Typical examples include mutations in PDGFB or PDGFRB growth factor/receptor causing primary familial brain calcification (idiopathic basal ganglia calcification, Fah’s disease), mutations in receptor NOTCH3 associated with cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and tight junction OCLN in band-like calcification with simplified gyration and polymicrogyria (BLC-PMG), and mutations in basement membrane COL4A1 and COL4A2 underlying cerebral small vessel disease or LAMA2 in congenital muscular dystrophy 1A (MDC1A). Here, PDGFRB is linked to bilateral striopallidodentate calcinosis.