Acta Neuropathologica

, Volume 60, Issue 1–2, pp 137–141 | Cite as

Pachygyria and congenital nephrosis disorder of migration and neuronal orientation

  • O. Robain
  • T. Deonna
Short Original Communications


A case of pachygyria with associated nephrosis has been studied. Several microscopic abnormalities have been identified: cytoarchitectonic disorders including neuronal ectopies in the molecular layer and in the meninges, improperly oriented neurons shown with Golgi stain, fetal aspect of inferior olives. The mechanism of the disorder of migration and neuronal and dendritic orientation are discussed. The significance of the association of microcephaly and nephrosis is also reviewed in light of recent literature.

Key words

Microcephaly Congenital nephrosis Golgi stain Inverted neurons 


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  1. Brun A (1965) The subpial granular layer of the fetal cerebral cortex in man. Acta Pathol Microbiol Scand [Suppl] 176:7–98Google Scholar
  2. Caviness VS, Evrard P, Lyon G (1978) Radial neuronal assemblies, ectopia and necrosis of developing cortex. A case analysis. Acta Neuropathol (Berl) 41:67–72Google Scholar
  3. Caviness VS, Williams RS (1979) Cellular pathology of developing human cortex. Congenital and acquired cognitive disorders. New York, pp 69–89Google Scholar
  4. Crome L (1956) Pachygyria. J Pathol Bacteriol 71:335–352Google Scholar
  5. Daube J, Chou SM (1966) Lissencephaly: two cases. Neurology (Minneap) 16:179–191Google Scholar
  6. Galloway WH, Mowatt AP (1968) Congenital microcephaly with hiatus hernia and nephrotic syndrome in two sibs. J Med Genet 5:319–321Google Scholar
  7. Globus A, Scheibel AB (1967) Pattern and field in cortical structure: the rabbit. J Comp Neurol 131:155–172Google Scholar
  8. König N, Hornung JP, Van der Loos H (1981) Identification of Cajal-Retzius cells in immature rodent cerebral cortex: a combined Golgi-EM. study. Neurosci Lett 27:225–229Google Scholar
  9. Lyon G, Evrard PH (1982) Disorders of telencephalic development. IXth International Congress of Neuropathology, ViennaGoogle Scholar
  10. Norio R (1966) Heredity in the congenital nephrotic syndrome. A genetic study of 57 Finnish families with a review of reported cases. Ann Paediat Finn [Suppl 27] 12Google Scholar
  11. Pinto Lord MC, Caviness VS (1979) Determinans of cell shape and orientation: A comparative Golgi analysis of cell axon interrelationships in the developing neocrotex of normal and reeler mice. J Comp Neurol 187:49–70Google Scholar
  12. Robain O, Lyon G (1972) Les micrencéphalies familiales par malformations cérébrales. Acta Neuropathol (Berl) 20:96–109Google Scholar
  13. Shapiro LR, Duncan PA, Farnsworth PB, Lefkowitz M (1976) Congenital microcephaly, hiatus hernia, and nephrotic syndrome; an autosomal recessive syndrome. Birth Defects 12:275–278Google Scholar
  14. Sievers J, Mangold U, Berry M, Allen C, Schlossberger HG (1981) Experimental studies on cerebellar foliation. I. A qualitative morphological analysis of cerebellar fissuration defects after neonatal treatment with G-OHDA in the rat. J Comp Neurol 203:751–769Google Scholar
  15. Stewart RM, Richman DP, Caviness VS Jr (1975) Lissencephaly and pachygyria: An architectonic and topographical analysis. Acta Neuropathol (Berl) 31:1–12Google Scholar
  16. Van Der Loos H (1965) The improperly oriented pyramidal cell in the cerebral cortex and its possible bearing on problems of neuronal growth and cell orientation. Bull John Hopkins Hosp 117: 228–250Google Scholar

Copyright information

© Springer-Verlag 1983

Authors and Affiliations

  • O. Robain
    • 1
  • T. Deonna
    • 2
  1. 1.INSERM U. 154Hôpital Saint Vincent de PaulParis Cédex 14France
  2. 2.Centre Hospitalier Universitaire VaudoisLausanneSwitzerland

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