What is the expert's option on antiplatelet therapy in moyamoya disease? Results of a worldwide Survey

Background and purpose: Moyamoya disease (MMD) is a rare idiopathic vasculopathy characterized by an extensive network of fine collaterals in the setting of bilateral progressive stenosis and finally occlusion of the intracranial portion of the internal carotid artery and proximal anterior and medial arteries of Circle of Willis. Owing to the rarity of the disease and ethical concerns, double-blinded, randomized controlled trials about treatment options are completely lacking. The appropriate conservative treatment instead, before or after revascularization surgery, is nearly completely neglected in scientific literature, even in Asia.

Methods: We developed a questionnaire with response options offered in the multiple choice method. The survey was sent with the request to reply within September 2010 to January 2011 by email to experts in the treatment of MMD. As an international expert, authors were selected who had written more than one scientific article or book chapter on the subject. We took special care not to select only Asian scientists, but also considered experts on the disease in Caucasian patients.

Results: Among the 77 physicians surveyed, 32 (41.55%) responded. Twenty-one (65.6%) Asian experts and 11 (34.4%) non-Asian experts participated in this survey. The majority of experts thought that long-term antiplatelet therapy is not essential in the treatment of MMD. Ten of 32 (31%) participants agreed to long-term antiplatelet treatment with acetylsalicylic acid 100 mg/day. There was a significant difference in answers between Asian and non-Asian experts (P = 0.0128).

Conclusion: It is interesting that the majority of Non-Asian respondents recommend antiplatelet drugs, while this is an unusual approach in MMD of the Asian experts. Perhaps, this significant difference is well founded by the different experiences of the experts related to the difference in disease presentation between Asians and Caucasians. The role of conservative and surgical treatment in MMD needs further evaluation with larger cohorts and a focus on long-term clinical outcome.