CASE REPORTS
TRANSIENT FOIX-CHAVANY-MARIE SYNDROME AFTER
SURGICAL RESECTION OF A RIGHT INSULO-OPERCULAR
LOW-GRADE GLIOMA: CASE REPORT
Hugues Duffau, M.D.,
Ph.D.
Department of Neurosurgery,
Hôpital de la Salpêtrière, Paris,
France
Carine Karachi, M.D.
Department of Neurosurgery,
Hôpital de la Salpêtrière, Paris,
France
Peggy Gatignol, S.T.
Department of Neurology, Hôpital
de la Salpêtrière, Paris, France
Laurent Capelle, M.D.
Department of Neurosurgery,
Hôpital de la Salpêtrière, Paris,
France
Reprint requests:
Hugues Duffau, M.D., Ph.D.,
Department of Neurosurgery,
Hôpital de la Salpêtrière, 47-83
Boulevard de l’Hôpital, 75651
Paris Cedex 13, France.
Email:
[email protected]
Received, January 28, 2003.
Accepted, March 19, 2003.
OBJECTIVE AND IMPORTANCE: We describe an atypical case of transient FoixChavany-Marie syndrome, or faciopharyngoglossomasticatory diplegia with automatic
voluntary dissociation, occurring after surgical resection of a right insulo-opercular glioma.
CLINICAL PRESENTATION: A 26-year-old right-handed man experienced partial
seizures that were poorly controlled by antiepileptic drugs during a 2-year period as a
result of a right insulo-opercular low-grade glioma, leading to the proposal of surgical
resection. In addition, 1 year before the operation, the patient experienced a severe
brain injury that resulted in a coma. A computed tomographic scan revealed left
opercular contusion. The patient recovered completely within 6 months.
INTERVENTION: Intraoperative corticosubcortical electrical functional mapping was
performed along the resection, allowing the identification and preservation of the
facial and upper limb motor structures. A subtotal removal of the glioma was achieved.
The patient had postoperative anarthria, with loss of voluntary muscular functions of
the face and tongue, and he had trouble chewing and swallowing. All of these
symptoms resolved within 3 months.
CONCLUSION: These findings provide insight into the use of surgery to treat a right
insulo-opercular tumor. First, surgeons must be particularly cautious in cases with a
potential contralateral lesion (e.g., history of head injury), even if such a lesion is not
visible on magnetic resonance imaging scans; preoperative metabolic imaging and
electrophysiological investigations should be considered before an operative decision
is made. Second, surgeons must perform intraoperative functional mapping to identify
and to attempt to preserve the corticosubcortical facial motor structures. A procedure
performed while the patient is awake should be discussed to detect the structures
involved in chewing and swallowing in cases of suspected bilateral lesions. Third, the
patient must be informed of this particular risk before surgery is performed.
KEY WORDS: Foix-Chavany-Marie syndrome, Insulo-opercular tumor, Intraoperative functional mapping,
Low-grade glioma
Neurosurgery 53:426-431, 2003
F
oix-Chavany-Marie syndrome (FCMS), or faciopharyngoglossomasticatory diplegia with automatic voluntary
dissociation (e.g., preserved automatic, involuntary,
emotional innervation), has been considered to be a corticosubcortical type of suprabulbar palsy (2, 19, 35, 53). Symptoms
include anarthria, loss of voluntary muscular functions of the
face and tongue, and problems with chewing and swallowing.
This exceptional entity, classically attributed to bilateral lesions of the frontal operculum, most often occurs after multiple strokes (5, 18, 33, 36, 37, 44, 45, 48, 51); it is more rarely
reported after infections (9, 22, 39, 46), developmental abnor-
426 | VOLUME 53 | NUMBER 2 | AUGUST 2003
DOI: 10.1227/01.NEU.0000073990.94180.54
www.neurosurgery-online.com
malities related to neuronal migration disorders (3, 7), status
epilepticus in children (8, 21, 26), neurodegenerative disorders
(6, 30), and head injury (31). Recovery is generally poor (53).
We report an original case of FCMS. The case was atypical
for the following reasons: 1) it was generated by the surgical
resection of a low-grade glioma; 2) it was the result of a
unilateral right insulo-opercular lesion; and 3) the patient
experienced favorable functional outcome within 3 months.
