SLC19A3 and biotin-responsive basal ganglia disease: There is a phenotypic spectrum of diseases in people associated with abnormalities in different regions of the SLC19A3 gene, including biotin-responsive basal ganglia disease (BBGD) [17], Wernicke's-like encephalopathy (WLE)[6], and an encephalopathy described in 4 related Japanese boys [9].