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

Case report

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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.

Abbreviations used in this paper:AF = arcuate fasciculus; DTI = diffusion tensor imaging; FAT = frontal aslant tract; FCMS = Foix-Chavany-Marie syndrome; fMRI = functional MRI; SLF = superior longitudinal fasciculus; SMA = supplementary motor area.
Article Information

Contributor Notes

Address correspondence to: Juan Martino, M.D., Ph.D., Department of Neurosurgery, Hospital Universitario Marqués de Valdecilla and Instituto de Formación e Investigación Marqués de Valdecilla (IFIMAV), Av de Valdecilla s/n, 39008, Pabellón 19 bajos, Santander, Cantabria, Spain. email: juan.martino@hotmail.com.Please include this information when citing this paper: published online September 7, 2012; DOI: 10.3171/2012.7.JNS12404.
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References
  • 1

    Augustine JR: Circuitry and functional aspects of the insular lobe in primates including humans. Brain Res Brain Res Rev 22:2292441996

    • Search Google Scholar
    • Export Citation
  • 2

    Bakar MKirshner HSNiaz F: The opercular-subopercular syndrome: four cases with review of the literature. Behav Neurol 11:971031998

    • Search Google Scholar
    • Export Citation
  • 3

    Catani MDell'acqua FVergani FMalik FHodge HRoy P: Short frontal lobe connections of the human brain. Cortex 48:2732912012

    • Search Google Scholar
    • Export Citation
  • 4

    Catani MJones DKffytche DH: Perisylvian language networks of the human brain. Ann Neurol 57:8162005

  • 5

    Christen HJHanefeld FKruse EImhäuser SErnst JPFinkenstaedt M: Foix-Chavany-Marie (anterior operculum) syndrome in childhood: a reappraisal of Worster-Drought syndrome. Dev Med Child Neurol 42:1221322000

    • Search Google Scholar
    • Export Citation
  • 6

    Cosnett JEMoodley MBill PLBullock R: Operculum syndrome from brain abscess in a left-hander. J Neurol Neurosurg Psychiatry 51:3073081988

    • Search Google Scholar
    • Export Citation
  • 7

    Duffau H: Introduction. Surgery of gliomas in eloquent areas: from brain hodotopy and plasticity to functional neurooncology. Neurosurg Focus 28:2Introduction2010

    • Search Google Scholar
    • Export Citation
  • 8

    Duffau H: A personal consecutive series of surgically treated 51 cases of insular WHO Grade II glioma: advances and limitations. Clinical article. J Neurosurg 110:6967082009

    • Search Google Scholar
    • Export Citation
  • 9

    Duffau HGatignol PMandonnet EPeruzzi PTzourio-Mazoyer NCapelle L: New insights into the anatomo-functional connectivity of the semantic system: a study using cortico-subcortical electrostimulations. Brain 128:7978102005

    • Search Google Scholar
    • Export Citation
  • 10

    Duffau HKarachi CGatignol PCapelle L: Transient Foix-Chavany-Marie syndrome after surgical resection of a right insulo-opercular low-grade glioma: case report. Neurosurgery 53:4264312003

    • Search Google Scholar
    • Export Citation
  • 11

    Duffau HLopes MArthuis FBitar ASichez JPVan Effenterre R: Contribution of intraoperative electrical stimulations in surgery of low grade gliomas: a comparative study between two series without (1985–96) and with (1996–2003) functional mapping in the same institution. J Neurol Neurosurg Psychiatry 76:8458512005

    • Search Google Scholar
    • Export Citation
  • 12

    Duffau HMoritz-Gasser SGatignol P: Functional outcome after language mapping for insular World Health Organization Grade II gliomas in the dominant hemisphere: experience with 24 patients. Neurosurg Focus 27:2E72009

    • Search Google Scholar
    • Export Citation
  • 13

    Duffau HGatignol PMandonnet ECapelle LTaillandier L: Intraoperative subcortical stimulation mapping of language pathways in a consecutive series of 115 patients with Grade II glioma in the left dominant hemisphere. J Neurosurg 109:4614712008

    • Search Google Scholar
    • Export Citation
  • 14

    Fernández-Miranda JCRhoton AL JrKakizawa YChoi CAlvarez-Linera J: The claustrum and its projection system in the human brain: a microsurgical and tractographic anatomical study. J Neurosurg 108:7647742008

    • Search Google Scholar
    • Export Citation
  • 15

    Foix CChavany JAMarie J: Diplégie facio-linguo-masticatrice d'origine cortico-sous-corticale sans paralysie des membres. Rev Neurol (Paris) 33:2142191926

    • Search Google Scholar
    • Export Citation
  • 16

    Fusco LVigevano F: Reversible operculum syndrome caused by progressive epilepsia partialis continua in a child with left hemimegalencephaly. J Neurol Neurosurg Psychiatry 54:5565581991

    • Search Google Scholar
    • Export Citation
  • 17

    Giraldo-Chica MMRamírez JDUribe CLopera F: [Biopercular syndrome caused by unilateral ischemia. Report of one case.]. Rev Med Chil 138:3413452010. (Span)

    • Search Google Scholar
    • Export Citation
  • 18

    Hamdy SAziz QRothwell JCPower MSingh KDNicholson DA: Recovery of swallowing after dysphagic stroke relates to functional reorganization in the intact motor cortex. Gastroenterology 115:110411121998

