Abstract
The corticospinal tract develops over a rather long period of time, during which malformations involving this main central motor pathway may occur. In rodents, the spinal outgrowth of the corticospinal tract occurs entirely postnatally, but in primates largely prenatally. In mice, an increasing number of genes have been found to play a role during the development of the pyramidal tract. In experimentally studied mammals, initially a much larger part of the cerebral cortex sends axons to the spinal cord, and the site of termination of corticospinal fibers in the spinal grey matter is much more extensive than in adult animals. Selective elimination of the transient corticospinal projections yields the mature projections functionally appropriate for the pyramidal tract. Direct corticomotoneuronal projections arise as the latest components of the corticospinal system. The subsequent myelination of the pyramidal tract is a slow process, taking place over a considerable period of time. Available data suggest that in man the pyramidal tract develops in a similar way. Several variations in the funicular trajectory of the human pyramidal tract have been described in otherwise normally developed cases, the most obvious being those with uncrossed pyramidal tracts.
A survey of the neuropathological and clinical literature, illustrated with autopsy cases, reveals that the pyramidal tract may be involved in a large number of developmental disorders. Most of these malformations form part of a broad spectrum, ranging from disorders of patterning, neurogenesis and neuronal migration of the cerebral cortex to hypoxic-ischemic injury of the white matter. In some cases, pyramidal tract malformations may be due to abnormal axon guidance mechanisms. The molecular nature of such disorders is only beginning to be revealed.
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Ahdab-Barmada M, Claassen D (1990) A distinctive triad of malformations of the central nervous system in the Meckel-Gruber syndrome. J Neuropathol Exp Neurol 49:610–620
Aida N, Nishimura G, Hachiya Y, Matsui K, Takeuchi M, Itani Y (1998) MR imaging of perinatal brain damage: Comparison of clinical outcome with initial and follow-up MR findings. AJNR Am J Neuroradiol 19:1909–1921
Altman J, Bayer SA (2001) Development of the Human Spinal Cord. An interpretation based on experimental studies in animals. Oxford University Press, New York
Anton G (1922) Ueber Ersatz der Bewegungsleistungen beim Menschen und Entwicklungsstörungen des Kleinhirns. Zbl Ges Neurol Psychiatr 30:372–374
Armand J (1982) The origin, course and termination of corticospinal fibers in various mammals. Prog Brain Res 57:329–360
Armand J, Edgley SA, Lemon RN, Olivier E (1994) Protracted postnatal development of corticospinal projections from the primary motor cortex to hand motoneurones in the macaque monkey. Exp Brain Res 101:178–182
Armand J, Olivier E, Edgley SA, Lemon RN (1997) Postnatal development of corticospinal projections from motor cortex to the cervical enlargement in the macaque monkey. J Neurosci 17:251–266
Back SA, Luo NL, Borenstein NS, Levine JM, Volpe JJ, Kinney HC (2001) Late oligodendrocyte progenitors coincide with the developmental window of vulnerability for human perinatal white matter injury. J Neurosci 21:1302–1312
Bagnard D, Lohrum M, Uziel D, Püschel AW, Bölz J (1998) Semaphorins act as attractive and repulsive guidance signals during the development of cortical projections. Development 125:5043–5053
Bagri A, Marín O, Plump AS, Mak Y, Pleasure SJ, Rubinstein JLR, Tessier-Lavigne M (2002) Slit proteins prevent midline crossing and determine the dorsoventral position of major axonal pathways in the mammalian forebrain. Neuron 33:233–248
Banker BQ, Larroche JC (1962) Periventricular leukomalacia of infancy. Arch Neurol 7:386–410
Barkovich AJ, Kuzniecky RI, Jackson GD, Guerrini R, Dobyns WB (2001) Classification system for malformations of cortical development. Update 2001. Neurology 57:2168–2178
Barth PG, Mullaart R, Stam FC, Slooff JL (1982) Familial lissencephaly with extreme neopallial hypoplasia. Brain Dev 4:145–151