We discuss the pathophysiology of FCMS on the basis of these
findings, and we consider the implications for surgery of the
insulo-opercular region.
www.neurosurgery-online.com
POSTOPERATIVE FOIX-CHAVANY-MARIE SYNDROME
CASE REPORT
Patient Presentation
A 26-year-old right-handed man presented with partial seizures in September 2000; examination revealed a right insuloopercular tumor. At that time, a stereotactic biopsy was performed, which permitted a diagnosis of low-grade
oligodendroglioma. No surgery was proposed to the patient.
Despite the use of antiepileptic drugs (gabapentin and valproate, then gabapentin and carbamazepine), the patient still
experienced approximately two partial seizures per week,
during which his speech was transitorily interrupted and he
drooled.
In June 2001, the patient experienced a severe head injury
and coma, which necessitated his admission to our department. A computed tomographic scan revealed a diffuse subarachnoid hemorrhage associated with a mild left central contusion (symmetrical to the tumor). Artificial ventilation with
tracheostomy was necessary for 15 days. One month later, the
patient’s neurological status had dramatically improved, with
normal consciousness and the beginning of ability to walk, but
right brachiofacial paresis and cognitive disorders persisted.
Neurological rehabilitation allowed a complete functional recovery within 6 months. A new magnetic resonance imaging
(MRI) scan performed at that time and 6 months later revealed
no residual abnormality in the left hemisphere (as assessed by
T1-weighted images with and without gadolinium, T2weighted images, and fluid-attenuated inversion recovery images). Because the seizures were not being controlled, and
because the volume of the right glioma increased slightly (50
ml on the preoperative MRI scan), with invasion of the whole
insula and the frontal operculum (Fig. 1), it was decided to
surgically resect the tumor in August 2002, 2 years after the
tumor was diagnosed. On the preoperative Edinburgh inventory (40), the patient scored ⫹100 (right-handedness).
FIGURE 1. Preoperative coronal (A and C) and axial (B and D)
T1-weighted, gadolinium-enhanced MRI scans revealing a right insuloopercular low-grade glioma. The neurological examination was normal.
NEUROSURGERY
Intervention
The resection was conducted with the patient under general
anesthesia. We used a method of intraoperative electrical motor mapping that we reported previously (12, 14) (Fig. 2). After
identification of the tumor boundaries by intraoperative ultrasonography, direct cortical stimulations were performed, allowing the detection of the primary motor sites of the superior
limb and face (5-mm spaced-tip bipolar electrode, biphasic
current with pulse duration of 1 ms, frequency of 60 Hz,
intensity of 8 mA [Ojemann cortical stimulator; Radionics,
Burlington, MA]). Because the patient was intubated, it was
impossible to note whether involuntary swallowing was elicited by stimulation.
The frontal operculum was resected. The sylvian fissure was
opened laterally by using functional limits posteriorly provided by subcortical stimulation, which identified the pyramidal pathways of the face and the superior limb within the
corona radiata. Once the insular surface was exposed, cortical
mapping was performed at its level. No motor response was
elicited. The portion of the glioma involving the insula was
then removed, again using deep subcortical stimulations, as
previously reported (13, 14).
FIGURE 2. a, intraoperative photograph obtained before tumor removal. The
tumor boundaries identified via ultrasonography are marked by letter tags
(A–G). The primary motor cortical sites of the superior limb (1, 2) and of the face
(3) were detected via electrical mapping. b, intraoperative photograph obtained
after tumor removal. The primary motor cortical sites and their corresponding
descending corticospinal pathways (49, 50), both identified all along the resection
by use of repeated corticosubcortical electrical stimulations, represent the posterior functional boundaries of the resection. Anterior is to the left.
VOLUME 53 | NUMBER 2 | AUGUST 2003 | 427
DUFFAU
ET AL.
Postoperative Course
The patient was extubated immediately after surgery. There
was no limb deficit; however, the patient experienced mutism
and an inability to swallow. Examination revealed a bilateral
complete palsy of the lower facial muscles. The mouth remained half-open, and the patient drooled. The tongue remained immobile inside the mouth; voluntary deglutition and
chewing were impossible, so the patient was fed through a
nasogastric tube. Nevertheless, some possibility of automatic
swallowing seemed likely. Laryngoscopy revealed that the
vocal cords were not paralyzed. The patient could not utter a
sound or articulate simple words. He could not produce suction movements or throw a kiss, either on command or by
imitation. No upper facial disturbance or ophthalmoplegia
was observed; the upper facial motility was bilaterally intact.
