Biopercular syndrome: report of two cases and literature review
Keywords:
cerebral infarction, pseudobulbar palsy, mental retardation, deglutition disorders, language disorders
Abstract
The anterior opercular or biopercular syndrome is a cortical pseudobulbar palsy due to bilateral lesions of the anterior brain operculum. It is characterized by preservation of reflex function and automatic activity, without mental impairment. Two cases are reported herein and the relevant literature reviewed.The first case was a 73-year-old female with a history of a stroke occurring seven years previously, without sequelae in the interim. She presented with sudden loss of consciousness. The neurological examination showed a right facial central palsy and anarthria, with reflex acts such as smiling, blinking and yawning, not elicited by commands; she also had a right hemiparesis and walking impairment. A brain CT scan showed an old ischemic infarction in the region of the right medial cerebral artery. Because the right motor involvement did not correlate with the findings of the initial CT scan, another CT scan two days later showed an acute brain infarction in the vicinity of the left medial cerebral artery.
The second case was an 8-year-old girl with mental retardation and impairment of verbal development, caused by of biopercular pachygyria. Facio-pharyngo-glosso-masticatory diplegia and volitional selective palsy of the oro-facial muscles was seen in both patients. The neuropsychological assessment showed cognitive, emotional and social interaction impairment in both cases -as part of the frontal convexity syndrome in the first case and of mental retardation in the second. The two patients had difficulty in mastication and swallowing. The prognosis for recovery of verbal capacity is poor, although generally most patients recover the ability to swallow.
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References
1. Foix C, Chavany JA, Marie J. Diplégie facio-linguo-masticatrice dorigine cortico-sous-corticale sans paralysie des membres. Rev Neurol (Paris). 1926;33: 214-9.
2. De Smet Y. The anterior operculum syndrome. J Neurol. 1994;241:570-1.
3. Mao CC, Coull BM, Golper LA, Rau MT. Anterior operculum syndrome. Neurology. 1989;39:1169-72.
4. Bakar M, Kirshner HS, Niaz F. The opercular-subopercular syndrome: four cases with review of the literature. Behav Neurol. 1998;11:97-103.
5. Pineda DA, Mejía SE, Rosselli M, Ardila A, Romero MG, Pérez C. Variabilidad en la prueba de Boston para el diagnóstico de las afasias en adultos laboralmente activos. Rev Neurol. 1998;26:962-70.
6. Godefroy O. Frontal syndrome and disorders of executive functions. J Neurol. 2003;250:1-6
7. Trimble MR. Behavior and personality disturbances. En: Bradley WG, Bradley WG, Daroff RB, Fenichel GM, editors. Neurology in Clinical Practice. Boston: Butterworth-Heinemann; 1991. p. 81-100.
8. Bruyn GW, Gathier JC. The operculum syndrome. Handbook of Clinical Neurology. En: Vinken PJ, Bruyn GW, editors. Localization in clinical neurology. Vol. 2. Amsterdam: North Holland Publishing; 1969. p. 776-87.
9. Christen HJ, Hanefald F, Kruse E, Imhauser S, Ernst JP, Finkenstaedt M. Foix-Chavany-Marie (anterior operculum) syndrome in childhood: a reappraisal of Worster-Drought syndrome. Dev Med Child Neurol. 2000;42:122-32.
10. Kuzniecky R, Andermann F, Guerrini R. Congenital bilateral perisylvian syndrome: study of 31 patients. The CBPS Multicenter Collaborative Study. Lancet. 1993;341:608-12.
11. Gropman AL, Barkovich AJ, Vezina LG, Conry JA, Dubovsky EC, Packer RJ. Pediatric congenital bilateral perisylvian syndrome: clinical and MRI feature in 12 patients. Neuropediatrics. 1997;28:198-203.
12. Puertas I, García-Soldevilla M, Jiménez FJ, Cabrera F, Jabbour T, García E. Mano distónica bilateral secundaria a síndrome biopercular o síndrome de Foix-Chavany-Marie. Rev Neurol. 2002;35:430-3.
13. Bara-Jiménez W, Shelton P, Sanger TD, Hallett M. Sensory discrimination capabilities in patients with focal hand dystonia. Ann Neurol. 2000;47:377-80.
14. Santos S, Casadevall T, Ríos C, López-García E, Tejero C, Garcés-Redondo M, et al. Síndrome opercular de etiología vascular. Rev Neurol. 2002;34:1129-32.
15. Wolf RW, Schultze D, Fretz C, Weissert M, Waibel P. Atypical herpes simplex encephalitis presenting as operculum syndrome. Pediatr Radiol. 1999;29:191-3.
16. Van der Poel JC, Haenggeli CA, Overweg-Plansoen. Operculum syndrome: unusual feature of herpes simplex encephalitis. Pediatr Neurol. 1995;12:246-9.
