Abstract
BACKGROUND AND PURPOSE: Patients with episodic ataxia type 2 (EA2) suffer from recurrent paroxysmal episodes of vertigo and oscillopsia. Pathophysiologically, altered neuronal excitability has been suspected. Vestibular excitability in 22 EA2 patients and 22 age-matched healthy participants was compared. METHODS: Galvanic vestibular stimulation (GVS) was used to assess vestibular excitability by vestibular motion perception thresholds and mean postural sway velocity during various visual and proprioceptive conditions in the two groups. Control stimuli using sham and no GVS were established to identify the specificity of GVS-induced postural sway. RESULTS: In the baseline condition, EA2 patients showed larger postural instability. However, motion perception thresholds and the increase in mean postural sway velocity during vestibular stimulation (stimulation ratio) did not differ between groups. Postural sway during suprathreshold GVS increased with the vestibular motion perception threshold in EA2 patients, in contrast to healthy participants. CONCLUSIONS: The larger postural unsteadiness of EA2 patients probably reflects their progressive cerebellar degeneration. It is not related to abnormal visual (Romberg's ratio) or proprioceptive control of stance. Postural unsteadiness during vestibular stimulation does not indicate altered vestibular excitability in EA2 patients. However, vestibular stimulation increasingly destabilized postural control of EA2 patients with higher motion perception thresholds when proprioceptive information was diminished. This conclusion, however, is restricted to the postural control of EA2 patients in the interval between the vestibulo-cerebellar episodes.