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15 - Opercular epilepsies with oromotor dysfunction
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- By Javier Salas-Puig, Department of Neurology, Hospital General de Asturias, Oviedo, Spain, Angeles Pérez-Jiménez, Department of Neurology, Hospital General de Asturias, Oviedo, Spain, Pierre Thomas, Department of Neurology, CHU, Nice, France, Ingrid I. E. Scheffer, Department of Neurology, Austin and Repatriation Medical Centre, University of Melbourne, Heidelberg, Victoria, Australia, Bernardo Dalla Bernardina, Neuropediatric Department, Borgo Roma Hospital, Verona, Italy, Renzo Guerrini, Neurosciences Unit, Institute of Child Health, The Wolfson Centre, London, UK
- Edited by Renzo Guerrini, University of London, Jean Aicardi, Hôpital Robert-Debré, Paris, Frederick Andermann, Montreal Neurological Institute & Hospital, Mark Hallett, National Institutes of Health, Baltimore
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- Book:
- Epilepsy and Movement Disorders
- Published online:
- 03 May 2010
- Print publication:
- 13 December 2001, pp 251-268
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- Chapter
- Export citation
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Summary
Introduction
The term operculum insulae denotes the cortical region that covers the insulae Reili. It is made up of frontal, parietal, and temporal cortical convolutions. A bilateral structural or functional disturbance of the cortical motor areas of the anterior part (frontal) operculum, including the inferior rolandic area, and their corticonuclear projections to the nuclei of the 5th, 7th, 9th, and 10th cranial nerves, causes a unique clinical picture of a supranuclear (pseudobulbar) palsy. It presents with swallowing difficulties, anarthria or severe dysarthria, and loss of the ability to imitate oral gestures, resulting from a central disturbance of volitional control of the facio-linguo-glosso-pharyngo-masticatory muscles, while preserving automatic, involuntary, emotional innervation, and reflex motor activity. Pathological crying and laughing are conspicuously absent. It is also designated Foix–Chavany–Marie Syndrome (FCMS), in honour of the authors who described it in 1926, in two adults with bilateral infarction of the anterior operculum (Foix et al., 1926). Bilateral cortical impairment underlies the symptomatology of FCMS as lower cranial nerve brainstem receive innervation from both hemispheres.
The best known form of the syndrome is the classical FCMS due to serial strokes in adults with cerebrovascular disease (Bruyn & Gathier, 1969). FCMS can also occur in infancy and childhood, in relation to diverse etiologies and different clinical evolutions. It can be permanent, secondary to congenital malformation or to acquired structural lesion in early life, or can have an intermittent or reversible expression in the background of certain epileptic disorders involving the perisylvian regions (Christen et al., 2000).