Foix-Chavany-Marie Syndrome [Neurosurgery Wiki]

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====== Foix-Chavany-Marie Syndrome ======

//J.Sales-Llopis, P. Gonzalez Lopez//

//Neurosurgery Service, Alicante University General Hospital, Alicante Institute for Health and
Biomedical Research (ISABIAL - FISABIO Foundation), Alicante, Spain.//


Foix-Chavany-Marie [[Syndrome]] (FCMS) or [[bilateral opercular syndrome]] is characterised by faciopharyngoglossomasticatory [[diplegia]] with automatic voluntary dissociation and is a rare form of [[pseudobulbar palsy]].

The first case was reported in [[1837]] by Magnus. The syndrome was described by Foix Chavany et Marie in [[1926]]
((Foix C, Chavany JA, Marie J (1926) Diplégie facio-linguomasticatrice
d’origine souscorticale sans paralysie des membres
(contribution à l’étude de la localisation des centres de la face du
membre supérieur). Rev Neurol 33:214–219))
, and called SFMC by Weller (1993). His literature review of 62 SFMC allowed the differentiation of five clinical types: the classical and most common form associated with cerebrovascular disease, a subacute form caused by central nervous system infections, a developmental form, a reversible form in children with epilepsy and a rare type associated with neurodegenerative disorders
((Laurent-Vannier A, Fadda G, Laigle P, Dusser A, Leroy-Malherbe V.
[Foix-Chavany-Marie syndrome in a child caused by a head trauma]. Rev Neurol
(Paris). 1999 May;155(5):387-90. Review. French. PubMed PMID: 10427603.
)).
===== Etiology =====
It most commonly occurs secondary to bilateral opercular stroke, but other cases reported in the literature
include unilateral opercular contusions following traumatic brain injury.

FCMS may also arise from defects existing at birth that may be inherited or nonhereditary. 

----
A [[transopercular approach]] to insuloopercular gliomas can generate [[Foix-Chavany-Marie syndrome]] (FCMS), especially in cases of previous contralateral lesions
((Martino J, de Lucas EM, Ibáñez-Plágaro FJ, Valle-Folgueral JM,
Vázquez-Barquero A. Foix-Chavany-Marie syndrome caused by a disconnection between
the right pars opercularis of the inferior frontal gyrus and the supplementary
motor area. J Neurosurg. 2012 Nov;117(5):844-50. doi: 10.3171/2012.7.JNS12404.
Epub 2012 Sep 7. PubMed PMID: 22957529.
)).

===== Clinical features =====

Anarthria and bilateral central facio-linguovelo-pharyngeo-masticatory paralysis with "automatic voluntary dissociation" are the clinical hallmarks of Foix-Chavany-Marie syndrome (FCMS), the corticosubcortical type of suprabulbar palsy. 

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===== Differential diagnosis =====
FCMS has many parallels with the much more commonly encountered [[supplementary motor area syndrome]] (SMAS).
SMAS is characterised by a loss of volitional movement contralateral to the site of injury (usually surgical) in the posterior medial frontal lobe immediately anterior to the primary motor cortex. It is easily recognised clinically by the profound contralateral plegia with maintenance of reflex movements
((Potgieser AR, de Jong BM, Wagemakers M, Hoving EW, Groen RJ. Insights from the
supplementary motor area syndrome in balancing movement initiation and
inhibition. Front Hum Neurosci. 2014 Nov 28;8:960. doi: 10.3389/fnhum.2014.00960.
eCollection 2014. Erratum in: Front Hum Neurosci. 2015;9:19. PubMed PMID:
25506324; PubMed Central PMCID: PMC4246659.
)).

===== Outcome =====

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
((Martino J, de Lucas EM, Ibáñez-Plágaro FJ, Valle-Folgueral JM,
Vázquez-Barquero A. Foix-Chavany-Marie syndrome caused by a disconnection between
the right pars opercularis of the inferior frontal gyrus and the supplementary
motor area. J Neurosurg. 2012 Nov;117(5):844-50. doi: 10.3171/2012.7.JNS12404.
Epub 2012 Sep 7. PubMed PMID: 22957529.
)).

In others, little improvement is seen over longer time frames
((Laurent-Vannier A, Fadda G, Laigle P, Dusser A, Leroy-Malherbe
V (1999) Foix-Chavany-Marie syndrome in a child caused by a
head trauma. Rev Neurol (Paris) 155(5):387–390))
((Nowak DA, Griebl G, Dabitz R, Ochs G (2010) Bilateral anterior
opercular (Foix-Chavany-Marie) syndrome. J Clin Neurosci 17:
1441–1442)).

===== Reviews =====
A literature review of 62 FCMS reports allowed the differentiation of five clinical types of FCMS: (1) the classical and most common form associated with cerebrovascular disease, (2) a subacute form caused by central nervous system infections, (3) a developmental form probably most often related to neuronal migration disorders, (4) a reversible form in children with epilepsy, and (e) a rare type associated with neurodegenerative disorders. Bilateral opercular lesions were confirmed in 31 of 41 patients who had CT or MRI performed, and by necropsy in 7 of 10 patients. FCMS could be attributed to unilateral lesions in 2 patients. The typical presentation and differential diagnosis of FCMS provide important clues to lesion localization in clinical neurology. FCMS is a paretic and not an apraxic disorder and is not characterized by language disturbances. Its clinical features prove divergent corticobulbar pathways for voluntary and automatic motor control of craniofacial muscles. Precise clinico-neuroradiological correlations should facilitate the identification of the structural substrate of "automatic voluntary dissociation" in FCMS
((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. 1993;240(4):199-208.
Review. PubMed PMID: 7684439.
)).
===== Case reports =====

[[Foix-Chavany-Marie Syndrome case reports]].


===== References =====