The corneal reflex was preserved.
Over the course of 3 days, the patient had complete anarthria and could communicate only by writing. He could write
fluently, with no feature of aphasic agraphia: the phrases were
semantically and syntactically correct. There was no apraxia of
the right hand. Comprehension was normal. The patient was
examined daily by a speech therapist. Four days after surgery,
the patient began to utter a few sounds. One day later, he was
able to articulate some words, and after the seventh day, he
could speak in short sentences. Lower facial and lingual palsy
progressively recovered in 2 weeks. The nasogastric tube was
removed 3 weeks after the operation, allowing the patient to
return home with rehabilitation. Two months later, the functional status was the same as it had been preoperatively; in
particular, normalization of swallowing and speech had
occurred.
The patient experienced no seizures after the operation; he
continued therapy with the same antiepileptic drugs that had
been administered before he underwent surgery. Control MRI
scans performed 3 months after the operation revealed a subtotal resection according to the classification of Berger et al.
(4), with less than 10 ml of residue remaining (approximately
4 ml in the posterior insula) (Fig. 3). The histological examination confirmed the diagnosis of low-grade oligodendroglioma, and no radiotherapy or chemotherapy was performed.
DISCUSSION
We describe here a unique case of FCMS. To our knowledge, this is the first case of FCMS reported after brain tumor
surgery. Moreover, this syndrome occurred after a unilateral
right operculoinsular lesion, and the symptoms were
transient.
Postoperative FCMS
The seminal publications of Magnus (35) in 1837 and FoixChavany-Marie (19) in 1926 described the association of anarthria and bilateral central faciolinguovelopharyngeomasticatory paralysis with automatic-voluntary dissociation. Since
then, the etiologies detailed by Weller (53) in 1993 in an
428 | VOLUME 53 | NUMBER 2 | AUGUST 2003
FIGURE 3. Postoperative coronal (A and C) and axial (B and D)
T1-weighted, gadolinium-enhanced MRI scans revealing subtotal glioma
removal with a small residue within the posterior part of the insula. The
anterior and middle parts of the insular lobe and the whole frontal operculum were totally resected.
extensive review of the literature indicated that the causes in
approximately 63 patients were as follows: vascular (5, 18, 33,
36, 37, 44, 45, 48, 51), infectious (9, 22, 39, 46), developmental
(3, 7), epileptic (8, 21, 26), and degenerative (6, 30). On the
basis of these findings, Weller (53) proposed the differentiation of five clinical types of FCMS. More recently, LaurentVannier et al. (31) reported a case of FCMS caused by head
trauma in a child.
In our case, the FCMS was not directly induced by the
glioma; there was no preoperative deficit despite the progressive tumor growth. This could be explained by brain plasticity
mechanisms, widely described in low-grade gliomas (11, 16,
47, 49, 54). Nevertheless, the patient had a transient speech
arrest and drooled during partial seizures—perhaps an equivalent of an FCMS-like disorder despite the impossibility of
better detailing the patient’s neurological status during these
phases because the patient completely recovered in 1 minute,
which did not permit time for examination. FCMS occurred
immediately after the tumor resection; thus, it was likely the
direct result of the surgical act itself. Glioma surgery is known
to potentially induce specific syndromes postoperatively, such
as the supplementary motor area syndrome after resection of
frontomesial structures (20, 28) or athymhormic syndrome
after removal of paralimbic gliomas involving the right striatum (15). However, to our knowledge, no FCMS has been
reported after resection of an operculoinsular tumor.
FCMS Caused by Unilateral Right
Operculoinsular Lesion
Classically, FCMS is caused by bilateral cortical or subcortical damage involving the frontal operculum with or without
involvement of the insula (2, 19, 53). There are only five
reports of FCMS in patients with seemingly unilateral brain
www.neurosurgery-online.com
POSTOPERATIVE FOIX-CHAVANY-MARIE SYNDROME
lesions (9, 21, 26, 35, 48), among them two putative (structural
or functional) lesions in three cases (9, 21, 26).
