Öz
Bilateral paralysis of facial, pharyngeal, lingual, and masticatory muscles with automatic-voluntary dissociation characterizes Foix-Chavany-Marie syndrome (FCMS). The syndrome is a rare neurological disorder which usually develops secondary to cerebrovascular accidents affecting bilateral opercular regions, typically as a result of subsequent strokes. A 75-year-old woman presented with acute onset of inability to speak and swallow. Neurological examination demonstrated bilateral palsy of lower motor cranial nerves with preservation of reflex and automatic movements. Magnetic resonance images of the brain showed an acute right frontal opercular infarction and old subcortical lacunar infarctions in the bilateral hemispheres. Although FCMS occurs due to bilateral opercular lesions, rarely unilateral opercular lesion accompanied by contralateral subcortical lesions may cause the typical clinical features of the syndrome.
Anahtar Kelimeler
Opercular syndrome Foix-Chavany-Marie syndrome Pseudobulbar palsy
Kaynakça
- Neau JP, Bogousslavsky J. Superficial middle cerebral artery syndromes. In: Bogousslavsky J, Caplan L (Eds). Stroke syndromes. 2nd ed. Cambridge, Cambridge University Press; 2001; pp 405-7.
- 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; 240:199-208.
- Foix C, Chavany JA, Marie J. Diplegie facio-linguo-masticatrice d‟origine cortico-sous-cortical sans paralysie des membres. Rev Neurol 1926;33:214-9.
- Magnus A. Fall von Aufhebung des Willenseinflusses auf einige Hirnnerven. Müllers Arch Anat Physiol Wissensch Med 1837;258-66.
- Bakar M, Kirshner HS, Niaz F. The opercular-subopercular syndrome: four cases with review of the literature. Behav Neurol 1998; 11: 97-103.
- Christen HJ, Hanefeld F, Kruse E, Imhäuser 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.
- Cosnett JE, Moodley M, Bill PL, Bullock R. Operculum syndrome from brain abscess in a left-hander. J Neurol Neurosurg Psychiatry 1988; 51: 307-8.
- Starkstein SE, Berthier M, Leiguarda R. Bilateral opercular syndrome and crossed aphemia due to a right insular lesion: a clinicopathological study. Brain Lang 1988; 34: 253-61.
- Graff-Radford NR, Bosch EP, Stears JC, Tranel D. Developmental Foix- Chavany-Marie syndrome in identical twins. Ann Neurol 1986; 20: 632-5.
- 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 1996; 144:44-58.
- Grassi MP, Borella M, Clerici F, Perin C, Bini MT, Mangoni A. Reversible bilateral toxoplasmosis. Ital J Neurol Sci 1994; 15:115-7. syndrome secondary to
- AIDS-associated cerebral
- Koeda T, Takeshita K, Kisa T. Bilateral opercular syndrome: an unusual complication of perinatal difficulties. Brain Dev 1995; 17: 193-5.
- 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 Psychiatr Neurol Sci 1989; 239: 188-93.
- 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; 155: 387-90.
- Moodley M, Bamber S. The operculum syndrome: an unusual complication of tuberculous meningitis. Dev Med Child Neurol 1990; 32: 919-22.
- Pender MP, Ferguson SM. Dysarthria and dysphagia due to the opercular syndrome in multiple sclerosis. Mult Scler 2007; 13: 817-9.
- Dejong RN. DeJong‟s The Neurological Examination, 6th ed. Philadelphia, Pa: Lippincott Williams and Wilkins; 2005: pp 208-26.
- Posteraro L, Pezzoni F, Varalda E, Fugazza G, Mazzucchi A. A case of unilateral opercular syndrome associated with a subcortical lesion. J Neurol 1991; 238: 337-9.
- Kutluay E, Colakoglu Z, Dirlik A, Kumral K. Brain SPECT in anterior opercular syndrome due to a unilateral lesion. J Neurol 1996; 243:427-9.
- Code C. Opercular syndrome. In: McNeil MR (Eds). Clinical management of sensorimotor speech disorders. New York, Thieme Medical Publishers; 2009; pp 357-8.
- Baijens LW, Speyer R, Roodenburg N, Manni JJ. The effects of neuromuscular electrical stimulation for dysphagia in opercular syndrome: a case study. Eur Arch Otorhinolaryngol 2008; 265: 825-30.
