Ocular palatal tremor plus dystonia - new syndromic association

Mov Disord Clin Pract. 2015 Sep 1;2(3):267-270. doi: 10.1002/mdc3.12193. Epub 2015 Jun 17.

Abstract

Objective: Ocular palatal tremor 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 six patients with ocular palatal plus dystonia.

Results: Among six patients with ocular palatal tremor two had focal dystonia, three had multifocal dystonia, and one had generalized dystonia. The dystonia affected the upper extremities and neck in four patients, the lower extremities in three and the face in two. Three out 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 ocular palatal tremor with dystonia. We speculate that in such variant the dystonia is possibly could be a result of abnormal cerebellar outflow in patients with a breach in Guillain-Mollaret triangle.

Keywords: brainstem; cerebellum; inferior olive; nystagmus; oscillation.