https://www.selleckchem.com/products/gsk269962.html The age-dependency represents a striking new aspect of these phenotypes und highlights a pivotal role for P/Q channels in the development of the central nervous system in a defined time window. While several reviews addressed the clinical presentation and treatment of FHM1 and EA2, an overview of the newly described age-dependent manifestations is lacking. In this Mini-Review we present a clinical update, delineate genotype-phenotype correlations as well as summarize evidence on the pathophysiological mechanisms underlying the expanded phenotype associated with CACNA1A variants.Background Moyamoya angiopathy (MMA), which includes moyamoya disease (MMD) and moyamoya syndrome (MMS), is an uncommon cerebrovascular condition characterized by recurrent stroke. We carried out a bibliometric analysis to examine the development of and research trends in MMA research. Methods Studies published between 2010 and 2019 on MMA were retrieved from the Web of Science Core Collection (WoSCC) on August 14, 2020, and bibliometric and visualization-based analyses were performed by using three different scientometric tools HistCite, VOSviewer, and CiteSpace. Results A total of 1,896 publications published in 384 journals by 6,744 authors, 1,641 institutions and 56 countries/regions were included in the analyses. Annual publication outputs increased from 2010 to 2019. The USA, Japan and China were three key contributors to this study field. Capital Medical University, Seoul National University, and Stanford University were three major institutions with larger numbers of publications. Zhang D, World Neurosurgery, Kuroda S, and STROKE were the most prolific author, prolific journal, top co-cited author and top co-cited journal, respectively. The top five keywords during this period were moyamoya disease, revascularization, stroke, children and surgery, while revascularization surgery and RNF213 were the most common frontier topics. Conclu