Amelocerebrohypohidrotic Syndrome Watchlist (log in to enable) Retrieved 2021-01-23 Source Orphanet Trials — Genes ROGDI, SLC13A5, ACADSB, AUH, DHCR7, HSD17B10, ACAD8, FKBP14 Drugs — Interested in hearing about new therapies? Registered! Kohlschütter-Tönz syndrome (KTS) is a genetically heterogeneous autosomal recessive syndrome characterized by the triad of amelogenesis imperfect, infantile onset epilepsy, intellectual disability with or without regression and dementia.