In our patient, FCMS occurred after the resection of a glioma
involving the whole right insula and the right frontal operculum, without any cortical or subcortical contralateral damage,
as revealed by multiple preoperative MRI scans (in particular,
sensitive fluid-attenuated inversion recovery images). This is a
surprising result because we had never before observed such
a postoperative deficit in our experience of more than 40
procedures involving insular-paralimbic-opercular gliomas
(11, 13, 14), nor had such a deficit ever been described in the
surgical literature of this kind of tumor (29, 52, 55, 57).
On the other hand, direct electrical stimulation revealed that
both the frontal operculum and the right (anterior) insula were
involved in swallowing (41, 42). Transcranial magnetic stimulations (24) and functional neuroimaging methods (25, 38, 56)
provide data that converge with reports of dysphagia after
unilateral anterior insular lesions (10) or opercular lesions (43).
Moreover, these two structures are also known to be implicated in orofacial movements and chewing (1, 41, 42, 50), as
emphasized by Starkstein et al. (48), who reported a case of
bilateral opercular syndrome and crossed aphemia that was
the result of a right insular lesion (i.e., aphemia induced by a
lesion of the right non-language-dominant hemisphere in a
right-handed patient).
Therefore, taking into account these data, we might rather
ask the question: why is there usually no FCMS after resection
of an operculoinsular tumor? It was previously suggested that
the contralateral hemisphere could compensate for the facial
motor cortex (32) and the insula (11); thus, one explanation
might be that not only the slow-growing, low-grade glioma
but also the surgical act itself generates functional reshaping
(16)—namely, the recruitment of the contralateral homologous
area (23), as already indicated after supplementary motor area
resection via pre- and postoperative functional MRI scans (27).
In the present case, the only difference in comparison to the
other patients who underwent surgery for an insulo-opercular
tumor is the fact that the patient previously sustained a brain
injury with left opercular damage. Thus, even if the MRI scan
revealed nothing abnormal 1 year after the head trauma, we
can hypothesize that the possible persistence of a left (insulo)opercular hypometabolism (as demonstrated in the literature
that describes findings revealed via single-photon emission
computed tomography [51]) prevented an efficient compensatory recruitment of these areas, leading to the occurrence of an
FCMS. However, no metabolic study was performed in our
patient to permit us to confirm this hypothesis. The hypothesis
of a prolonged postoperative epilepsy seems less likely for
two reasons: 1) the symptoms slowly regressed over the
course of several weeks (without fluctuation and then sudden
arrest [7], and/or with shorter duration [26]); and 2) the patient’s electroencephalogram revealed nothing abnormal.
Transient FCMS
One classic feature of FCMS is a poor recovery, except in the
particular case of status epilepticus in children (53). One expla-
NEUROSURGERY
nation of the favorable functional outcome in our case could be
the delayed recruitment of the contralateral hemisphere, previously suggested in swallowing recovery after stroke (23), by the
lifting of inhibition via the transcallosal pathways (34), even if the
functional compensation was not possible immediately after surgery for the reasons considered above (as in the supplementary
motor area syndrome, lasting usually 10–20 d [17]). Moreover,
the use of intraoperative corticosubcortical stimulations allowed
the identification and subsequent preservation of the essential
structures for the movements of the face (but not for the movements of the tongue or for deglutition—i.e., no detection of the
whole corticonuclear pathways). Therefore, it seems likely that
these facial motor areas participate, at least partially, in the functional recovery after a transient postoperative stage of “sideration,” which is classically observed after a corticosubcortical
glioma resection limited by functional boundaries (12). According to this hypothesis, a combined resection of the insular lobe,
frontal operculum, and primary motor area of the face should be
carefully discussed individually on the basis of a rigorous functional preoperative assessment to try to find the areas in the
ipsilateral and contralateral hemispheres that are still potentially
dysfunctional (as a result of tumor or a previous injury) and
areas able potentially to compensate for the function, so that
postoperative defects can be predicted in cases of bilaterally
symmetrical lesions.