    • Search Google Scholar
    • Export Citation
  • 19

    Hamdy SMikulis DJCrawley AXue SLau HHenry S: Cortical activation during human volitional swallowing: an event-related fMRI study. Am J Physiol 277:G219G2251999

    • Search Google Scholar
    • Export Citation
  • 20

    Iannetti PRaucci UBasile LASpalice AParisi PFariello G: Benign epilepsy of childhood with centrotemporal spikes and unilateral developmental opercular dysplasia. Childs Nerv Syst 10:2642691994

    • Search Google Scholar
    • Export Citation
  • 21

    Krainik ALehéricy SDuffau HVlaicu MPoupon FCapelle L: Role of the supplementary motor area in motor deficit following medial frontal lobe surgery. Neurology 57:8718782001

    • Search Google Scholar
    • Export Citation
  • 22

    Magnus A: Fall von Aufhebung des Willenseinflusses auf einige Hirnnerven. Arch Anat Physiol Wiss Med 2582661837

  • 23

    Martin REGoodyear BGGati JSMenon RS: Cerebral cortical representation of automatic and volitional swallowing in humans. J Neurophysiol 85:9389502001

    • Search Google Scholar
    • Export Citation
  • 24

    Martino JDe Witt Hamer PCBerger MSLawton MTArnold CMde Lucas EM: Analysis of the subcomponents and cortical terminations of the perisylvian superior longitudinal fasciculus: a fiber dissection and DTI tractography study. Brain Struct Funct [epub ahead of print]2012

    • Search Google Scholar
    • Export Citation
  • 25

    Martino JTaillandier LMoritz-Gasser SGatignol PDuffau H: Re-operation is a safe and effective therapeutic strategy in recurrent WHO grade II gliomas within eloquent areas. Acta Neurochir (Wien) 151:4274362009

    • Search Google Scholar
    • Export Citation
  • 26

    Martino JVergani FRobles SGDuffau H: New insights into the anatomic dissection of the temporal stem with special emphasis on the inferior fronto-occipital fasciculus: implications in surgical approach to left mesiotemporal and temporoinsular structures. Neurosurgery 66:3 Suppl Operative4122010

    • Search Google Scholar
    • Export Citation
  • 27

    Moragas Garrido MCardona Portela PMartínez-Yélamos SRubio Borrego F: [Heterogeneous topography of Foix-Chavany-Marie syndrome.]. Neurologia 22:3333362007. (Span)

    • Search Google Scholar
    • Export Citation
  • 28

    Peck KKBranski RCLazarus CCody VKraus DHaupage S: Cortical activation during swallowing rehabilitation maneuvers: a functional MRI study of healthy controls. Laryngoscope 120:215321592010

    • Search Google Scholar
    • Export Citation
  • 29

    Peraud AMeschede MEisner WIlmberger JReulen HJ: Surgical resection of grade II astrocytomas in the superior frontal gyrus. Neurosurgery 50:9669772002

    • Search Google Scholar
    • Export Citation
  • 30

    Schmahmann JDPandya DNWang RDai GD'Arceuil HEde Crespigny AJ: Association fibre pathways of the brain: parallel observations from diffusion spectrum imaging and autoradiography. Brain 130:6306532007

    • Search Google Scholar
    • Export Citation
  • 31

    Starkstein SEBerthier MLeiguarda R: Bilateral opercular syndrome and crossed aphemia due to a right insular lesion: a clinicopathological study. Brain Lang 34:2532611988

    • Search Google Scholar
    • Export Citation
  • 32

    Szabo KGass ARossmanith CHirsch JGHennerici MG: Diffusion- and perfusion-weighted MRI demonstrates synergistic lesions in acute ischemic Foix-Chavany-Marie syndrome. J Neurol 249:173517372002

    • Search Google Scholar
    • Export Citation
  • 33

    Thiebaut de Schotten MFfytche DHBizzi ADell'Acqua FAllin MWalshe M: Atlasing location, asymmetry and inter-subject variability of white matter tracts in the human brain with MR diffusion tractography. Neuroimage 54:49592011

    • Search Google Scholar
    • Export Citation
  • 34

    Vassal MLe Bars EMoritz-Gasser SMenjot NDuffau H: Crossed aphasia elicited by intraoperative cortical and subcortical stimulation in awake patients. Clinical article. J Neurosurg 113:125112582010

    • Search Google Scholar
    • Export Citation
  • 35

    Weller M: Anterior opercular cortex lesions cause dissociated lower cranial nerve palsies and anarthria but no aphasia: Foix-Chavany-Marie syndrome and “automatic voluntary dissociation” revisited. J Neurol 240:1992081993

    • Search Google Scholar
    • Export Citation
  • 36

    Williams PLWarwick RDyson MBannister LH: Gray's Anatomy ed 37New YorkChurchill Livingstone1989

  • 37

    Yordanova YNMoritz-Gasser SDuffau H: Awake surgery for WHO Grade II gliomas within “noneloquent” areas in the left dominant hemisphere: toward a “supratotal” resection. Clinical article. J Neurosurg 115:2322392011

    • Search Google Scholar
    • Export Citation
  • 38

    Zentner JHufnagel APechstein UWolf HKSchramm J: Functional results after resective procedures involving the supplementary motor area. J Neurosurg 85:5425491996

    • Search Google Scholar
    • Export Citation
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