Hostname: page-component-84b7d79bbc-lrf7s Total loading time: 0 Render date: 2024-07-26T19:06:26.509Z Has data issue: false hasContentIssue false

The Place of Neuronal Migration Abnormalities in Child Neurology

Published online by Cambridge University Press:  18 September 2015

Jean Aicardi*
Affiliation:
Hôpital des Enfants Malades, 149 rue de Sèvres, Paris, France
*
The Wolfson Centre, Mecklenburgh Square, London WC1N 2AP England
Rights & Permissions [Opens in a new window]

Abstract:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

With the development of modern imaging techniques, disturbances of neuronal migration appear to be a major cause of epilepsy, mental retardation and chronic neurological disability in childhood. Sixty-nine cases are presented, including 46 of diffuse migration abnormalities and 23 of localized dysplasia. Patients with diffuse migration disorders presented with mental retardation, gross motor impairment and severe seizure disorders whereas in those with focal anomalies, epilepsy was the chief complaint. Magnetic resonance imaging, although usually diagnostic of migration disorders often does not allow definition of the pathologic type. Some EEG patterns, such as high amplitude fast rhythms or the theta-delta pattern are highly suggestive. Most cases of abnormal migration are sporadic and probably acquired. Some are due to chromosomal anomalies, especially of chromosome 17p where a gene for lissencephaly has been mapped. Familial cases occur with both recessive and possibly dominant inheritance. Epilepsy due to migration abnormalities is often intractable. Resection of dysplastic cortex may be effective for localized disease and callosotomy has been proposed for diffuse anomalies.

Type
Original Articles
Copyright
Copyright © Canadian Neurological Sciences Federation 1994

