Other less frequent complications included bacterial meningitis (2.8 %), spontaneous visual loss (1.4 %), paraplegia (0.9 %) and transient ischaemic attacks (0.9 %). Neurological complications occurred more frequently in children with sickle cell anaemia than in those with Hb SC disease (P = 0.002, 95 % CI 1.450–82.870). Adverse neurological events are common in Nigerian children with SCD, with a significantly higher risk in Hb SS than Hb SC disease. Stroke represents a major underlying cause of symptomatic epilepsy in SCD. Institution of primary preventive measures for stroke in SCD will significantly reduce the burden of stroke and epilepsy associated with SCD in Nigeria.


Sickle cell disease Neurological Complications Stroke 



The authors acknowledge the support of the resident doctors, Department of Paediatrics, UCH, Ibadan.

Conflict of interest

The authors have nothing to disclose and there is no conflict of interest.


  1. 1.
    Ohene-Frempong K. Abnormalities of haemoglobin structure and function. Sickle Cell Disease. In: Rudolph CD, Rudolph AM, Hostetter MK, Lister G, Siegel NJ, editors. Rudolph pediatrics. New York: McGraw Hill Medical Publishing Division, 2003. p. 1534–40.Google Scholar
  2. 2.
    Saunthararajah Y, Vichinsky EP, Embury SH. Sickle cell disease. In: Hoffman R, Benz EJ, Shattil SJ, et al., editors. Haematology basic principles and practice. Philadelphia: Elsevier; 2005. p. 605–43.Google Scholar
  3. 3.
    Serjeant GR. Sickle cell disease. Oxford: Oxford University Press; 1985. p. 1–12.Google Scholar
  4. 4.
    Hebbel RP, Boogaertes MAB, Eaton JN, Steinberg MH. Erythrocyte adherence to endothelium in sickle cell anaemia; a possible determinant of disease severity. N Engl J Med. 1980;302:992–5.PubMedCrossRefGoogle Scholar
  5. 5.
    Elion J, Laurance S, Lapoumeroulie C. Pathophysiology of sickle cell disease. Med Trop (Mars). 2010;70:454–8.Google Scholar
  6. 6.
    Samaik AS, Lusher JM. Neurological complications of sickle cell anaemia. Am J Pediatr Hematol Oncol. 1982;4:386–91.CrossRefGoogle Scholar
  7. 7.
    Amayo EO, Owade JN, Aluochi JR, Njeru EK. Neurological complications of sickle cell anaemia at KNH: a five year retrospective study. East Afr Med J. 1992;69:660–2.PubMedGoogle Scholar
  8. 8.
    Kirkham FJ. Therapy insight: stroke risk and its management in patients with sickle cell disease. Nat Clin Pract Neurol. 2007;3:264–78.PubMedCrossRefGoogle Scholar
  9. 9.
    Prengler M, Pavlakis SG, Prohovnik I, Adams RJ. Sickle cell disease: the neurological complications. Ann Neurol. 2002;51:543–52.PubMedCrossRefGoogle Scholar
  10. 10.
    Ali SB, Reid M, Fraser R, MooSang M, Ali M. Seizures in the Jamaican cohort study of sickle cell disease. Br J Haematol. 2010;151:265–72.PubMedCrossRefGoogle Scholar
  11. 11.
    Brown BJ, Okereke JO, Lagunju IA, Orimadegun AE, Ohaeri JU, Akinyinka OO. Burden of healthcare of caregivers of children with sickle cell disease in Ibadan. Health Soc Care Community. 2010;18:289–95.PubMedGoogle Scholar
  12. 12.
    Adams RJ. Big strokes in small persons. Arch Neurol. 2007;64:1567–74.PubMedCrossRefGoogle Scholar
  13. 13.
    Ohene-Frempong K, Weiner S, Sleeper L, et al. Cerebrovascular accidents in sickle cell disease- rates and risk factors. Blood. 1998;91:288–94.PubMedGoogle Scholar
  14. 14.
    Fatunde OJ, Adamson FG, Ogunseyinde O, et al. Stroke in Nigerian children with sickle cell disease. Afr J Med Med Sci. 2005;34:157–60.PubMedGoogle Scholar
  15. 15.
    Kehinde MO, Temiye EO, Danesi MA. Neurological complications of sickle cell anaemia in Nigerian Africans––A case–control study. J Natl Med Assoc. 2008;100:394–9.PubMedGoogle Scholar
  16. 16.
    Lagunju IA, Brown BJ, Famosaya A. Childhood stroke in sickle cell disease. J Ped Neurol. 2011;9:49–53.Google Scholar
  17. 17.
    Lewis DW. Headaches in Infants and Children. In: Swaiman KF, Ashwal S, Ferriero DM, editors. Pediatric neurology principles and practice (Fourth Edition). Philadelphia:Mosby Elseiver; 2006. p. 1183–202.Google Scholar
  18. 18.
    Commission on Epidemiology and Prognosis, International League Against Epilepsy. Guidelines for epidemiological studies on epilepsy. Epilepsia. 1993;34:592.Google Scholar
  19. 19.