CONCLUSION
This study indicates the possibility of generating an FCMS
after the resection of a right insulo-opercular glioma in a righthanded patient. These findings suggest the following: 1) surgeons should be particularly cautious in the case of a potential
contralateral lesion (e.g., history of head injury), even if such a
lesion is not visible on anatomic images. Preoperative metabolical imaging and electrophysiological investigations should be
considered to make the operative decision; 2) intraoperative
functional mapping should be performed to identify and eventually preserve in selected patients the corticosubcortical primary
motor structures of the face (when the insula is also invaded),
even in the nondominant hemisphere—in other words, a combined resection of the insular lobe, frontal operculum, and primary motor area of the face might be avoided if contralateral
compensation is not possible. Moreover, a procedure performed
while the patient is awake should be discussed to detect the
structures involved in chewing and swallowing in cases of suspected (anatomic, electrophysiological, or metabolic) bilateral lesions; and 3) the patient should be informed of this particular risk
before the surgical procedure is performed.
REFERENCES
1. Augustine JR: Circuitry and functional aspects of the insular lobe in primates including humans. Brain Res Brain Res Rev 22:229–244, 1996.
2. Bakar M, Kirshner HS, Niaz F: The opercular-subopercular syndrome: Four
cases with review of the literature. Behav Neurol 11:97–103, 1998.
3. Becker PS: Developmental Foix-Chavany-Marie syndrome: Polymicrogyria
or macrogyria? Ann Neurol 27:693–694, 1990.
VOLUME 53 | NUMBER 2 | AUGUST 2003 | 429
DUFFAU
ET AL.
4. Berger MS, Deliganis AV, Dobbins JD, Keles GE: The effect of extent of
resection on recurrence in patients with low-grade cerebral hemisphere
gliomas. Cancer 74:1784–1791, 1994.
5. Besson G, Bogousslavsky J, Regli F, Maeder P: Acute pseudobulbar or
suprabulbar palsy. Arch Neurol 48:501–507, 1991.
6. Broussolle E, Bakchine S, Tommasi M, Laurent B, Bazin B, Cinotti L, Cohen
L, Chazot G: Slowly progressive anarthria with late anterior opercular
syndrome: A variant form of frontal cortical atrophy syndromes. J Neurol
Sci 144:44–58, 1996.
7. Christen HJ, Hanefeld F, Kruse E, Imhauser S, Ernst JP, Finkenstaedt M: FoixChavany-Marie (anterior operculum) syndrome in childhood: A reappraisal of
Worster-Drought syndrome. Dev Med Child Neurol 42:122–132, 2000.
8. Colamaria V, Sgro R, Caraballo R, Simeone M, Zullini E, Fontana E, Zanetti
R, Grimeau-Merino R, Dalla Bernardina B: Status epilepticus in benign
rolandic epilepsy manifesting as anterior operculum syndrome. Epilepsia
32:329–334, 1991.
9. Cosnett JE, Moodley M, Bill PLA, Bullock R: Operculum syndrome from brain
abscess in a left-hander. J Neurol Neurosurg Psychiatry 51:307–308, 1988.
10. Daniels SK, Foundas AL: The role of the insular cortex in dysphagia.
Dysphagia 12:146–156, 1997.
11. Duffau H, Bauchet L, Lehéricy S, Capelle L: Functional compensation of the
left insula dominant for language. Neuroreport 12:2159–2163, 2001.
12. Duffau H, Capelle L, Denvil D, Sichez N, Gatignol P, Taillandier L, Lopes M,
Mitchell MC, Roche S, Muller JC, Bitar A, Sichez JP, Van Effenterre R:
Usefulness of intraoperative electrical sub-cortical mapping in surgery of
low-grade gliomas located within eloquent regions: Functional results in a
consecutive series of 103 patients. J Neurosurg 98:764–778, 2003.
13. Duffau H, Capelle L, Lopes M, Bitar A, Sichez JP, Van Effenterre R: Medically intractable epilepsy from insular low-grade gliomas: Improvement
after an extended lesionectomy. Acta Neurochir (Wien) 144:563–573, 2002.
14. Duffau H, Capelle L, Lopes M, Faillot T, Sichez JP, Fohanno D: The insular lobe:
Physiopathological and surgical considerations. Neurosurgery 47:801–811, 2000.
15. Duffau H, Denvil D, Capelle L: Absence of movements disorders after surgical
resection of right striatum invaded by glioma. J Neurosurg 97:363–369, 2002.
