The patient had adult GM1 gangliosidosis (type 3) with severe impairme
nt of mastication caused by dystonia of anterior digastric muscles (ja
w-opener) on clenching. This is the first report on jaw dystonia sever
e enough to cause the masticatory impairment in adult GM1 gangliosidos
is. The discordance of closing and opening muscles during mastication
might be caused by a basal ganglia lesion in this disease. (C) 1997 El
sevier Science BN.