**4. Treatment modalities**

Medical treatment with antithrombotic drugs (aspirin [56], cilostazol [57], clopidogrel [58, 59], low molecular-weight heparin, argatroban) [60] or calcium channel blockers [61] is used for mild asymptomatic cases, although their effectiveness has never been proven convincingly [15, 62]. Cilostazol is preferred over aspirin and clopidogrel because of its lower hemorrhagic risk [60], and clopidogrel recommended when aspirin is not tolerated [60]. These antithrombotic agents are advised for the ischemic-type moyamoya disease [15]. In any case, they are not very useful [63] as some researchers have reported an almost 3-fold chance of future neurological deterioration in the patients treated conservatively compared to those treated surgically [54, 64].

Direct, indirect or combined revascularization provides much better long-term results than conservative treatment, minimising the risk of ischemic and haemorrhagic strokes [54, 65, 66]. Early surgical intervention, particularly in paediatric patients, reduces cerebrovascular events and should be considered in asymptomatic children [44, 67]. Specific hospital perioperative morbidity and mortality have to be considered when recommending surgical treatment to these patients [12]. Thus, surgical treatment should be advised a soon as there is any sign of clinical or subclinical deterioration.
