Brain, Vol. 124, No. 7, 1350-1361,
July 2001
© 2001 Oxford University Press
Persistent reelin-expressing Cajal–Retzius cells in polymicrogyria
1 The National Society for Epilepsy and Epilepsy Research Group, University Department of Clinical Neurology and 2 Department of Neuropathology, Institute of Neurology, 3 Department of Histopathology, Institute of Child Health, University College London, London, 4 Department of Neuropathology, Radcliffe Infirmary, Oxford, UK
Correspondence to:
Dr S. M. Sisodiya, Epilepsy Research Group, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK E-mail: sisodiya{at}ion.ucl.ac.uk
Cajal–Retzius (CR) cells are early-developing cells important in mammalian corticogenesis. Reelin, a protein secreted by CR cells, is essential for completion of neuronal migration and cortical lamination. Lack of reelin causes the `reeler' phenotype in mice and autosomal recessive lissencephaly with cerebellar hypoplasia in man. Focal increases in reelin and CR cells are associated with thickening and local invaginations of the marginal zone and microgyria in animal studies. It has been suggested that abnormalities of reelin expression may be involved in human polymicrogyria. We have studied CR cells and reelin expression in pathological sections of human polymicrogyria to explore this possibility. Occurrence, distribution, morphology and reelin expression in CR cells were studied in 12 cases of human polymicrogyria, ranging from 21 gestational weeks to 10 years of age. Findings were compared with age-matched controls. Large, reelin-positive CR-like cells were more numerous in the majority of the polymicrogyria cases and persisted for longer than usual, up to 10 years of age. The CR-like cells tended to cluster and were most frequent in fused molecular layers in the polymicrogyria. Reelin-expressing CR-like cells were also found in bridges between the molecular layer and overlying leptomeningeal heterotopia and within the heterotopia itself. Clusters of CR-like cells were also found in adjacent non-polymicrogyric cortex. No clusters were seen in the control subjects. Increased numbers of CR-like cells were seen in both familial and acquired cases. In contrast to previous reports, the findings show that large CR-like cells persisted for longer than usual, up to 10 years of age, and that they may continue to express reelin. Their maximal aggregation in regions of polymicrogyria and overlying leptomeningeal heterotopia suggest an association between the presence of these cells and polymicrogyria, which we interpret in the light of recent findings concerning the roles of reelin and its downstream signalling pathway in neuronal and glial developmental dynamics and post-developmental function.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  M. F. Paredes, G. Li, O. Berger, S. C. Baraban, and S. J. Pleasure Stromal-Derived Factor-1 (CXCL12) Regulates Laminar Position of Cajal-Retzius Cells in Normal and Dysplastic Brains. J. Neurosci., September 13, 2006; 26(37): 9404 - 9412. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Alcantara, E. Pozas, C. F. Ibanez, and E. Soriano BDNF-modulated Spatial Organization of Cajal-Retzius and GABAergic Neurons in the Marginal Zone Plays a Role in the Development of Cortical Organization Cereb Cortex, April 1, 2006; 16(4): 487 - 499. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R Paetau, J Saraneva, O Salonen, L Valanne, J Ignatius, and S Salenius Electromagnetic function of polymicrogyric cortex in congenital bilateral perisylvian syndrome J. Neurol. Neurosurg. Psychiatry, May 1, 2004; 75(5): 717 - 722. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. M. Rodier Environmental Causes of Central Nervous System Maldevelopment Pediatrics, April 1, 2004; 113(4/S1): 1076 - 1083. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Patel and A. J. Barkovich Analysis and Classification of Cerebellar Malformations AJNR Am. J. Neuroradiol., August 1, 2002; 23(7): 1074 - 1087. [Abstract] [Full Text] [PDF]
|
|