Abstract
We here report a case of monozygotic female twins, only one of whom had congenital bilateral perisylvian syndrome (CBPS).
The affected girl had all of the essential and some additional diagnostic criteria according to Kuzniecky et al. [1993]; her twin sister was completely normal both clinically and neuroradiologically.
This observation may contribute towards our understanding of the etiopathogenetic mechanisms of cortical dysplasia in CBPS: fetal brain ischemia or hypoxia in only one twin seems to be a reasonable explanation in this particular case.
Sommario
Viene qui riportato il caso di due gemelle monozigoti, di cui una sola affetta da “sindrome congenita perisilviana bilaterale”.
La ragazza affetta aveva tutti i caratteri clinici e neuroradiologici specific della sindrome, mentre la sorella era clinicamente normale.
La presente osservazione ha permesso di escludere alcune delle ipotesi eziopatogenetiche formulate per spiegare la displasia corticale nella sindrome in oggetto (congenite, tossiche o infettive) e di valorizzare invece, come più probabile, l'ipotesi di una ischemia (ipossia) cerebrale fetale in uno solo dei gemelli.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Barth P.:Disorders of neuronal migration. Can. J. Neurol. Sci., 14: 1–16, 1987.
Becker S., Dixon A., Troncoso J.:Bilateral opercular polymicrogyria. Ann. Neurol., 25: 90–92, 1989.
Foix C., Chavany J., Marie J.:Diplegie facio-linguomasticatrice d'origine cortico - sous - corticale sans paralysie des membres. Rev. Neurol., 33: 214–219, 1926.
Friede R.L.:Developmental neuropathology. 2nd Ed., Berlin: Springer-Verlag, 156–168, 1989.
Graff-Radford N., Bosh E., Stears J., Tranel D.:Developmental Foix-Chavany-Marie syndrome in identifical twins. Ann. Neurol., 20: 632–635, 1976.
Kuzniecky R., Andermann F., Guerrini R. andThe CBPS Multicenter Collaborative Study:Congenital bilateral perisylvian syndrome: study of 31 patients. Lancet, 341: 608–612, 1993.
Kuzniecky R.:Magnetic resonance imaging in developmental disorders of the cerebral cortex. Epilepsia, 35 (Suppl. 6): S44-S56, 1994 a.
Kuzniecky R., Andermann F., Guerrini R. andThe CBPS Multicenter Collaboration Study:The epileptic spectrum in the congenital bilateral perisylvian syndrome. Neurology, 44: 379–385, 1994 b.
Kuzniecky R., Gilliam F., Faught E.:Discordant occurrence of cerebral unilateral heterotopia and epilepsy in monozygotic twins. Epilepsia, 36: 1155–1157, 1995.
Larroche J.C.I., Drouelle P., Delezoide A.L. et al.:Brain damage in monozygous twins. Biol. Neonate, 57: 261–278, 1990.
Rosen G.D., Press D.M., Sherman G.F., Galaburda A.M.:The development of induced cerebrocortical microgyria in the rat. J. Neuropath. Exp. Neurol., 51: 601–610, 1992.
Sugama S., Kusano K.:Monozygous twin with polymicrogyria and normal co-twin. Pediatr. Neurol., 11: 62–63, 1994.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Lenti, C., Triulzi, F. Discordant clinical and neuroradiological features of congenital bilateral perisylvian syndrome in monozygotic female twins. Ital J Neuro Sci 17, 287–290 (1996). https://doi.org/10.1007/BF01997788
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01997788