Öz
Fasiyal, farengeal, lingual ve mastikatör kasların otomatik-volanter disosiasyon ile birlikte bilateral paralizisi Foix-Chavany-Marie sendromunu (FCMS) karakterize eder. Sendrom, tipik olarak bilateral operküler bölgeleri etkileyen ardışık serebrovasküler olaylara ikincil gelişir. 75 yaşında kadın hasta akut başlangıçlı konuşma ve yutma güçlüğü nedeniyle başvurdu. Nörolojik muayenede refleks ve otomatik hareketlerin korunması ile birlikte alt kraniyal motor sinirlerin bilateral paralizisi saptandı. Beyin manyetik rezonans görüntüleme, sağ frontal operküler bölgede akut enfarkt alanı ile birlikte bilateral hemisferlerde eski subkortikal laküner enfarktlar olduğuna işaret etti. FCMS‟nun bilateral operküler lezyonlara bağlı oluşmasına karşın, nadiren kontrlateral subkortikal laküner enfarktlarla birlikte unilateral operküler bir lezyon da sendromun tipik klinik özelliklerine neden olabilir
Anahtar Kelimeler
kler: Operküler sendrom Foix-Chavany-Marie sendromu Psödobulber paralizi
Kaynakça
- Neau JP, Bogousslavsky J. Superficial middle cerebral artery syndromes. In: Bogousslavsky J, Caplan L (Eds). Stroke syndromes. 2nd ed. Cambridge, Cambridge University Press; 2001; pp 405-7.
- 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; 240:199-208.
- Foix C, Chavany JA, Marie J. Diplegie facio-linguo-masticatrice d‟origine cortico-sous-cortical sans paralysie des membres. Rev Neurol 1926;33:214-9.
- Magnus A. Fall von Aufhebung des Willenseinflusses auf einige Hirnnerven. Müllers Arch Anat Physiol Wissensch Med 1837;258-66.
- Bakar M, Kirshner HS, Niaz F. The opercular-subopercular syndrome: four cases with review of the literature. Behav Neurol 1998; 11: 97-103.
- Christen HJ, Hanefeld F, Kruse E, Imhäuser 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.
- Cosnett JE, Moodley M, Bill PL, Bullock R. Operculum syndrome from brain abscess in a left-hander. J Neurol Neurosurg Psychiatry 1988; 51: 307-8.
- Starkstein SE, Berthier M, Leiguarda R. Bilateral opercular syndrome and crossed aphemia due to a right insular lesion: a clinicopathological study. Brain Lang 1988; 34: 253-61.
- Graff-Radford NR, Bosch EP, Stears JC, Tranel D. Developmental Foix- Chavany-Marie syndrome in identical twins. Ann Neurol 1986; 20: 632-5.
- 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 1996; 144:44-58.
- Grassi MP, Borella M, Clerici F, Perin C, Bini MT, Mangoni A. Reversible bilateral toxoplasmosis. Ital J Neurol Sci 1994; 15:115-7. syndrome secondary to
- AIDS-associated cerebral
- Koeda T, Takeshita K, Kisa T. Bilateral opercular syndrome: an unusual complication of perinatal difficulties. Brain Dev 1995; 17: 193-5.
- 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 Psychiatr Neurol Sci 1989; 239: 188-93.
- 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; 155: 387-90.
- Moodley M, Bamber S. The operculum syndrome: an unusual complication of tuberculous meningitis. Dev Med Child Neurol 1990; 32: 919-22.
- Pender MP, Ferguson SM. Dysarthria and dysphagia due to the opercular syndrome in multiple sclerosis. Mult Scler 2007; 13: 817-9.
- Dejong RN. DeJong‟s The Neurological Examination, 6th ed. Philadelphia, Pa: Lippincott Williams and Wilkins; 2005: pp 208-26.
- Posteraro L, Pezzoni F, Varalda E, Fugazza G, Mazzucchi A. A case of unilateral opercular syndrome associated with a subcortical lesion. J Neurol 1991; 238: 337-9.
- Kutluay E, Colakoglu Z, Dirlik A, Kumral K. Brain SPECT in anterior opercular syndrome due to a unilateral lesion. J Neurol 1996; 243:427-9.
- Code C. Opercular syndrome. In: McNeil MR (Eds). Clinical management of sensorimotor speech disorders. New York, Thieme Medical Publishers; 2009; pp 357-8.
- Baijens LW, Speyer R, Roodenburg N, Manni JJ. The effects of neuromuscular electrical stimulation for dysphagia in opercular syndrome: a case study. Eur Arch Otorhinolaryngol 2008; 265: 825-30.
Ayrıntılar
| Birincil Dil | İngilizce |
|---|---|
| Konular | Sağlık Kurumları Yönetimi |
| Bölüm | Olgu Sunumları |
| Yazarlar | |
| Yayımlanma Tarihi | 11 Haziran 2010 |
| Yayımlandığı Sayı | Yıl 2010Cilt: 32 Sayı: 2 |