Objective: Moyamoya disease (MMD) is a progressive steno-occlusive cerebrovascular phenomenon with unknown pathogenesis. Considering the abundance of articles addressing Moyamoya disease, a detailed analysis concerning the publication trends is of paramount importance. The aim of the study is to report the current knowledge of the top-100 most cited articles on Moyamoya disease in the literature. Methods: A non-time restricted keyword-based search was performed in June 2020 using the Scopus database. The search keywords included the following: “Moyamoya”, “Moyamoya disease”, and “Moyamoya syndrome”. The search result was used to rank the articles based on their citation count. The top-100 most-cited articles were obtained and classified into seven categories. Results: A total of 3,543 articles on Moyamoya disease were published between 1955 and 2020. The Top-100 articles were published between 1977 and 2016 with a total of 16,119 citations, per year, and 7.23% rate of self-citation. The 1990s was the most productive decade (N=42). The most contributing country to the list was Japan (N=60). Stroke was the most active journal (N=23). Houkin, K., a Japanese neurosurgeon, was the most prolific author (N=15). Conclusions Moyamoya disease has been extensively investigated in the literature throughout the years. The majority of articles published in the literature were addressing the surgical management and clinical outcome. Authors from neurosurgical backgrounds were the most active contributors to the field of Moyamoya disease.

Objective: Moyamoya disease (MMD) is a progressive steno-occlusive cerebrovascular phenomenon with unknown pathogenesis. Considering the abundance of articles addressing Moyamoya disease, a detailed analysis concerning the publication trends is of paramount importance. The aim of the study is to report the current knowledge of the top-100 most cited articles on Moyamoya disease in the literature. Methods: A non-time restricted keyword-based search was performed in June 2020 using the Scopus database. The search keywords included the following: “Moyamoya”, “Moyamoya disease”, and “Moyamoya syndrome”. The search result was used to rank the articles based on their citation count. The top-100 most-cited articles were obtained and classified into seven categories. Results: A total of 3,543 articles on Moyamoya disease were published between 1955 and 2020. The Top-100 articles were published between 1977 and 2016 with a total of 16,119 citations, per year, and 7.23% rate of self-citation. The 1990s was the most productive decade (N=42). The most contributing country to the list was Japan (N=60). Stroke was the most active journal (N=23). Houkin, K., a Japanese neurosurgeon, was the most prolific author (N=15). Conclusions Moyamoya disease has been extensively investigated in the literature throughout the years. The majority of articles published in the literature were addressing the surgical management and clinical outcome. Authors from neurosurgical backgrounds were the most active contributors to the field of Moyamoya disease.