    WHO Monica Project Investigators. The WHO MONICA Project (Monitoring trends and determinants in cardiovascular disease). J Clin Epidemiol. 1988;41:105–14.CrossRefGoogle Scholar
  20. 20.
    Greenwood DM, Bojang K, Whitty CJ, Targett GA. Malaria. Lancet. 2005;365:1487–98.PubMedCrossRefGoogle Scholar
  21. 21.
    Boulet SL, Yanni EA, Creary MS, Olney RS. Health status and healthcare use in a national sample of children with sickle cell disease. Am J Prev Med. 2010;38:S528–35.PubMedCrossRefGoogle Scholar
  22. 22.
    Hines PC, McKnight TP, Seto W, Kwiatkowski JL. Central nervous system events in children with sickle cell disease presenting acutely with headache. J Pediatr. 2011;159:472–8.PubMedCrossRefGoogle Scholar
  23. 23.
    Liu JE, Gzesh DJ, Ballas SK. The spectrum of epilepsy in sickle cell anaemia. J Neurol Sci. 1994;123:6–11.PubMedCrossRefGoogle Scholar
  24. 24.
    Adams RJ. Neurological complications. In: Embury SH, Hebbel RP, Mohandas N, Steinberg MH, editors. Sickle cell disease: basic principles and clinical practice. New York: Raven Press; 1994. p. 599–621.Google Scholar
  25. 25.
    Osuntokun BO, Adeuja AO, Nottidge VA, et al. Prevalence of epilepsies in Nigerian Africans: a community-based study. Epilepsia. 1987;28:272–9.PubMedCrossRefGoogle Scholar
  26. 26.
    Longe AC, Osuntokun BO. Prevalence of neurological disorders in Udo, a rural community in Southern Nigeria. Trop Geogr Med. 1989;41:36–40.PubMedGoogle Scholar
  27. 27.
    Izuora GI, Azubike JC. Prevalence of seizure disorders in Nigerian children around Enugu. East Afr Med J. 1977;54:276–80.PubMedGoogle Scholar
  28. 28.
    Adamolekun B, Durosinmi MA, Olowu W, Adediran I. The prevalence and classification of epileptic seizures in Nigerians with sickle cell anaemia. J Trop Med Hyg. 1993;96:288–90.PubMedGoogle Scholar
  29. 29.
    Prengler M, Pavlakis SG, Boyd S, et al. Sickle cell disease: ischaemia and seizures. Ann Neurol. 2005;58:290–302.PubMedCrossRefGoogle Scholar
  30. 30.
    Izuora GI, Kaine WN, Emodi I. Neurological disorders in Nigerian children with homozygous sickle cell anaemia. East Afr Med J. 1989;66:653–7.PubMedGoogle Scholar
  31. 31.
    DeBaun MR, Sarnaik SA, Rodeghier MJ, et al. Associated risk factors for silent cerebral infarcts in sickle cell anaemia: low baseline hemoglobin, sex and relative high systolic blood pressure. Blood. 2012;119:3684–90.PubMedCrossRefGoogle Scholar
  32. 32.
    Platt OS. Prevention and management of stroke in sickle cell anaemia. Hematology Am Soc Hematol Educ program. 2006;5:54–7.CrossRefGoogle Scholar
  33. 33.
    Adams RJ, McKie V, Nichols F, et al. The use of transcranial Doppler ultrasonography to predict stroke in sickle cell disease. New Engl J Med. 1992;326:605–10.PubMedCrossRefGoogle Scholar
  34. 34.
    Adams RJ, McKie VC, Hsu L, et al. Prevention of a first stroke by transfusions in children with sickle cell anaemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. 1998;339:5–11.PubMedCrossRefGoogle Scholar
  35. 35.
    Bernaudin F, Verlhac S, Arnaud C, et al. Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anaemia cohort. Blood. 2011;117:1130–40.PubMedCrossRefGoogle Scholar
  36. 36.
    Carrizosa J, Lopera JE, Cornejo W. Cerebrovascular disease in children with sickle cell anaemia. Rev Neurol. 2003;37:511–4.PubMedGoogle Scholar
  37. 37.
    Osafo-Kwaako A, Kimani K, Ilako D, et al. Ocular manifestations of sickle cell disease at the Korle-bu Hospital, Accra, Ghana. Eur J Ophthalmol. 2011;21:484–9.PubMedCrossRefGoogle Scholar
  38. 38.
    Fadugbagbe AO, Gurgel RQ. Mendon Asa CQ, Cipolotti R, dosSantos AM, Guevas LE. Ocular manifestations of sickle cell disease. Ann Trop Paediatr. 2010;30:19–26.PubMedCrossRefGoogle Scholar
  39. 39.
    Balogun RA, Obalum DC, Giwa SO, Adekoya-Cole TO, Ogo CN, Emmeluzo GO. Spectrum of musculoskeletal disorders in sickle cell disease in Lagos, Nigeria. J Orthop Surg Res. 2010;5:2.PubMedCrossRefGoogle Scholar
  40. 40.
    Rothman SM, Nelson JS. Spinal cord infarction in a patient with sickle cell anaemia. Neurology. 1980;30:1072–6.PubMedCrossRefGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2012

Authors and Affiliations

  1. 1.Department of Paediatrics, College of Medicine University of IbadanIbadanNigeria
  2. 2.Department of PaediatricsUniversity College HospitalIbadanNigeria

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