Published September 25, 2012 | Version v1

Moyamoya Disease, a Rare Cause of Recurrent Strokes in an African Sickle Cell Child: Does hydroxyurea have a Role in this Context?

  • 1. School of Medicine, Muhimbili University Health and Allied Sciences, Tanzania
  • 2. Muhimbili-Welcome Trust Sickle research Project, Tanzania

Description

Background: Neurological complications are a significant cause of morbidity and mortality in sickle cell

patients with reported incidence of stroke in Africa as high as 1􀀁3/100 patient per year [1,2]. There is an association

between sickle cell disease (as well as other hemoglobinopathies) and Moyamoya disease [3]. Data on the occurrence

of this condition in African sickle patient are scare. Likewise the role of hydroxyurea among patients with both sickle cell

anemia and Moyamoya disease in preventing stroke has not yet been studied in Africa.

Case presentation: In the present report, we describe an African child who had a recurrent stroke. She was later

diagnosed as having Moyamoya disease while already receiving hydroxyurea.

Conclusion: Moyamoya disease is a rare condition associated with recurrent stroke in African sickle children. The role of

hydroxyurea in this context is still unclear.

Files

IJCHNV1N1A10-Makubi.pdf

Files (422.7 kB)

Name Size
md5:0b140a7e116e905b984ce645d385e318
422.7 kB Preview Download

Additional details

References

  • Izuora GI, Kaine WN, Emodi I. Neurological disorders in Nigerian children with homozygous sickle cell anaemia. East Afr Med J 1989; 66: 653-57
  • Amayo EO, Owade JN, Aluoch JR, Njeru EK. Neurological complications of sickle cell anaemia at KNH: a five year retrospective study. East Afr Med J 1992; 69: 660-62.
  • Fryer RH, Chiriboga CA, Feldstein NA. Sickle cell anemia with moyamoya disease. outcome after EDAS procedure. Paediat Neurol 2003; 29: 124-30. http://dx.doi.org/10.1016/S0887-8994(03)00047-X