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

Abstract

Foix-Chavany-Marie syndrome (FCMS) is a rare type of suprabulbar palsy characterized by an automatic-voluntary 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.