Table 2.

Main acquired and inherited MMS and minimal etiologic investigations when discovering a moyamoya angiopathy

Current causes of acquired MMS	Key diagnosis features
Cephalic or neck irradiation112	Interview
Skull base tumor111	Imaging of the skull base
Atherosclerosis of skull base arteries	Complete imaging of cervical arteries
Chronic meningitis (especially tuberculosis meningitis), cerebral vasculitis110	Cerebrospinal fluid analysis
Autoimmune angiitis113	Autoimmune blood tests (anti-nuclear antibodies)
Prothrombotic disorders114	Antithrombin, protein C/protein S, activated protein C resistance, factor V Leiden mutation, prothrombin mutation
Current causes of inherited MMS
Sickle cell disease or trait136–138	Hemoglobin electrophoresis (if from African or Caribbean origin)

Abbreviation: MMS, moyamoya syndromes.