Orodental self-mutilation (ODSM) has not gained sufficient recognition
in familial dysautonomia (FD). Among 38 patients with FD, ODSM was fo
und in 14 (36.8%). ODSM may be due to peripheral neuropathy with insen
sibility to pain, which is characteristic of FD. Elimination of the sh
arp edges of teeth was found to be helpful.