The Congenital Bilateral Peri-Sylvian Syndrome

Imaging Findings
  • R. Kuzniecky
  • F. Andermann
  • R. Guerrini
  • The CBPS Multicenter Collaborative Study
Part of the NATO ASI Series book series (NSSA, volume 264)


Advances in diagnostic imaging techniques, particularly magnetic resonance (MR) imaging, have permitted the recognition of developmental malformations of the central nervous system with a high degree of accuracy (Barth, 1987; Friede, 1989; Byrd et al., 1989; Barkovich and Kjos, 1992). Recent clinico-radiological correlative studies have demonstrated the broad spectrum of these malformations and have contributed to the recognition that some of these disorders have common pathogenic mechanisms (Graff-Radford et al., 1986; Kuzniecky et al., 1988; Palmini et al, 1991; Barkovich and Kjos, 1992a,b).


Full Scale Intelligence Quotient Magnetic Resonance Examination Supramarginal Gyrus Inversion Recovery Sequence Insular Region 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Barkovich, A., and Kjos, B., 1992a, Nonlissencephalic cortical dysplasias: Correlation of imaging findings with clinical deficits, AJNR 13: 85–103.PubMedGoogle Scholar
  2. Barkovich, A., and Kjos, B., 1992b, Schizencephaly: Correlation of clinical findings with MR characteristics, AJNR 13: 85–94.PubMedGoogle Scholar
  3. Barth, P., 1987, Disorders of neuronal migration, Can J Neurol Sci. 14: 1–16.PubMedGoogle Scholar
  4. Barth, P., and van der Harten, J., 1985, Parabiotic twin syndrome with topical isocortical disruption and gastroschisis, Acta Neuropathol. 67: 345–349.PubMedCrossRefGoogle Scholar
  5. Benton, A., and Van Allen, M., 1968, Impairment in facial recognition in patients with cerebral disease, Cortex 4: 344–358.Google Scholar
  6. Bordarier, C., Robain, O., and Ponsot, G., 1991, Bilateral poroencephalic defect in the newborn after injection of Benzol during pregnancy, Brain Dev. 13: 126–129.PubMedCrossRefGoogle Scholar
  7. Byrd, S., Osborn, R., Bohan, T. and Naidich, T., 1989, The CT and MR evaluation of migrational disorders of the brain. Part II. Schizencephaly, heterotopia and polymicrogyria, Pediatr Radiol. 19: 219–222.PubMedCrossRefGoogle Scholar
  8. Dvorák, K., Feit, J., and Juránková, Z., 1978, Experimentally induced focal microgyria and status verrucosis deformis in rats: Pathogenesis and interrelation, histological study, Acta Neuropathol. 44: 121–129.PubMedCrossRefGoogle Scholar
  9. Foix, C., Chavany, J., and Marie, J., 1926, Diplegie facio-linguo-masticatrice d’origine cortico-sous-cortical sans paralysie des membres, Rev Neurol. 33: 214–219.Google Scholar
  10. Friede, R., 1989, Dysplasias of the cerebral cortex, in: “Developmental Pathology,” R. Friede, ed., Springer-Verlag, Vienna.Google Scholar
  11. Graff-Radford, N., Bosh, E., Stears, J. and Tranel, D., 1986, Developmental Foix-Chavany-Marie syndrome in identical twins, Ann Neurol. 20: 632–635.PubMedCrossRefGoogle Scholar
  12. Hamilton, W., Boyd, J., and Mossman, H., 1962, Human embryology, Williams and Wilkins, Baltimore, 350.Google Scholar
  13. Humphreys, P., Rosen, G., Press, D., Sherman, G., and Galaburda, M., 1991, Freezing lesions of the developing rat brain: A model for cerebrocortical microgyria. J Neuropathol Exp Neurol. 50: 145–160.PubMedCrossRefGoogle Scholar
  14. Kuzniecky, R., Berkovic, S., Andermann, F., Melanson, D., Olivier, A., and Robitaille, Y., 1988, Focal cortical myoclonus and rolandic cortical dysplasia: Clarification by MRI, Ann Neurol. 23: 317–325.PubMedCrossRefGoogle Scholar
  15. Kuzniecky, R., Andermann, F., Tampieri, D., Melanson, D., Olivier, A., and Leppik, T., 1989, Bilateral central macrogyria: Epilepsy, pseudobulbar palsy and mental retardation—A recognizable neuronal migration disorder, Ann Neurol. 25: 547–554.PubMedCrossRefGoogle Scholar
  16. Leiter, G., 1980, Leiter International Performance Scale (Revised), Stoeling, Chicago.Google Scholar
  17. Levine, D., Fisher, M., and Caviness, V., 1974, Porencephaly with microgyria: a pathologic study, Acta Neuropathol (Berl)., 99–113.Google Scholar
  18. Mariani, C., Spinnler, H., Sterzi, R., and Vallar, G., 1980, Bilateral peri-sylvian softenings: Bilateral anterior opercular syndrome (Foix-Chavany-Marie syndrome), J Neurol. 223: 269–284.PubMedCrossRefGoogle Scholar
  19. Norman, R., Urich, H. and Woods, G., 1958, The relationship between prenatal porencephaly and the encephalomacias of early life, J Ment Sci. 104: 758–771.PubMedGoogle Scholar
  20. Palmini, A., Andermann, F., Olivier, A., Tampieri, D., and Melanson, D., 1991, Focal neuronal migration disorders and intractable epilepsy: A study of 30 patients, Ann Neurol. 30: 741–749.PubMedCrossRefGoogle Scholar
  21. Wechsler, D., 1981, “Wechsler Adult Intelligence Scale (Revised),” Psychological Corporation, New York.Google Scholar
  22. Williams, R., and Caviness, V., 1976, The cellular pathology of microgyria. A Golgi analysis, Acta Neuropathol. 36: 269–283.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1994

Authors and Affiliations

  • R. Kuzniecky
    • 1
  • F. Andermann
    • 1
  • R. Guerrini
    • 1
  • The CBPS Multicenter Collaborative Study
  1. 1.UAB Epilepsy Center Department of NeurologyUniversity of Alabama at BirminghamBirminghamUSA

Personalised recommendations