In dystroglycanopathies, hydrocephalus is driven by aqueduct obstruction due to aberrational integrity of basement membranes in the brain, caused by mutant genes encoding for glycosyltransferases, that is, protein O-mannosyltransferase 1 (POMT1), Fukutin-related protein (FKRP), acetylglucosaminyltransferase-like protein (LARGE), and protein kinase B (AKT) (Langenbach and Rando, 2002; Akasaka-Manya et al., 2004; Satz et al., 2008). Here, FKRP is linked to neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.