References

1.Rakic, R.Cell migration and neuronal ectopias in the brain. Birth Defects 1975; 11:95129.Google ScholarPubMed
2.Barkovick, AJ, Chuang, SH, Norman, D.MR of neuronal migration anomalies. Am J Neuroradiol 1987; 8: 10091017.Google Scholar
3.McConnell, S.Perspectives in early brain development and the epilepsies. In: Engel, Jet al., eds. Molecular Neurobiology of Epilepsy, Amsterdam: Elsevier 1992: 183191.Google Scholar
4.Dobyns, WB, Elias, ER, Newlin, AC, Pagon, RA, Ledbetter, DH.Causal heterogeneity in isolated lissencephaly. Neurology 1991; 42: 13751388.CrossRefGoogle Scholar
5.Aicardi, J.Diseases of the Nervous System in Childhood. London: MacKeith Press 1992: 150172.Google Scholar
6.Dobyns, WB.Developmental aspects of lissencephaly and the lissencephaly syndromes. Birth Defects 1993; 23: 225241.Google Scholar
7.Sarnat, HB.Disturbances of late neuronal migrations in the perinatal period. Am J Dis Child 1987; 141: 969980.Google ScholarPubMed
8.Lyon, G.Beaugerie, A.Disturbances of neuronal migration. In: Levene, MIet al., eds. Fetal and Neonatal Neurology and Neurosurgery. Edinburgh: Churchill Livingstone 1988: 231248.Google Scholar
9.Williams, RS, Caviness, VS.Normal and abnormal development of the brain. In: Tarter, RE, Goldstein, J, eds. Advances in Clinical Neuropsychology. Vol. 2. New York: Plenum 1984: 162.Google Scholar
10.Stewart, RM, Richman, DP, Caviness, VS.Lissencephaly and pachygyria: an architectonic and topographical analysis. Acta Neuropathol 1975; 31: 112.CrossRefGoogle ScholarPubMed
11.Titelbaum, DS, Hayward, JC, Zimmerman, RA.Pachygyrialike changes: topographic appearance at MR imaging and CT and correlation with neurologic status. Radiology 1989; 173: 663667.CrossRefGoogle Scholar
12.Meencke, HJ, Janz, D.Neuropathological findings in primary generalized epilepsy. A study of eight cases. Epilepsia 1984; 25: 821.CrossRefGoogle ScholarPubMed
13.Meencke, HJ, Veith, J.Migration disturbances in epilepsy. In: Engel, Jet al., eds. Molecular Neurobiology of Epilepsy. Amsterdam: Elsevier 1992:3140.Google Scholar
14.Huttenlocher, PR, Taravath, S, Mojtahedi, S.Periventricular heterotopia and epilepsy. Ann Neurol 1994; 44: 5155.Google ScholarPubMed
15.Kamuro, K, Tenokuchi, YI.Familial periventricular nodular heterotopia. Brain Dev 1993; 15: 237241.CrossRefGoogle ScholarPubMed
16.Della Giustina, E, Goffinet, AM, Landrieu, P, Lyon, G.A Golgi study of the brain malformation in Zellweger’s cerebro-hepato-renal disease. Acta Neuropathol 1981; 55: 2328.CrossRefGoogle ScholarPubMed
17.Böhm, N, Uy, J, Kiessling, M, Lehnert, W.Multiple acyl-CoA-dehydrogenase deficiency (glutaric aciduria type II), congenital polycystic kidneys and symmetric warty dysplasia of the cerebral cortex in two newborn brothers. II. Morphology and pathogenesis. Eur J Pediatr 1982; 139: 6065.CrossRefGoogle Scholar
18.Robain, O, Floquet, C, Heldt, N, Rozenberg, F.Hemimegalencephaly: a clinicopathological study of four cases. Neuropathol Appl Neurobiol 1988; 14: 125135.CrossRefGoogle ScholarPubMed
19.Chevrie, JJ, Aicardi, J.The Aicardi syndrome, In: Pedley, TP, Meldrum, BS, eds. Recent Advances in Epilepsy, vol. 3. Edinburgh: Churchill Livingstone 1986: 189210.Google Scholar
20.Fukuyama, Y, Osawa, M, Suzuki, H.Congenital progressive dystrophy of the Fukuyama type – clinical, genetic and pathological considereations. Brain Dev 1981; 3: 129.CrossRefGoogle Scholar
21.Dobyns, WB, McCluggage, CW.Computed tomographic appearance of lissencephaly syndromes. Am J Neuroradiol 1985; 6: 545550.Google ScholarPubMed
22.De Rijk van Andel, JF, Arts, WF, Barth, PG, Loonen, MC.Diagnostic features and clinical signs of 21 patients with lissencephaly type I. Dev Med Child Neurol 1990; 32: 707717.CrossRefGoogle Scholar
23.Dobyns, WB, Greenberg, F.Syndromes with lissencephaly. I. Miller-Dieker and Norman-Roberts syndromes and isolated lissencephaly. Am J Med Genet 1984; 18: 509526.CrossRefGoogle ScholarPubMed
