Our finding of comorbid ASD and PANS in the three SHANK3 cases is similar to observations made by Bey et al., who identified de novo SHANK3 variants in four girls with lifelong, stable developmental delay (DD) who developed subacute, severe psychiatric symptoms resembling PANS, that responded, with varying degrees of success, to immunotherapy66. Here, SHANK3 is linked to dentin dysplasia.