Orofacial Movement: past, present and future

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Vol. 7 No. 3 (2020): September 2020

Special Issue: Orofacial Movement and communicaton disorders Orofacial movement and communication disorders is a special edition for the dissemination of scientific knowledge and updates on the clinical approach to orofacial motor disorders (chewing, swallowing and breathing) and communication (speech, language and related areas) in speech therapy. The special issue publishes original articles, review articles and case series and case reports. The articles can be subbmited in spanish or english. All submitted material is subject to a strict peer review process.

Foix-Chavany-Marie syndrome caused by a disconnection between the right pars opercularis of the inferior frontal gyrus and the supplementary motor area

Foix-Chavany-Marie syndrome (FCMS) is a rare type of suprabulbar palsy characterized by an automaticvoluntary dissociation of the orofacial musculature. Here, the authors report an original case of FCMS that occurred intraoperatively while resecting the pars opercularis of the inferior frontal gyrus. This 25-year-old right-handed man with an incidentally diagnosed right frontotemporoinsular tumor underwent surgery using an asleep-awake-asleep technique with direct cortical and subcortical electrical stimulation and a transopercular approach to the insula. While resecting the anterior part of the pars opercularis the patient suffered sudden anarthria and bilateral facial weakness. He was unable to speak or show his teeth on command, but he was able to voluntarily move his upper and lower limbs. This syndrome lasted for 8 days. Postoperative diffusion tensor imaging tractography revealed that connections of the pars opercularis of the right inferior frontal gyrus with the frontal aslant tract (FAT) and arcuate fasciculus (AF) were damaged. This case supplies evidence for localizing the structural substrate of FCMS. It was possible, for the first time in the literature, to accurately correlate the occurrence of FCMS to the resection of connections between the FAT and AF, and the right pars opercularis of the inferior frontal gyrus. The FAT has been recently described, but it may be an important connection to mediate supplementary motor area control of orofacial movement. The present case also contributes to our knowledge of complication avoidance in operculoinsular surgery. A transopercular approach to insuloopercular gliomas can generate FCMS, especially in cases of previous contralateral lesions. The prognosis is favorable, but the patient should be informed of this particular hazard, and the surgeon should anticipate the surgical strategy in case the syndrome occurs intraoperatively in an awake patient.

Management of Blepharospasm and Hemifacial Spasm

Benign essential blepharospasm and hemifacial spasm are chronic and disabling medical conditions. Both disorders can result in uncontrollable blinking or frank spasms of the eyelids and face, which may interfere with the activities of daily living and may even render a patient functionally blind and occupationally handicapped. Often, when untreated, the eyelid and facial spasms are so emotionally unsettling that the patients become withdrawn, frustrated, and desperate. Essential blepharospasm is the most common manifestation of orofacial movement disorders. The term is used to describe a movement disorder limited to the eyelid protractors without a secondary inciting cause, and is characterized by spontaneous, repetitive, forceful eyelid closure. Benign essential blepharospasm is caused by forceful contraction of the eyelid protractors, which include the orbicularis oculi, corrugator supercilii, and procerus muscles. The prevalence is 32 in 100,000 and women are more commonly affected than men (3:2). The peak onset is in the fifth to sixth decades, and symptoms peak 3 years after onset. When contractions are limited to the orbital and periorbital muscles, the term “benign essential blepharospasm” is used. Often, subsequent contractions of lower face and neck occur concurrently. This is termed Meige syndrome, orofacial dystonia, or oromandibular dystonia (Brueghel syndrome). Furthermore, dystonia outside of the facial nerve distribution is called segmental cranial dystonia or craniocervical dystonia. Initially, benign essential blepharospasm can manifest as increased frequency in blinking in response to several stimuli, including wind, air pollution, sunlight, noise, and movements of the head or eyes. It can significantly impair quality of life by causing difficulty in reading, writing, and driving. In severe cases, patients may be functionally blind. Several eyelid changes have been noted with longstanding blepharospasm including dermatochalasis, eyelid and brow ptosis, entropion, and canthal tendon abnormalities. Common symptoms that precede the official diagnosis of benign essential blepharospasm include eye irritation, photophobia, tearing, and ocular pain. Early symptoms include an increase in blink rate (77%), lid spasms (66%), ocular irritation (55%), midfacial or lower facial spasm (59%), brow spasm (24%), and eyelid tic (22%). Several conditions relieve blepharospasm; these include sleep, relaxation, inferior gaze, artificial tears, traction on eyelids, and humming.