Moyamoya disease is a cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia. The name “moyamoya” means “puff of smoke” in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage. The disease primarily affects children, but it can also occur in adults.
Sickle-cell disease (SCD), also known as sickle-cell anaemia (SCA), is a group of genetically passed down blood disorders. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances.
Researchers believe that there is an association between sickle cell disease (as well as other hemoglobinopathies) and Moyamoya disease, especially in African children, and they suspect hydroxyurea has a role in it.
In this case study, condition of an African child is described who had a recurrent stroke. She was later diagnosed as having Moyamoya disease while already receiving hydroxyurea.
A 12 year old female child with Sickle Cell Anemia symptoms like fever, dry cough, and vomiting was admitted, resulting in repetitive colonic seizures after eighteen hours. Her complex medical history showed that she was diagnosed sickle cell anaemic at the age of 18 months. She was normal until six years of age when in 2006 she had a stroke followed by another in 2008 and then another in 2009, resulting in both arm and bilateral leg weakness.
With no family history of sickle cell anaemia and no drug allergies, the patient was given home medications from 2009 which included folic acid, junior aspirin, and hydroxyurea. Another stroke in 2010 leads to expressive dysphasia, leading to irregular blood transfusion.
Upon physical examination, the patient was in her senses with normal heart rate and temperature though she was paraplegic and bound to the wheelchair. Series of tests were performed including urinalysis, chromatography, complete blood picture, chest radiograph, ultrasound, computerized tomography, MRA findings leading to the presence of Moyamoya disease.
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.
Makubi1, A., Soka, D. and Makani, J., Moyamoya Disease, a Rare Cause of Recurrent Strokes in an African Sickle Cell Child: Does hydroxyurea have a Role in this Context?, International Journal of Child Health and Nutrition, 82-85.