16. Duffau H, Denvil D, Capelle L: Long term reshaping of language, sensory
and motor maps following glioma resection: A new parameter to integrate
in the surgical strategy. J Neurol Neurosurg Psychiatry 72:511–516, 2002.
17. Duffau H, Lopes M, Denvil D, Capelle L: Delayed onset of supplementary
motor area syndrome after surgical resection of the mesial frontal lobe: A
time course study using intraoperative mapping on awake patient.
Stereotact Funct Neurosurg 76:74–82, 2002.
18. Ferrari G, Boninsegna C, Beltramello A: Foix-Chavany-Marie syndrome: CT
study and clinical report of three cases. Neuroradiology 18:41–42, 1979.
19. Foix C, Chavany JA, Marie J: Diplégie facio-linguo-masticatrice d’origine
cortico-sous-corticale sans paralysie des membres. Rev Neurol (Paris) 33:
214–219, 1926.
20. Fontaine D, Capelle L, Duffau H: The somatotopy of supplementary motor
area: Evidence by correlation between the extent of surgical resection and
the clinical patterns of deficit. Neurosurgery 50:297–305, 2002.
21. Fusco L, Vigevano F: Reversible operculum syndrome caused by progressive epilepsia partialis continua in a child with left hemimegalencephaly.
J Neurol Neurosurg Psychiatry 54:556–558, 1991.
22. Grattan-Smith PJ, Hopkins IJ, Shield LK, Boldt DW: Acute pseudobulbar
palsy due to bilateral focal cortical damage: The opercular syndrome of
Foix-Chavany-Marie. J Child Neurol 4:131–136, 1989.
23. Hamdy S, Aziz Q, Rothwell JC, Power M, Singh KD, Nicholson DA, Tallis
RC, Thompson DG: Recovery of swallowing after dysphagic stroke relates
to functional reorganization in the intact motor cortex. Gastroenterology
115:1104–1112, 1998.
24. Hamdy S, Aziz Q, Rothwell JC, Singh KD, Barlow J, Hughes DG, Tallis RC,
Thompson DG: The cortical topography of human swallowing musculature
in health and disease. Nat Med 2:1217–1224, 1996.
25. Hamdy S, Rothwell JC, Brooks DJ, Bailey D, Aziz Q, Thompson DG: Identification of cerebral loci procession human swallowing with H215O PET
activation. J Neurophysiol 81:1917–1926, 1999.
26. Ianetti P, Raucci U, Basile LA, Spalice A, Parisi P, Fariello G, Imperato G:
Benign epilepsy of childhood with centrotemporal spikes and unilateral
developmental opercular dysplasia. Childs Nerv Syst 10:264–269, 1994.
430 | VOLUME 53 | NUMBER 2 | AUGUST 2003
27. Krainik A, Lehéricy S, Duffau H, Cornu P, Capelle L, Menu Y, Le Bihan D,
Marsault C: Functional recovery following lesion of the supplementary
motor area: A fMRI study. Neuroimage 13:6(S1206), 2001 (abstr).
28. Krainik A, Lehéricy S, Duffau H, Vlaicu M, Poupon F, Capelle L, Cornu P,
Clémenceau S, Sahel M, Valéry CA, Boch AL, Mangin JF, Le Bihan D,
Marsault C: The role of the supplementary area in motor deficit following
medial frontal lobe surgery. Neurology 57:871–878, 2001.
29. Lang FF, Olansen NE, DeMonte F, Gokaslan ZL, Holland EC, Kalhorn C,
Sayawa R: Surgical resection of intrinsic insular tumors: Complication
avoidance. J Neurosurg 95:638–650, 2001.
30. Lang C, Reichwein J, Iro H, Treig T: Foix-Chavany-Marie syndrome: Neurological, neuropsychological, CT, MRI, and SPECT findings in a case progressive
for more than 10 years. Eur Arch Psychiatry Neurol Sci 239:188–193, 1989.
31. Laurent-Vannier A, Fadda G, Laigle P, Dusser A, Leroy-Malherbe V: FoixChavany-Marie syndrome in a child caused by a head trauma [in French].
Rev Neurol (Paris) 155:387–390, 1999.
32. LeRoux PD, Berger MS, Haglund MM, Pilcher WH, Ojemann GA: Resection
of intrinsic tumors from nondominant face motor cortex using stimulation
mapping: Report of two cases. Surg Neurol 36:44–48, 1991.
