Moyamoya Syndrome Associated with Protein C and S Deficiency as a Neurological Complication in Children with Sickle Cell Disease

Abstract

Background: Sickle Cell Disease (SCD) is an autosomal recessive hemoglobinopathy containing mutant sickle cell Hemoglobin (HbS). Acute and chronic organ dysfunction, chronic hemolytic anemia, and recurrent painful episodes are the main features of sickle cell disease. There is a wide variety of neurological complications, including: headache, cognitive difficulties, seizures, visual loss, ischemic and hemorrhagic stroke, transient ischemic attacks, soft neurological signs coma, altered mental status and covert or silent infarction. Moyamoya is an uncommon cerebral vasculopathy that’s also found in children with SCD. Case Presentation: Here, we reported two children diagnosed with SCD who presented with headache, recurrent ischemic strokes, seizures and cognitive decline. They showed abnormalities on different neuroimaging including: CT and /or MRI, MRA and/or CT angiography, also transcranial color coded duplex, EEG and Stanford-Binet Intelligence scales-Fifth Edition. They also showed abnormal level of protein C and protein S. They also had irregular blood transfusion and were diagnosed as Moyamoya syndrome. Conclusion: According to our findings, Moyamoya syndrome was a rare complication of SCD in children, but should be considered with proper approach, diagnosis and management.