17. Moodley M, Bamber S. The Operculum syndrome: an unusual complication of tuberculosis meningitis. Dev Med Child Neurol. 1990;32:919-22.
18. Laurent-Vannier A, Fadda G, Laigle P, Dusser A, Leroy-Malherbe V. Syndrome de Foix-Chavany-Marie dorigine traumatique. Rev Neurol (Paris). 1999; 155:387-90.
19. Biller J, Asconape J, Challa VR, Toole JF, McLean WT. A case for cerebral thromboangiitis obliterans. Stroke. 1981;12:686-9.
20. Alajouanine TH, Boudin G, Pertuiset B, Pépin B. Le syndrome unilatéral de lopercule rolandique avec atteinte contralatérale du territoire des V, VII, IX, X, XI et XII nerfs craniens. Rev Neurol (Paris). 1959;101:168-71.
21. Pascual-Castroviejo I, Pascual-Pascual SI. Síndrome opercular tras cirugía cerebral bilateral: presentación de un caso. Rev Neurol. 2004;39:624-7.
22. Broussolle E, Bakchine S, Tommasi M, Laurent B, Bazin B, Cinotti L, et al. Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes. J Neurol Sci. 1996;144:44-58.
23. Shevell MI, Carmant L, Meagher-Villemure K. Developmental bilateral perisylvian dysplasia. Pediatr Neurol. 1992;8:299-302.
24. Márquez-Belandria G, Rondón-Hernández F, Pascual-Castroviejo I. Síndrome opercular bilateral congénito: presentación de tres casos. Rev Neurol. 2003;36:938-40.
25. Gropman AL, Barkovich AJ, Vezina LG, Conry JA, Dubovsky EC, Packer RJ. Pediatric congenital bilateral perisylvian syndrome: clinical and MRI feature in 12 patients. Neuropediatrics. 1997;28:198-203.
26. Yamamoto T, Koeda T, Maegaki Y, Tanaka C, Takeshita K. Bilateral opercular syndrome caused by perinatal difficulties. Europ J Paediatr Neurol. 1997;1: 73-7.
27. Prats JM, Garaizar C, García-Nieto ML, Madoz P. El síndrome opercular epiléptico: una forma peculiar de epilepsia parcial benigna de la infancia con paroxismos rolándicos. Rev Neurol. 1999;29:375-80.
28. Colamaria V, Sgro V, Caraballo R, Simeone M, Zullini E, Fontana E, et al. Status epilepticus in benign rolandic epilepsy manifesting as anterior operculum syndrome. Epilepsia. 1991;32:329-34.
29. Pascual-Castroviejo I, Pascual-Pascual SI, Peña W, Talavera M. Status epilepticus-induced brain damage and opercular syndrome in childhood. Dev Med Child Neurol. 1999;41:420-3.
30. Besson G, Bogousslavsky J, Maeder P. Acute pseudobulbar or suprabulbar palsy. Arch Neurol. 1991;48:501-7.
31. Pineda D, Ardila A. Lasting mutism associated with buccofacial apraxia. Aphasiol. 1992;6:285-92.
2. De Smet Y. The anterior operculum syndrome. J Neurol. 1994;241:570-1.
3. Mao CC, Coull BM, Golper LA, Rau MT. Anterior operculum syndrome. Neurology. 1989;39:1169-72.
4. Bakar M, Kirshner HS, Niaz F. The opercular-subopercular syndrome: four cases with review of the literature. Behav Neurol. 1998;11:97-103.
5. Pineda DA, Mejía SE, Rosselli M, Ardila A, Romero MG, Pérez C. Variabilidad en la prueba de Boston para el diagnóstico de las afasias en adultos laboralmente activos. Rev Neurol. 1998;26:962-70.
6. Godefroy O. Frontal syndrome and disorders of executive functions. J Neurol. 2003;250:1-6
7. Trimble MR. Behavior and personality disturbances. En: Bradley WG, Bradley WG, Daroff RB, Fenichel GM, editors. Neurology in Clinical Practice. Boston: Butterworth-Heinemann; 1991. p. 81-100.
8. Bruyn GW, Gathier JC. The operculum syndrome. Handbook of Clinical Neurology. En: Vinken PJ, Bruyn GW, editors. Localization in clinical neurology. Vol. 2. Amsterdam: North Holland Publishing; 1969. p. 776-87.
9. Christen HJ, Hanefald F, Kruse E, Imhauser S, Ernst JP, Finkenstaedt M. Foix-Chavany-Marie (anterior operculum) syndrome in childhood: a reappraisal of Worster-Drought syndrome. Dev Med Child Neurol. 2000;42:122-32.
10. Kuzniecky R, Andermann F, Guerrini R. Congenital bilateral perisylvian syndrome: study of 31 patients. The CBPS Multicenter Collaborative Study. Lancet. 1993;341:608-12.
