[Functional neurology of blepharospasm]

Benign essential blepharospasm is characterized by abnormal repetitive movements of lid closure and spasm of the orbiculari oculi muscles. Modern theories postulate that this movement disorder originates by abnormal processing of afferent information with further disintegration of the sensorimotor neural program at central levels of the nervous system all of which is seen as dystonic movements in genetically susceptible people. Different investigations including neuroimagin, genetic and neurophysiological studies have discovered new findings on what structures are involved and how this abnormal movement is generated. Among these research is noteworthy the study of electrically elicited blink reflex. It consists of three responses called non-nociceptive (R1), nociceptive (R2) and ultranociceptive (R3). Such blink reflexes, mostly the ultranociceptive response (R3), seem to be very useful to understand more deeply the pathophysiology of this focal dystonia, to perform the functional endophenotyping and to do a more appropriate follow-up of this complex neurological problem.