Abstract
We report neuropathological studies of five cases of type II lissencephaly from three fetuses and two infants. This comparative study allowed us to determine the developmental course of the cerebral lesions. Two distinct developmental events seem to generate this type of brain malformation: firstly, an early disturbance in cortex formation, which results both from a disorder of radial migration and a pial barrier disruption; secondly, a late perturbation of cerebral surface organization, resulting in fusion of the cerebral surface. All these features can be related to a primitive meningeal pathology, and more generally, to a neurocristopathy. Accordingly to our observations, this brain malformation appears during both migrational and post-migrational stages and may be considered more like a polymicrogyria than a lissencephaly.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Alcolado R, Weller RO, Parrish EP, Garrod D (1988) The cranial arachnoid and pia mater in man: anatomical and ultrastructural observations. Neuropathol Appl Neurobiol 14:1–17
Bordarier C, Aicardi J, Goutieres F (1984) Congenital hydrocephalus and eyes abnormalities with severe developmental brain defects: Warburg's syndrome. Ann Neurol 16:60–65
Choi BH, Matthias SC (1987) Cortical dysplasia associated with massive ectopia of neurons and glia within the subarachnoid space. Acta Neuropathol Berl 73:105–109
Dobyns WB, Pagon R, Armstrong D, et al (1989) Diagnostic criteria for Walker-Warburg Syndrome. Am J Med Genet 32: 195–210
Friede R (1989) Dysplasias of the cerebral cortex. In: R. L.Friede (ed) Developmental neuropathology, 2nd edn. Springer-Verlag, Berlin Heidelberg New-York Tokyo, pp 339–340
Fukuyama Y, Osawa M, Suzuki H (1981) Congenital progressive muscular dystrophy of the Fukuyama type-clinical, genetic and pathological considerations. Brain Dev 3:1–29
Goulding MD, Chelapakis G, Deutsch U, Erselius JR, Gruss P (1991) PAX-3, a novel murine DNA binding protein expressed during early neurogenesis. EMBO J 10:1135–1147
Gruss P, Walther C (1992) PAX in development. Cell 69: 719–722
Gude S, Burmester J, Pehlemann FW, Sievers J (1987) Meningeal cells produce constituents of the intertial matrix and the basal lamina at the cerebellar surface. In: Elsner N, Creutzfeldt O (eds) New frontiers in brain research. Thieme, Stuttgart, p 233
Harding BN (1988) Cerebro-ocular dysplasia with muscular involvement. Neuropathol Appl Neurobiol 14:258
Harding B (1992) Malformations of the central nervous system. In: Adams JH, Duchen LW (eds) Griendfield's neuropathology 5th edn. Arnold, London pp 569–572
Hartmann D, Sievers J, Pehlemann FW, Berry M (1992) Destruction of meningeal cells over the medial cerebral hemisphere of newborn hamsters prevents the formation of the infrapyramidal blade of the dentate gyrus. J Comp Neurol 320: 33–61
Knebel-Doeberitz C von, Sievers J, Sadler M, Pehlemann FW, Berry M, Halliwell P (1986) Destruction of meningeal cells over newborn hamster cerebellum with 6-Hydroxydopamine prevents foliation and lamination in the rostral cerebellum. Neuroscience 17:409–426
Kuban KCK, Gilles FH (1985) Human telencephalic angiogenesis. Ann Neurol 17:539–548
Larroche JC, Nessmann C (1993) Focal anomalies and retinal dysplasia in a 23–24-weeks-old fetus. Brain Dev 15:51–56
Leyten QH, Renkawek K, Renier WO, et al (1991) Neuropathological findings in muscle-eye-brain disease (MEB-D). Neuropathological delineation of MEB-D from congenital muscular dystrophy of the Fukuyama type. Acta Neuropathol 83:55–60
Miller G, Ladda RL, Towfighi J (1991) Cerebro-ocular dysplasia-muscular dystrophy (Walker Warburg) syndrome. Findings in a 20 weeks old fetus. Acta Neuropathol 82:234–238
Moase CE, Trasler DG (1991) N-CAM alterations in sploch neural tube defect in mouse embryos. Development 113:1049–1058
Mooy CM, Clark BJ, Lee WR (1990) Posterior axial corneal malformation and uveoretinal angiodysgenesis: a neurocristopathy? Graefes Arch Clin Exp Ophthalmol 228:9–18
Normann MG, O'Kusky JR (1986) The growth and development of microvasculature in human cerbral cortex. J. Neuropathol Exp. Neurol 45:222–232
Pavone L, Gullotta F, Grasso S, Vannucchi C (1986) Hydrocephalus, lissencepahly, ocular abnormalities and congenital muscular dystrophy. A Warburg syndrome variant? Neuropediatrics 17:206–211
Santavuori P, Sommer H, Sainio K, et al (1989) Muscle-Eye-Brain disease (MEB). Brain Dev 11:147–153
Sidman RL, Rakic P (1982) Development of the human central nervous system. In: Haymaker W, Adams RD (eds) Cytology and Cellular neuropathology. C C Thomas, Springfield, IL, pp 94–110
Squier MV (1993) Development of the cortical dysplasia of type II lissencephaly. Neuropathol Appl Neurobiol 19:209–213
Takada K, Nakamura H (1990) Cerebellar polymicrogyria in Fukuyama congenital muscular dystrophy: observations in fetal and pediatric cases. Brain Dev 12:774–778
Takada K, Nakamura H, Tanaka J (1984) Cortical dysplasia in congenital muscular dystrophy with central nervous system involvement (Fukuyama type). J Neuropathol Exp Neurol 43: 395–407
Takada K, Nakamura H, Suzumori K, Ishikawa T, Sugiyama N (1987) Cortical dysplasia in a 23-weeks fetus with Fukuyama congenital muscular dystrophy (FCMD). Acta Neuropathol (Berl) 74:300–306
Takada K, Nakamura H, Takashima S (1988) Cortical dysplasia in Fukuyama congenital muscular dystrophy (FCMD): a Golgi and angioarchitectonic analysis. Acta Neuropathol 76: 170–178
Tamagawa K, Scheidt P, Friede RL (1989) Experimental production of leptomeningeal heterotopias from dissociate fetal tissue. Acta Neuropathol 78:153–158
Walker AE (1942) Lissencephaly. Arch Neurol Psychiatry 48: 13–29
Yamaguchi E, Hayashi T, Kondoh H, et al (1993) A case of Walker-Warburg syndrome with uncommon findings; double cortical layer, temporal cyst and increased serum IgM. Brain Dev 15:61–66
Yoshioka M, Kuroki S, Kondo T (1990) Ocular manifestations in Fukuyama type congenital muscular dystrophy. Brain Dev 12:423–426
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Gelot, A., Billette de Villemeur, T., Bordarier, C. et al. Developmental aspects of type II lissencephaly. Acta Neuropathol 89, 72–84 (1995). https://doi.org/10.1007/BF00294262
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00294262