Hypertrophied Frenuli, Oligophrenia, Familial Trembling and Anomalies of the Hand

Abstract

IN 1954 Papillon-Léage and Psaume^{1 , 2} collected from the literature and from their own files a group of 8 patients having similar malformations. This symptom complex was described as affecting only females and inherited as an incomplete recessive. It consisted of abnormally developed frenuli that, by extension, cleft the jaws and tongue. These clefts were situated in the lateral incisor-canine regions. Malposition of maxillary canine teeth, frequent infraocclusion and absence of mandibular lateral incisors were commonly observed. Frequently associated with these oral anomalies were the following: hypoplasia of the base of the skull and extension of the cranial vault, with increased . . .