Skip to main content
Log in

The CT and MR evaluation of migrational disorders of the brain

Part I. Lissencephaly and pachygyria

  • Originals
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

The migrational disorders are a rare group of congenital malformations of the brain. They consist of the following entities — lissencephaly (agyriapachygyria), pachygyria, schizencephaly, heterotopia and polymicrogyria. We studied 40 children with migrational disorders radiologically with CT and MR. This article (part I) deals with our patients with lissencephaly and pachygyria. It emphasizes their characteristic CT and MR findings along with their clinical presentation and course. These patients presented with one or a combination of the following symptoms, hypotonia, seizures, failure to thrive, microcephaly and occasionally hydrocephalus. These two groups of migrational disorders have abnormalities affecting the gyral-sulcal pattern of the cortex and gray-white matter distribution of the brain. MR provided better delineation of these disorders than CT. Because some forms of the migrational disorders can be inherited, it is extremely important for the radiologist to understand the characteristic findings for correct diagnosis which is essential for parental counseling.

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. DeMeyer W (1971) Classification of cerebral malformations. Birth Defects 8: 78

    Google Scholar 

  2. Stewart RM, Richman DP, Caviness VS Jr (1974) Lissencephaly and pachygyria. Acta Neuropathol (Berl) 31: 1

    Google Scholar 

  3. Hanaway J, Lee SS, Netsky MG (1968) Pachygyria: relation of findings to modern embryologic concepts. Neurology 18: 791

    CAS  PubMed  Google Scholar 

  4. Sellinger K, Rett S (1976) Agyria — pachygyria. Neuropadiatrie 7: 66

    Google Scholar 

  5. Schenk VWD (1966) Quantitative analysis in a case of lissencephalia. Psychiatr Neurol Neurchir 69: 297

    CAS  Google Scholar 

  6. Yakovlev PS, Wadsworth RC (1946) A study of the congenital clefts in the cerebral mantle. I. Clefts with fused lips. J Neuropathol Exp Neur 5: 116

    Google Scholar 

  7. Yakovlev PS, Wadsworth RC (1946) A study of the congenital clefts in the cerebral Mantle. II. Clefts with hydrocephalus and lips separated. J Neuropathol Exp Neur 5: 169

    Google Scholar 

  8. Barth PG (1987) Disorders of neuronal migration. Can J Neurol Sci 14: 1

    CAS  PubMed  Google Scholar 

  9. Dobyns WB, McCluggage SW (1985) Computed tomographic appearance of lissencephaly syndrome. Am J Neuroradiol 6: 545

    CAS  PubMed  Google Scholar 

  10. Zimmerman RA, Bilaniuk LT, Grossman RS (1983) Computed tomography in migratory disorders of human brain development. Neuroradiology 25: 257

    Article  CAS  PubMed  Google Scholar 

  11. Dobyns WB, Stratton RF, Greenberg F (1984) Syndromes with lissencephaly: 1. Miller-Dieker and Norman-Roberts syndrome and isolated lissencephaly. Am J Med Genet 48: 509

    Google Scholar 

  12. Dobyns WB, Stratton RF, Parke JT, Greenberg F, Nussbaum RL, Ledbetter DH (1983) Miller-Dieker syndrome: lissencephaly and monosomy y17p. J Pediatr, p 552

  13. Dobyns WB, Kirkpatrick JB, Hittner HM, Roberts RM, Kretzer FL (1985) Syndrome with lissencephaly: Walker-Warburg and cerebro-ocular muscular syndrome with a new syndrome with type II lissencephaly. Am J Med Genet 22: 157

    CAS  PubMed  Google Scholar 

  14. Dobyns WB, Gilbert EF, Opitz JM (1985) Further comments on the lissencephaly syndrome. Am J Med Gen 22: 197

    CAS  Google Scholar 

  15. Dobyns WB (1987) Developmental aspects of lissencephaly and the lissencephaly syndromes. Birth Defects 23: 225

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Byrd, S.E., Osborn, R.E., Bohan, T.P. et al. The CT and MR evaluation of migrational disorders of the brain. Pediatr Radiol 19, 151–156 (1989). https://doi.org/10.1007/BF02388643

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02388643

Keywords

Navigation