Síndrome biopercular producido por lesión unilateral

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.

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HIV associated Foix-Chavany-Marie syndrome

Shetty

D’sa

Jacob

et al. 2013

Int Jour of Biomed Res

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Foix Chavany Marie Syndrome also known as Opercular syndrome is characterised by loss of voluntary control of facial, pharyngeal, lingual & masticatory muscles with preserved reflexive and automatic emotional functions. It commonly presents as biopercular syndrome although unilateral cases are rarely reported. Its association with HIV is rare and we present a 34 year old HIV affected woman who presented with acute onset of dysarthria and dysphagia. MR Imaging of the brain revealed the characteristic high signal intensities in the left opercular area near the insula. Clinical improvement was documented following antiplatelet therapy.

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Síndrome biopercular producido por lesión unilateral

Cited by 3 publications

References 17 publications

Clinical Reasoning: An 11-year-old boy with language disorder and epilepsy

Clinical Reasoning: An 11-year-old boy with language disorder and epilepsy

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

HIV associated Foix-Chavany-Marie syndrome

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