Orofacial reflex myocloni. Definition, relation to epilepsy syndromes, nosological and prognosis significance. A focused review

PURPOSE There is increasing awareness that reflex epileptic mechanisms provide unique insight into ictogenesis in human epilepsies. Among the complex triggers of seizures, this review considers orofacial reflex myocloni (ORM) from the aspects of history and delineation, clinical and electroencephalographic presentation, syndromatic relations, prevalence, mechanisms of ictogenesis and nosological implications, treatment and prognosis. METHODS We reviewed all published articles and case reports on ORM in order to clarify clinical and electroencephalographic findings, treatment and outcome. RESULTS ORM, besides Reading Epilepsy (RE), is closely related to idiopathic generalized epilepsies especially Juvenile Myoclonic Epilepsy (JME) where hyperexcitability of the network supporting linguistic communication seems to provide the precondition for eliciting reflex myocloni in the perioral muscles active in the precipitating task. CONCLUSION The conclusions on ictogenesis derived from ORM support the concept of both, RE and JME, as system disorders of the brain.