Moya moya syndrome

Definition

• Chronic progressive stroke syndrome caused by bilateral carotid occlusion

Differential Diagnosis

For the radiographic appearance of moya moya

• Primary moya moya syndrome
• Atherosclerosis
• Cranial irradiation
• Pediatric diseases
  • Down/Turner/Alagille syndromes
  • Sickle cell disease
  • Neurofibromatosis type 1
  • Tuberous sclerosis
  • Sturge-Weber syndrome
  • Metabolic disorders
  • Gongenital heart disease
• Infections
  • Meningitis
  • Other viral or bacterial infections
• Hematologic disorders
  • Sick cell disease
  • Beta thalassemia
  • Fanconi anemia
  • Hereditary spherocytosis
  • Homocystinuria and hyperhomocyteinemia
  • Factor XI deficiency
  • Essential thrombocythemia
• Autoimmune disease
  • Systemic lupus erythematosus
  • Polyarteritis nodosa
  • Graves disease and thyroiditis
  • Antiphospholipid antibody syndrome
  • Type 1 diabetes
• Brain neoplasm
• Head trauma

Risk Factors

• Japanese and Asian population
• Family history

Management

• Antiplatelet therapy (e.g. aspirin)
• Revascularization
  • EC-IC bypass

Prognosis

• Often die from intracranial hemorrhage