33. Levine DN, Mohr JP: Language after bilateral cerebral infarctions: Role of
the minor hemisphere in speech. Neurology 29:927–938, 1979.
34. Liepert J, Hamzei F, Weiller C: Motor cortex disinhibition of the unaffected
hemisphere after acute stroke. Muscle Nerve 23:1761–1763, 2000.
35. Magnus A: Fall von Aufhebung des Willenseinflusses auf einige Hirnnerven. Arch Anat Physiol Wiss Med 258–266, 1837.
36. Mao CC, Coull BM, Golper LAC, Rau MT: Anterior operculum syndrome.
Neurology 39:1169–1172, 1989.
37. Mariani C, Spinnler H, Sterzi R, Vallar G: Bilateral perisylvian softenings:
Bilateral anterior opercular syndrome (Foix-Chavany-Marie syndrome).
J Neurol 223:269–284, 1980.
38. Martin RE, Goodyear BG, Gati JS, Menon RS: Cerebral cortical representation of automatic and volitional swallowing in humans. J Neurophysiol
85:938–950, 2001.
39. Moodley M, Bambar S: The operculum syndrome: An unusual complication
of tuberculous meningitis. Dev Child Neurol 32:919–922, 1990.
40. Oldfield RC: The assessment and analysis of handedness: The Edinburgh
Inventory. Neuropsychologia 9:97–113, 1971.
41. Penfield W, Boldery E: Somatic motor and sensory representation in the cerebral cortex of man as studied by electrical stimulation. Brain 60:389–443, 1937.
42. Penfield W, Faulk ME: The insula: Further observations on its function.
Brain 78:445–470, 1955.
43. Robbins J, Levine RL, Maser A, Rosenbek JC, Kempster GB: Swallowing
after unilateral stroke of the cerebral cortex. Arch Phys Med Rehabil 74:
1295–1300, 1993.
44. Sandyk R, Brennan MJW: The operculum syndrome. J Comput Assist
Tomogr 7:130–131, 1983.
45. Santos S, Casadevall T, Rios C, Lopez-Garcia E, Tejero C, Garces-Redondo
M, Fabre O, Mostacero E: Opercular syndrome of vascular aetiology. Rev
Neurol 34:1129–1132, 2002.
46. Sasaguri H, Sodeyama N, Maejima Y, Kanda T, Mizusawa H: Slowly progressive Foix-Chavany-Maris syndrome associated with chronic herpes simplex encephalitis. J Neurol Neurosurg Psychiatry 73:203–204, 2002.
47. Seitz RJ, Huang Y, Knorr U, Tellmann L, Herzog HJ: Large-scale plasticity of
the human motor cortex: Large-scale plasticity of the human motor cortex.
Neuroreport 6:742–744, 1995.
48. Starkstein SE, Berthier M, Leiguarda R: Bilateral opercular syndrome and
crossed aphemia due to a right insular lesions: A clinicopathological study.
Brain Lang 34:253–261, 1998.
49. Thiel A, Herholz K, Koyuncu A, Ghaemi M, Kracht LW, Habedank B, Heiss
WD: Plasticity of language networks in patients with brain tumors: A positron
emission tomography activation study. Ann Neurol 50:620–629, 2001.
50. Tognola G, Vignolo LA: Brain lesions associated with oral apraxia in stroke
patients: A clinico-neuroradiological investigation with the CT scan.
Neuropsychologia 18:257–272, 1980.
51. Tohgi H, Sano M, Takahashi S, Chiba K, Matsuoka S: Bilateral anterior
opercular syndrome: Localizing value of SPECT and MRI. Neuroradiology
30:579–581, 1988.
www.neurosurgery-online.com
POSTOPERATIVE FOIX-CHAVANY-MARIE SYNDROME
52. Vanaclocha V, Saiz-Sapena N, Garcia-Casasola C: Surgical treatment of
insular gliomas. Acta Neurochir (Wien) 139:1126–1135, 1997.
53. 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 240:199–208, 1993.
54. Wunderlich G, Knorr U, Herzog H, Kiwit JC, Freund HJ, Seitz RJ: Precentral
glioma location determines the displacement of cortical hand representation. Neurosurgery 42:18–27, 1998.
