Advertisement

Pediatric Radiology

, Volume 19, Issue 3, pp 151–156 | Cite as

The CT and MR evaluation of migrational disorders of the brain

Part I. Lissencephaly and pachygyria
  • Sharon E. Byrd
  • R. E. Osborn
  • T. P. Bohan
  • T. P. Naidich
Originals

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.

Keywords

Public Health Hydrocephalus Correct Diagnosis Congenital Malformation Microcephaly 
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.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    DeMeyer W (1971) Classification of cerebral malformations. Birth Defects 8: 78Google Scholar
  2. 2.
    Stewart RM, Richman DP, Caviness VS Jr (1974) Lissencephaly and pachygyria. Acta Neuropathol (Berl) 31: 1Google Scholar
  3. 3.
    Hanaway J, Lee SS, Netsky MG (1968) Pachygyria: relation of findings to modern embryologic concepts. Neurology 18: 791PubMedGoogle Scholar
  4. 4.
    Sellinger K, Rett S (1976) Agyria — pachygyria. Neuropadiatrie 7: 66Google Scholar
  5. 5.
    Schenk VWD (1966) Quantitative analysis in a case of lissencephalia. Psychiatr Neurol Neurchir 69: 297Google Scholar
  6. 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: 116Google Scholar
  7. 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: 169Google Scholar
  8. 8.
    Barth PG (1987) Disorders of neuronal migration. Can J Neurol Sci 14: 1PubMedGoogle Scholar
  9. 9.
    Dobyns WB, McCluggage SW (1985) Computed tomographic appearance of lissencephaly syndrome. Am J Neuroradiol 6: 545PubMedGoogle Scholar
  10. 10.
    Zimmerman RA, Bilaniuk LT, Grossman RS (1983) Computed tomography in migratory disorders of human brain development. Neuroradiology 25: 257CrossRefPubMedGoogle Scholar
  11. 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: 509Google Scholar
  12. 12.
    Dobyns WB, Stratton RF, Parke JT, Greenberg F, Nussbaum RL, Ledbetter DH (1983) Miller-Dieker syndrome: lissencephaly and monosomy y17p. J Pediatr, p 552Google Scholar
  13. 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: 157PubMedGoogle Scholar
  14. 14.
    Dobyns WB, Gilbert EF, Opitz JM (1985) Further comments on the lissencephaly syndrome. Am J Med Gen 22: 197Google Scholar
  15. 15.
    Dobyns WB (1987) Developmental aspects of lissencephaly and the lissencephaly syndromes. Birth Defects 23: 225PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1989

Authors and Affiliations

  • Sharon E. Byrd
    • 4
  • R. E. Osborn
    • 1
  • T. P. Bohan
    • 2
  • T. P. Naidich
    • 3
  1. 1.Department of RadiologyNaval HospitalSan Diego
  2. 2.Departments of Pharmacology and NeurologyThe University of Texas Health Science CenterHouston
  3. 3.Department of RadiologyBaptist Hospital of MiamiMiamiUSA
  4. 4.Division of Neuroimaging Department of RadiologyChildren's Memorial Hospital Northwestern University Medical SchoolChicagoUSA

Personalised recommendations