The clinical spectrum of stereotypies in frontotemporal lobar degeneration

We clinically assessed patients meeting international criteria for frontotemporal lobar degeneration (FTLD) for the presence and characteristics of stereotypies. Of 32 subjects with FTLD (50% women), 19 (60%) had stereotypies. The majority of patients with stereotypies were female (68%). The median age of symptom onset suggestive of FTLD was 58 years (range: 37-74), with a mean time to stereotypy onset of 2.1 years. In all cases, the stereotypies were simple. There were no differences in the age of FTLD onset, functional imaging results, neuropsychological test scores or frequency of mood disorders or psychoses between those with and without stereotypical movements. However, compulsive behavior was noted more often in those with stereotypies (58% vs. 15%, P = 0.02). Of the 19 patients with stereotypies, three had vocal stereotypies, and three had both vocal and motor stereotypies. Of the 16 subjects with motor stereotypies, 9(56%) were appendicular and 7 (44%) were craniocervical.