55. Yaşargil MG, von Ammon K, Cavazos E, Doczi T, Reeves JD, Roth P:
Tumours of the limbic and paralimbic systems. Acta Neurochir (Wien)
118:40–52, 1992.
56. Zald DH, Pardo JVP: The functional neuroanatomy of voluntary swallowing. Ann Neurol 46:281–286, 1999.
57. Zentner J, Meyer B, Stangi A, Schramm J: Intrinsic tumors of the insula: A
prospective surgical study of 30 patients. J Neurosurg 85:263–271, 1996.
COMMENTS
T
his article contributes to our knowledge of complication
avoidance in operculoinsular surgery. The techniques of
intraoperative functional mapping that use subcortical stimulation, as the authors describe, are particularly useful.
Robert G. Grossman
Houston, Texas
D
D
uffau et al. present an interesting case of an extremely
unusual neurological syndrome that normally occurs in a
setting other than that associated with a tumor resection. The
patient had bilateral dysfunction of the face, pharynx, tongue,
and muscles of mastication, and he was unable to speak. He had
a history of an injury to the dominant left operculum after
trauma. The resection performed by the authors included the
nondominant operculum, resulting in this transient symptom
complex, even though no permanent injury involving the dominant hemisphere was observed on the control magnetic resonance imaging scan.
Surgeons should thus be aware of situations in which a contralateral brain injury is a component of the patient’s history and
is symmetrical to the site of the proposed resection. This will
allow the surgeon to consult with the family and predict the
possibility of a transient neurological dysfunction that otherwise
would not be expected to be present postoperatively. In settings
like this, where there is previous traumatic brain injury, functional magnetic resonance imaging assessing subcortical white
matter pathways (i.e., diffusion tensor imaging) or a metabolic
imaging study such as positron emission tomography may indicate disrupted pathways or regions of hypometabolism that may
predict dysfunction in the setting of a contralateral resection. This
might alert the surgeon to a transient postoperative syndrome
that could be discussed with the family before the surgery. Thus,
this is a useful case report for all surgeons who do cortical- and
subcortical-based resections.
uffau et al. report an unusual neurological course after
subtotal resection of a low-grade oligodendroglioma involving the right frontal operculum and insula. In their recognition from classical neurology of the bilateral pathological
substrate underlying Foix-Chavany-Marie syndrome, the authors almost certainly are correct in invoking the earlier leftsided traumatic injury. It is surprising that as sensitive a
sequence as fluid-attenuated inversion recovery failed to reveal any residual injury, but the severity of the injury and the
associated brachial-facial deficit at the time of that injury lend
credence to the presumed contralateral disturbance.
What remains most provocative is the complete and fairly
rapid recovery from the surgically induced syndrome, when
attribution of its occurrence to a contralateral abnormality ought
to preclude the contribution of that disturbed dominant hemisphere to the recovery. Recovery of preserved but transiently
dysfunctional primary motor cortex must underlie this restoration of function. Functional magnetic resonance imaging might
have assessed this better than intraoperative mapping.
his report serves as a reminder of the potential for FoixChavany-Marie syndrome in tumor surgery. The majority
of reports concerning this syndrome indicate that bilateral
lesions must be present. Accordingly, the previous head injury
in this patient must have provided the substrate for the emergence of the neurological problems after nondominant opercular surgery. Regardless, the authors illustrate the usefulness
of intraoperative mapping well; this likely facilitated recovery.
Foix-Chavany-Marie syndrome is a rare entity, and this report
is, to my knowledge, the first to report it after tumor surgery.
David W. Roberts
Lebanon, New Hampshire
Joseph M. Piepmeier
New Haven, Connecticut
Mitchel S. Berger
San Francisco, California
T
Erratum
In a comment by Laligam N. Sekhar and Dinko Stimac regarding an article by Mir Jafer
Ali et al., titled “Arterial Reconstruction by Direct Surgical Clipping of a Basilar Artery
Dissecting Aneurysm after Failed Vertebral Artery Occlusion: Technical Case Report
and Literature Review” (Neurosurgery 52:1475–1481, 2003), the phrase “posterior communicating artery” in the second paragraph should have read “posterior cerebral
artery.” The publisher regrets the error.
NEUROSURGERY
VOLUME 53 | NUMBER 2 | AUGUST 2003 | 431