disease responds to vitamin B1 thiamine-responsive dysfunction syndrome https://github.com/monarch-initiative/mondo/issues/9437 A disorder of thiamine metabolism and transport results from deficiency of thiamine metabolism, comprises a group of clinically and genetically heterogeneous encephalopathies with autosomal recessive inheritance. thiamine-responsive dysfunction syndrome MONDO:0000152 OMIMPS:249270 GARD:0022716 disorder of thiamine metabolism and transport