Bergemann AD, Zhang L, Chiang MK, Brambilla R, Klein R, Flanagan JG (1998) Ephrin-B3, a ligand for the receptor EphB3, expressed in the midline of the developing neural tube. Oncogene 16:471–480
Bickers DS, Adams RD (1949) Hereditary stenosis of the aqueduct of Sylvius as a cause of congenital hydrocephalus. Brain 72:246–262
Bouza H, Dubowitz LM, Rutherford M, Pennock JM (1994) Prediction of outcome in children with congenital hemiplegia: A magnetic resonance imaging study. Neuropediatrics 25:60–66
Brody BA, Kinney HC, Kloman AS, Gilles FH (1987) Sequence of central nervous system myelination in human autopsy. I. An autopsy study of myelination. J Neuropathol Exp Neurol 46:283–301
Bubis JJ, Landau WM (1964) Agenesis of the pyramidal tracts associated with schizencephalic clefts in rolandic cortex. Neurology 14:821–824
Caroni P, Schwab ME (1988a) Two membrane protein fractions from rat central myelin with inhibitory properties for neurite growth and fibroblast spreading. J Cell Biol 106:1281–1288
Caroni P, Schwab ME (1988b) Antibody against myelin-associated inhibitors of neurite growth neutralizes nonpermissive substrate properties of CNS white matter. Neuron 1:85–96
Castellani V, Chédotal A, Schachner M, Faivre-Sarrailh C, Rougon G (2000) Analysis of the L1-deficient mouse phenotype reveals cross-talk between Sema3A and L1 signaling pathways in axonal guidance. Neuron 27:237–249
Chow CW, Halliday JL, Anderson RMcD, Danks DM, Fortune DW (1985) Congenital absence of pyramids and its significance in genetic diseases. Acta Neuropathol 65:313–317
Coad JE, Angel C, Pierpont MEM, Gorlin RJ, Anderson ML (1997) Microcephaly with agenesis of corticospinal tracts and arthrogryposis, hypospadias, single umbilical artery, hypertelorism, and renal and adrenal hypoplasia—previously undescribed syndrome. Am J Med Genet 71:458–462
Cohen MM Jr, Kreiborg S (1990) The central nervous system in the Apert syndrome. Am J Med Genet 35:36–45
Coonan JR, Greferath U, Messenger J, Hartley L, Murphy M, Boyd AW, Dottori M, Galea MP, Bartlett PF (2001) Development and reorganization of corticospinal projections in EphA4 deficient mice. J Comp Neurol 436:248–262
D’Agostino AN, Kernohan JW, Brown JR (1963) The Dandy-Walker syndrome. J Neuropathol Exp Neurol 22:450–470
Dahme M, Bartsch U, Martini R, Anliker B, Schachner M, Mantei N (1997) Disruption of the mouse L1 gene leads to malformations of the nervous system. Nature Genet 17:346–349
Dambska M, Wisniewski K, Sher JH (1984) An autopsy case of hemimegalencephaly. Brain Dev 6:60–64
Darian-Smith I, Galea MP, Darian-Smith C, Sugitani M, Tan A, Burman K (1996) The anatomy of manual dexterity. Adv Anat Embryol Cell Biol 133:1–145
de Carlos JA, O’Leary DD (1992) Growth and targeting of subplate axons and establishment of major cortical pathways. J Neurosci 12:1194–1211
de Vries LS, Groenendaal F, van Haastert IC, Eken P, Rademaker KJ, Meiners LC (1999) Asymmetrical myelination of the posterior limb of the internal capsule in infants with periventricular haemorrhagic infarction: an early predictor of hemiplegia. Neuropediatrics 30:314–319
Dobson CB, Villagra F, Clowry GJ, Smith M, Kenwrick S, Donnai D, Miller S, Eyre JA (2001) Abnormal corticospinal function but normal axonal guidance in human L1CAM mutations. Brain 124:2393–2406
Dottori M, Hartley L, Galea M, Paxinos G, Polizotto M, Kilpatrick T, Bartlett PF, Murphy M, Köntgen F, Boyd AW (1998) EphA4 (Sek1) receptor tyrosine kinase is required for the development of the corticospinal tract. Proc Natl Acad Sci USA 95:13248–13253
Dum RP, Strick PL (1991) The origin of corticospinal projections from the premotor areas in the frontal lobe. J Neuroscience 11:667–689
Dum RP, Strick PL (1996) Spinal cord terminations of the medial wall motor areas in macaque monkeys. J Neurosci 16:6513–6525
Duqué J, Thonnard J-L, Vandermeeren Y, Sébire G, Cosnard G, Olivier E (2003) Correlation between impaired dexterity and corticospinal tract dysgenesis in congenital hemiplegia. Brain 126:732–747
Eyre JA, Miller S, Ramesh V (1991) Constancy of central production delays during development in man: Investigation of motor and somatosensory pathways. J Physiol (Lond) 434:441–452
