Auditory startle reaction in primary blepharospasm.

Primary dystonia is associated with abnormal brainstem function, as shown by abnormalities of the blink reflex in blepharospasm (BSP) and of the auditory startle reaction in cervical dystonia. We examined the auditory startle reaction--a brainstem reflex elicited by an unexpected loud stimulus--in patients with primary BSP to expand knowledge on brainstem pathophysiology in primary focal dystonia. Thirteen patients with primary BSP were included and 13 age- and sex-matched healthy volunteers served as controls. Auditory startle responses (ASRs) were elicited by binaural high-intensity auditory stimuli, and reflex electromyographic activity was recorded simultaneously with surface electrodes bilaterally from masseter, orbicularis oculi, sternocleidomastoid, and biceps brachii muscles. Patients with BSP showed higher ASR probabilities (masseter, sternocleidomastoid, biceps brachii), shorter ASR onset latencies (masseter, orbicularis oculi, sternocleidomastoid), and larger ASR area-under-the-curve (masseter, sternocleidomastoid) as compared with normal controls. Habituation of ASRs did not differ significantly between patients and controls. These results corroborate previous findings of increased brainstem excitability in primary BSP but point to a different pattern of brainstem dysfunction compared to cervical dystonia, indicating that different pathophysiological mechanisms are involved in the two types of focal dystonia.