11. Gropman AL, Barkovich AJ, Vezina LG, Conry JA, Dubovsky EC, Packer RJ. Pediatric congenital bilateral perisylvian syndrome: clinical and MRI feature in 12 patients. Neuropediatrics. 1997;28:198-203.
12. Puertas I, García-Soldevilla M, Jiménez FJ, Cabrera F, Jabbour T, García E. Mano distónica bilateral secundaria a síndrome biopercular o síndrome de Foix-Chavany-Marie. Rev Neurol. 2002;35:430-3.
13. Bara-Jiménez W, Shelton P, Sanger TD, Hallett M. Sensory discrimination capabilities in patients with focal hand dystonia. Ann Neurol. 2000;47:377-80.
14. Santos S, Casadevall T, Ríos C, López-García E, Tejero C, Garcés-Redondo M, et al. Síndrome opercular de etiología vascular. Rev Neurol. 2002;34:1129-32.
15. Wolf RW, Schultze D, Fretz C, Weissert M, Waibel P. Atypical herpes simplex encephalitis presenting as operculum syndrome. Pediatr Radiol. 1999;29:191-3.
16. Van der Poel JC, Haenggeli CA, Overweg-Plansoen. Operculum syndrome: unusual feature of herpes simplex encephalitis. Pediatr Neurol. 1995;12:246-9.
17. Moodley M, Bamber S. The Operculum syndrome: an unusual complication of tuberculosis meningitis. Dev Med Child Neurol. 1990;32:919-22.
18. Laurent-Vannier A, Fadda G, Laigle P, Dusser A, Leroy-Malherbe V. Syndrome de Foix-Chavany-Marie dorigine traumatique. Rev Neurol (Paris). 1999; 155:387-90.
19. Biller J, Asconape J, Challa VR, Toole JF, McLean WT. A case for cerebral thromboangiitis obliterans. Stroke. 1981;12:686-9.
20. Alajouanine TH, Boudin G, Pertuiset B, Pépin B. Le syndrome unilatéral de lopercule rolandique avec atteinte contralatérale du territoire des V, VII, IX, X, XI et XII nerfs craniens. Rev Neurol (Paris). 1959;101:168-71.
21. Pascual-Castroviejo I, Pascual-Pascual SI. Síndrome opercular tras cirugía cerebral bilateral: presentación de un caso. Rev Neurol. 2004;39:624-7.
22. Broussolle E, Bakchine S, Tommasi M, Laurent B, Bazin B, Cinotti L, et al. Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes. J Neurol Sci. 1996;144:44-58.
23. Shevell MI, Carmant L, Meagher-Villemure K. Developmental bilateral perisylvian dysplasia. Pediatr Neurol. 1992;8:299-302.
24. Márquez-Belandria G, Rondón-Hernández F, Pascual-Castroviejo I. Síndrome opercular bilateral congénito: presentación de tres casos. Rev Neurol. 2003;36:938-40.
25. Gropman AL, Barkovich AJ, Vezina LG, Conry JA, Dubovsky EC, Packer RJ. Pediatric congenital bilateral perisylvian syndrome: clinical and MRI feature in 12 patients. Neuropediatrics. 1997;28:198-203.
26. Yamamoto T, Koeda T, Maegaki Y, Tanaka C, Takeshita K. Bilateral opercular syndrome caused by perinatal difficulties. Europ J Paediatr Neurol. 1997;1: 73-7.
27. Prats JM, Garaizar C, García-Nieto ML, Madoz P. El síndrome opercular epiléptico: una forma peculiar de epilepsia parcial benigna de la infancia con paroxismos rolándicos. Rev Neurol. 1999;29:375-80.
28. Colamaria V, Sgro V, Caraballo R, Simeone M, Zullini E, Fontana E, et al. Status epilepticus in benign rolandic epilepsy manifesting as anterior operculum syndrome. Epilepsia. 1991;32:329-34.
29. Pascual-Castroviejo I, Pascual-Pascual SI, Peña W, Talavera M. Status epilepticus-induced brain damage and opercular syndrome in childhood. Dev Med Child Neurol. 1999;41:420-3.
30. Besson G, Bogousslavsky J, Maeder P. Acute pseudobulbar or suprabulbar palsy. Arch Neurol. 1991;48:501-7.
31. Pineda D, Ardila A. Lasting mutism associated with buccofacial apraxia. Aphasiol. 1992;6:285-92.
How to Cite
1.
Uribe CS, Millán PA, Montes MI, Cabrera D, Arboleda A. Biopercular syndrome: report of two cases and literature review. biomedica [Internet]. 2008 Jun. 1 [cited 2024 May 19];28(2):183-90. Available from: https://revistabiomedica.org/index.php/biomedica/article/view/89
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