Ocular Palatal Tremor Plus Dystonia: New Syndromic Association

Abstract

Objective: Ocular palatal tremor (OPT) typically develops after a breach in the Guillian-Mollaret triangle. We herein describe a variant of this syndrome in which dystonia is also present, hence called, here, ocular palatal tremor plus dystonia. Methods: We assessed eye-head movements and dystonia in 6 patients with ocular palatal plus dystonia. Results: Among 6 patients with OPT, 2 had focal dystonia, 3 had multifocal dystonia, and 1 had generalized dystonia. The dystonia affected the upper extremities and neck in 4 patients, the lower extremities in 3, and the face in 2. Three of four cervical dystonia patients had head tremor. Two patients also had speech involvement. Lack of correlation between eye and head oscillations suggested that head oscillations were not compensatory or secondary to the eye oscillations and vice versa. Conclusions: We describe a novel variant of OPT with dystonia. We speculate that in such a variant, the dystonia possibly could be a result of abnormal cerebellar outflow in patients with a breach in the Guillain-Mollaret triangle.