Background: Benign essential blepharospasm (BEB), aberrant facial nerve degeneration and hemifacial spasm (HFS) are all examples dystonia which, though not life-threatening, can have a significant impact on patient quality of life. The need for reliable self-rating surveys to monitor functional disability is fundamental. The Blepharospasm Disability Index (BSDI) is already a widely utilised and validated self-rating score for blepharospasm whilst the functional disability score (FDS) requires further validation. The principle aim of this study is to repeat validation of the FDS against the BSDI, which has been validated by several groups since its original description but only in patients with BEB.

Methods: A randomised blinded prospective cohort study was conducted at a single unit on 38 patients with BEB, aberrant facial nerve degeneration and HFS. Patients were blinded to complete the FDS followed by the BSDI or the BSDI followed by the FDS with a 30-minute interval.

Results: Both the FDS and BSDI were found to be reliable with high internal consistency and test-retest reliability. Both scales were also found to be moderately correlated with the Jankovic disease severity score.

Conclusions: This study is the first to use the FDS as a rating scale in patients with HFS and aberrant facial nerve degeneration. It is also the first study to formally validate the FDS as an acceptable rating scale for patients with dystonia and in particular it provides validation for its use in patients with HFS and aberrant facial nerve degeneration.

Background: Research suggests that the analysis of facial expressions by a healthy brain would take place approximately 170 ms after the presentation of a facial expression in the superior temporal sulcus and the fusiform gyrus, mostly in the right hemisphere. Some researchers argue that a fast pathway through the amygdala would allow automatic and early emotional treatment around 90 ms after stimulation. This treatment would be done subconsciously, even before this stimulus is perceived and could be approximated by presenting the stimuli quickly on the periphery of the fovea. The present study aimed to identify the neural correlates of a peripheral and simultaneous presentation of emotional expressions through a frequency tagging paradigm.

Methods: The presentation of emotional facial expressions at a specific frequency induces in the visual cortex a stable and precise response to the presentation frequency [i.e., a steady-state visual evoked potential (ssVEP)] that can be used as a frequency tag (i.e., a frequency-tag to follow the cortical treatment of this stimulus. Here, the use of different specific stimulation frequencies allowed us to label the different facial expressions presented simultaneously and to obtain a reliable cortical response being associated with (I) each of the emotions and (II) the different times of presentations repeated (1/0.170 ms =~5.8 Hz, 1/0.090 ms =~10.8 Hz). To identify the regions involved in emotional discrimination, we subtracted the brain activity induced by the rapid presentation of six emotional expressions of the activity induced by the presentation of the same emotion (reduced by neural adaptation). The results were compared to the hemisphere in which attention was sought, emotion and frequency of stimulation.

Results: The signal-to-noise ratio of the cerebral oscillations referring to the treatment of the expression of fear was stronger in the regions specific to the emotional treatment when they were presented in the subjects peripheral vision, unbeknownst to them. In addition, the peripheral emotional treatment of fear at 10.8 Hz was associated with greater activation within the Gamma 1 and 2 frequency bands in the expected regions (frontotemporal and T6), as well as desynchronization in the Alpha frequency bands for the temporal regions. This modulation of the spectral power is independent of the attentional request.

Conclusions: These results suggest that the emotional stimulation of fear presented in the peripheral vision and outside the attentional framework elicit an increase in brain activity, especially in the temporal lobe. The localization of this activity as well as the optimal stimulation frequency found for this facial expression suggests that it is treated by the fast pathway of the magnocellular layers.