24.Aicardi, J.The agyria-pachygyria complex: a spectrum of cortical malformations. Brain Dev 1991; 13: 18.CrossRefGoogle ScholarPubMed
25.Goutières, F, Aicardi, J, Rethoré, MO, Lejeune, J.Syndrome de Miller-Dieker et translocation chromosomique (15; 17). Arch Franç Pédiatr 1987; 44: 501504.Google Scholar
26.Livingston, JH, Aicardi, J.Unusual MRI appearance of diffuse subcortical heterotopia or “double-cortex” in two children. J Neurol Neurosurg Psychiatry 1990; 53: 617620.CrossRefGoogle Scholar
27.Palmini, A, Andermann, F, Aicardi, Jet al. Diffuse cortical dysplasia or the “double cortex” syndrome: the clinical and epileptic syndrome in 10 patients. Neurology 1991; 41: 16561662.CrossRefGoogle ScholarPubMed
28.Kuzniecky, R, Andermann, F, Guerrini, Ret al. Congenital bilateral perisylvian syndrome: study of 31 patients. Lancet 1993; 341: 608612.CrossRefGoogle ScholarPubMed
29.Kuzniecky, R, Andermann, F, Tampieri, Det al. Bilateral central macrogyria, epilepsy, pseudobulbar palsy, and mental retardation. A recognizable neuronal migration disorder. Ann Neurol 1989; 25: 547554.CrossRefGoogle ScholarPubMed
30.Guerrini, R, Dravet, C, Raybaud, Cet al. Neurological findings and seizure outcome in children with bilateral opercular microgyric-like changes detected by MRI. Dev Med Child Neurol 1992; 34: 694705.CrossRefGoogle Scholar
31.Palmini, A, Andermann, F, Olivier, Aet al. Focal neuronal migration disorder and intractable epilepsy: a study of 30 patients. Ann Neurol 1991; 30:741749.CrossRefGoogle ScholarPubMed
32.Palmini, A, Andermann, F, Olivier, Aet al. Focal neuronal migration disorder and intractable epilepsy: results of surgical treatment. Ann Neurol 1991; 30: 750757.CrossRefGoogle Scholar
33.Guerrini, R, Dravet, C, Raynaud, C.Epilepsy and focal anomalies detected by MRI: electroclinico-morphological correlations and follow-up. Dev Med Child Neurol 1992; 34: 706718.CrossRefGoogle ScholarPubMed
34.Palmini, A, Andermann, F, de Grissac, Het al. Stages and patterns of centrifugal arrest of diffuse neuronal migration disorders. Dev Med Child Neurol 1993; 35: 331339.CrossRefGoogle ScholarPubMed
35.Ambrosetto, G.Unilateral opercular macrogyria and benign childhood epilepsy with centrotemporal (rolandic) spikes: report of a case. Epilepsia 1992; 33: 499503.CrossRefGoogle ScholarPubMed
36.Gastaut, H, Pinsard, N, Raybaud, C, Aicardi, J, Zifkin, B.Lissencephaly (agyria-pachygyria): clinical findings and serial EEG studies. Dev Med Child Neurol 1987; 29: 167180.CrossRefGoogle ScholarPubMed
37.Truwit, CG, Barkovich, AJ, Koch, TK, Ferriero, DM.Cerebral palsy: MR findings in 40 patients. Am J Neuroradiol 1992; 13: 6778.Google ScholarPubMed
38.Wilkund, LM, Uvebrandt, P, Flodmark, O.Morphology of cerebral lesions in children with congenital hemiplegia. Neuroradiol 1990; 32: 179186.Google Scholar
39.Koeda, T, Suganuma, I, Kohno, Y, Takamatsu, T, Takeshita, K.MR imaging of spastic diplegia. Neuroradiol 1990; 32: 187190.CrossRefGoogle ScholarPubMed
40.Worster-Drought, C.Suprabulbar paresis. Dev Med Child Neurol 1975; suppl. 30: 1.Google Scholar
41.Kuzniecky, R, Berkovic, S, Andermann, Fet al. Focal cortical myoclonus and rolandic cortical dysplasia: clarification by magnetic resonance imaging. Ann Neurol 1988; 23: 317325.CrossRefGoogle ScholarPubMed
42.Barkovich, AJ, Chuang, SA.Unilateral megalencephaly: correlation of MR imaging and pathologic characteritics. Am J Neuroradiol 1990; 11:523531.Google Scholar
43.De Rijk van Andel, JF, Arts, WFM, de Weerd, AW.EEG and evoked potentials in a series of 21 patients with lissencephaly type I. Neuropediatrics 1992; 23: 49.CrossRefGoogle Scholar
44.Dulac, O, Plouin, P, Perulli, Met al. Aspects électroencéphalographiques de l’agyrie-pachygyrie classique. Rev. Electro-encephalogr Neurophysiol Clin 1983; 13:232239.CrossRefGoogle Scholar
45.Quirk, JA, Kendall, B, Kinglsey, DPE, Boyd, S, Pitt, MC.EEG features of cortical dysplasia in children. Neuropediatrics 1993; 24: 193199.CrossRefGoogle ScholarPubMed
