Skip to main content

Advertisement

Log in

An unusual clinical presentation of bilateral schizencephaly

Un cas inhabituel de schizencéphalie bilatérale

  • Anatomic Malformation
  • Published:
Surgical and Radiologic Anatomy Aims and scope Submit manuscript

Summary

We present a case with a characteristic magnetic resonance image (MRI) of bilateral open-lipped schizencephaly and atypical clinical presentation. The patient is still alive and in good health in her forties, she has never presented seizures, and although the motor dysfunction is well correlated with cerebral lobe involvement, neurobehavioral dysfunction is not proportional to the MR image of the cerebral malformation.

Résumé

Nous présentons un cas de schizencéphalie bilatérale ouverte caractérisé par une présentation clinique atypique et une imagerie par résonance magnétique nucléaire caractéristique. La patiente est encore vivante, en bonne santé, à plus de 40 ans, elle n'a jamais présenté de crise comitiale et, bien que les troubles moteurs soient bien corrélés aux altérations cérébrales, les troubles neuro-comportementaux ne sont pas proportionnels aux images IRM de cette malformation cérébrale.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Aniskiewicz AS, Frumkin NL, Brady DE, Moore JB, Pera A (1990) Magnetic resonance imaging and neurobehavioral correlates in schizencephaly. Arch Neurol 47: 911–916

    Google Scholar 

  2. Arias A, Pereiro I, Requena I, Ventura M, Iglesias C (1994) Transtornos de la migración neuronal: manifestaciones clínicas y de neuroimagen en 21 pacientes. Neurología 9: 125–132

    Google Scholar 

  3. Barkovich AJ, Norman D (1988) MR imaging of schizencephaly. Am J Roentgenol 150: 1391–1396

    Google Scholar 

  4. Barkovich AJ, Chuang SH, Norman D (1988) MR of neuronal migration anomalies. Am J Roentgenol 150: 179–187

    Google Scholar 

  5. Barkovich AJ (1995) Pediatric neuroimaging, 2nd edn. Raven Press, New York, pp 219–225

    Google Scholar 

  6. Barkovich AJ, Kjos BO (1992) Schizencephaly: correlation of clinical findings with MR characteristics. AJNR 13: 85–94

    Google Scholar 

  7. Bird CR, Gilles FH (1987) Type I Schizencephaly: CT and neuropathologic findings. AJNR 8: 451–454

    Google Scholar 

  8. Brodtkorb E, Nilsen G, Smevik O, Rink PA (1992) Epilepsy and anomalies of neuronal migration: MRI and clinical aspects. Acta Neurol Scand 86: 24–32

    Google Scholar 

  9. Byrd SE, Osborn RE, Bohan TP, Naidich TP (1989) The CT and MR evaluation of migrational disorders of the brain. Part II. Schizencephaly, heterotopia and polymicrogyria. Pediatr Radiol 19: 219–222

    Google Scholar 

  10. Dominguez R, Aguirre A, Slopis JM, Bohan TP (1991) Brain and ocular abnormalities in infants with in utero exposure to cocaine and other street drugs. Am J Dis Child 145: 688–895

    Google Scholar 

  11. Haverkamp F, Zerres K, Ostertun B, Emons D, Lentze MJ (1995) Familial schizencephaly: further delineation of a rare disorder. J Med Genet 32: 242–244

    Google Scholar 

  12. Hosley MA, Abroms IF, Ragland RL (1992) Schizencephaly: case report of familial incidence. Pediatr Neurol 8: 148–150

    Google Scholar 

  13. Kuban KCK, Teele RL, Wallman J (1989) Septo-optic-dysplasia-schizencephaly. Pediatr Radiol 19: 145–150

    Google Scholar 

  14. Kuzniecky RI (1994) Magnetic resonance imaging in developmental disorders of the cerebral cortex. Epilepsia 35: S44-S56

    Google Scholar 

  15. Landrieu P, Lacroix C (1994) Schizencephaly, consequence of a developmental vasculopathy? Clin Neuropathol 13: 192–196

    Google Scholar 

  16. Landy HJ, Ramsay RE, Ajmone-Marsan C, Levin BE, Brown J, Pasarin G, Quencer RM (1992) Temporal lobectomy for seizures associated with unilateral schizencephaly. Surg Neurol 37: 477–481

    Google Scholar 

  17. Larsen WJ (1993) Human embryology. Churchill Livingstone, New York, pp 400–404

    Google Scholar 

  18. Osborn RE, Byrd SE, Naidich TP, Bohan TP, Friedman H (1988) MR Imaging of neuronal migrational disorders. AJNR 9: 1101–1106

    Google Scholar 

  19. Pati S, Helmbrecht GD (1994) Congenital schizencephaly associated with in utero warfarin exposure. Reprod Toxicol 8: 115–120

    Google Scholar 

  20. Silbergeld DL, Miller JW (1994) Rejective surgery for medically intractable epilepsy associated with schizencephaly. J Neurosurg 80: 820–825

    Google Scholar 

  21. Suchet IB (1994) Schizencephaly: antenatal and postnatal assessment with colour-flow doppler imaging. Can Assoc Radiol J 45: 193–200

    Google Scholar 

  22. Yakovlev PI, Wadsworth RC (1946) Schizencephaly: a study of congenital clefts of the cerebral mantle. I. Clefts with fused lips. J Neuropath Exp Neur 5: 116–130

    Google Scholar 

  23. Yakovlev PI, Wadsworth RC (1946) Schizencephalies II. Clefts with hydrocephalus and lips separated. J Neuropath Exp Neur 5: 169–206

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Avellanet, M., Mirapeix, R., Escudero, D. et al. An unusual clinical presentation of bilateral schizencephaly. Surg Radiol Anat 18, 271–273 (1996). https://doi.org/10.1007/BF01627604

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01627604

Key words

Navigation