46.Becket, PM, Dickson, AM, Troncoso, JC.Bilateral opercular poly microgyria. Ann Neurol 1989; 25: 9092.CrossRefGoogle Scholar
47.Norman, MG, Roberts, M, Sirois, J, Tremblay, LJM.Lissencephaly. Can J Neurol Sci 1976; 3: 3946.CrossRefGoogle ScholarPubMed
48.Wayne, ER, Burrington, JD, Myers, DN, Cotton, C, Block, W.Bilateral eventration of the diaphragm in a neonate with congenital cytomegalic inclusion disease. J Pediatr 1973; 83: 164165.CrossRefGoogle Scholar
49.Hayward, JC, Titelbaum, DS, Clancy, RR, Zimmerman, RA.Lissencephaly-pachygyria associated with congenital CMV infection. J Child Neurol 191; 6: 109114.CrossRefGoogle Scholar
50.Friede, RL.Developmental Neuropathology, 2nd edn. Berlin: Springer 1989: 340346.Google Scholar
51.Barkovich, AJ, Jos, D.Nonlissencephalic cortical dysplasia. Correlation of imaging findings with clinical deficits. Am J Neuroradiol 1992; 13:95103.Google ScholarPubMed
52.Cook, MJ, Free, SL, Fish, DRet al. Analysis of cortical patterns. In: Shorvon, SDet al., eds. Magnetic Resonance and Epilepsy. London: Plenum (in press).Google Scholar
53.Marques-Dias, MJ, Harmant-Van Rijckevorsel, G, Landrieu, P, Lyon, G.Prenatal cytomegalovirus disease and cerebral microgyria: evidence for perfusion failure, not disturbance of histogenesis, as the major cause of fetal cytomegalovirus encephalopathy. Neuropediatrics 1984; 15: 1824.CrossRefGoogle Scholar
54.Norman, MG.Encephaloclastic lesions in a 26-week gestation fetus: effect on neuronal migration. Can J Neurol Sci 1987; 14: 116.Google Scholar
55.Barth, PG.Disorders of neuronal migration. Can J Neurol Sci 1987; 14: 116.CrossRefGoogle ScholarPubMed
56.Menezes, L, Aicardi, J, Goutières, F.Absence of the septum pellucidum with porencephalia: a neuroradiologic syndrome with variable clinical expression. Arch Neurol 1988; 542545.CrossRefGoogle ScholarPubMed
57.Dvorak, K, Feit, J.Migration of neuroblasts through partial necrosis of the cerebral cortex in newborn rats. Contribution to the problem of morphological development and development period of cerebral microgyria. Acta Neuropathol 1977; 38: 203212.Google Scholar
58.Dvorak, K, Feit, J, Jurankova, Z.Experimentally induced focal microgyria and status verrucosus deformis in rats: autoradio-graphical study. Acta Neuropathol 1978; 44: 121129.CrossRefGoogle ScholarPubMed
59.Reiner, O, Carozzo, R, Shen, Y.Isolation of a Miller-Dieker lissencephaly gene containing G-protein beta subunit-like repeats. Nature 1993.CrossRefGoogle ScholarPubMed
60.Bordarier, C, Aicardi, J, Goutières, F.Congenital hydrocephalus and eye abnormalities with severe developmental brain defects: Warburg syndrome. Ann Neurol 1984; 16: 6065.CrossRefGoogle Scholar
61.Harbord, MG, Boyd, S, Hall-Crags, et al. Ataxia, developmental delay and an extensive migration abnormality in 2 siblings. Neuropediatrics 1990; 21: 218221.CrossRefGoogle Scholar
62.Parkind, H, Sotne, TT.Familial spastic paralysis: report of three cases in one family and observation at necropsy. Arch Neurol Psychiatry 1933; 30: 481500.CrossRefGoogle Scholar
63.Pavone, L, Gulotta, F, Incorpora, G, Grasso, S, Dobyns, WB.Isolated lissencephaly: report of four patients from two unrelated families. J Child Neurol 1990; 5: 5259.CrossRefGoogle ScholarPubMed
64.Andermann, E, Palmini, A, Andermann, Fet al. Familial bilateral congenital perisylvian syndrome: genetic determination of a localized neuronal migration disorder. Neurology 1992; 42 (suppl. 3): 354A.Google Scholar
65.Hosley, MA, Abroms, IF, Ragland, RL.Schizencephaly: case report of familial incidence. Pediatr Neurol 1992; 8: 148150.CrossRefGoogle ScholarPubMed
66.Robinson, RO.Familial schizencephaly. Dev Med Child Neurol 1991; 33: 10101014.CrossRefGoogle ScholarPubMed
67.Hilburger, AC, Willis, JK, Bouldin, E, Henderson-Tilton, A.Familial schizencephaly. Brain Dev 1993; 15: 234236.CrossRefGoogle ScholarPubMed