Eyre JA, Miller S, Clowry GJ, Conway EA, Watts C (2000) Functional corticospinal projections are established prenatally in the human foetus permitting involvement in the development of spinal motor centres. Brain 123:51–64
Eyre JA, Taylor JP, Villagra F, Smith M, Miller S (2001) Evidence of activity-dependent withdrawal of corticospinal projections during human development. Neurology 57:1543–1554
Finckh U, Schröder J, Ressler B, Veske A, Gal A (2000) Spectrum and detection rate of L1CAM mutations in isolated and familial cases with clinically suspected L1-disease. Am J Med Genet 92:40–46
Finger JH, Bronson RT, Harris B, Johnson K, Przyborski SA, Ackerman SL (2002) The netrin 1 receptors Unc5h3 and Dcc are necessary at multiple choice points for the guidance of corticospinal tract axons. J Neurosci 22:10346–10356
Fink GR, Frackowiak RSJ, Pietrzyk U, Passingham RF (1997) Multiple nonprimary motor areas in the human cortex. J Neurophysiol 77:2164–2174
Flament D, Goldsmith P, Lemon RN (1992) The development of corticospinal projections to tail and hindlimb motoneurons studied in infant macaques using magnetic brain stimulation. Exp Brain Res 90:225–228
Flament D, Hall EJ, Lemon RN (1992) The development of corticomotoneuronal projections investigated using magnetic brain stimulation in the infant macaque. J Physiol (Lond) 447:755–768
Flechsig P (1876) Die Leitungsbahnen im Gehirn und Rückenmark des Menschen auf Grund entwickelungsgeschichtlicher Untersuchungen dargestellt. Engelmann, Leipzig
Forssberg H, Eliasson AC, Kinoshita H, Johansson RS, Westling G (1991) Development of human precision grip. I. Basic coordination of force. Exp Brain Res 85:451–457
Fransen E, van Camp G, Vits L, Willems PJ (1997) L1-associated diseases: Clinical geneticists divide,molecular geneticists unite. Hum Mol Genet 6:1625–1632
Friede RL (1989) Developmental Neuropathology. Second edition. Springer, Wien-New York
Friede RL, Boltshauser E (1978) Uncommon syndromes of cerebellar vermis aplasia. I. Joubert syndrome. Dev Med Child Neurol 20:758–763
Friede RL, Mikolasek J (1978) Postencephalitic porencephaly, hydranencephaly or polymicrogyria. A review. Acta Neuropathol 43:161–168
Fujimori KE, Takeuchi K, Yazaki T, Uyemura K, Nojyo Y, Tamamaki N (2000) Expression of L1 and TAG-1 in the corticospinal, callosal, and hippocampal commissural neurons in the developing rat telencephalon as revealed by retrograde and in situ hybridization double labeling. J Comp Neurol 417:275–288
Fukuyama Y, Osawa M, Suzuki H (1981) Congenital progressive muscular dystrophy of the Fukuyama type: Clinical, genetic and pathologic considerations. Brain Dev 3:1–29
Galea MP, Darian-Smith I (1995) Postnatal maturation of the direct corticospinal projections in the macaque monkey. Cereb Cortex 5:518–540
Geyer S, Matelli M, Luppino G, Zilles K (2000) Functional neuroanatomy of the primate isocortical motor system. Anat Embryol 202:443–474
Gorgels TGMF, Oestreicher AB, de Kort EJM, Gispen WH (1987) Immunocyto-chemical distribution of the protein kinase C substrate B-50 (GAP43) in developing rat pyramidal tract. Neurosci Lett 83:59–64
Granata T, Farina L, Faiella A, Cardini R, d’Incerti L, Boncinelli E, Battaglia G (1997) Familial schizencephaly associated with EMX2 mutation. Neurology 48:1403–1406
Gribnau AAM, de Kort EJM, Dederen PJWC, Nieuwenhuys R (1986) On the development of the pyramidal tract in the rat. II. An anterograde tracer study of the outgrowth of the corticospinal fibers. Anat Embryol 175:101–110
Gropman AL, Barkovich AJ, Vezina LG, Conry JA, Dubovsky EC, Packer RJ (1997) Pediatric congenital bilateral perisylvian syndrome: Clinical and MRI features in 12 patients. Neuropediatrics 28:198–203
Halliday J, Chow CW, Wallace D, Danke DM (1986) X linked hydrocephalus: A survey of a 20 years period in Victoria, Australia. J Med Genet 23:23–31
Harding B, Copp AJ (1997) Malformations. In: Graham DI, Lantos PL (eds) Greenfield’s Neuropathology. Arnold, London, pp 397–533
He S-Q, Dum RP, Strick PL (1993) Topographic organization of corticospinal projections from the frontal lobe: Motor areas on the lateral surface of the hemisphere. J Neurosci 13:952–980
He S-Q, Dum RP, Strick PL (1995) Topographic organization of corticospinal projections from the frontal lobe: Motor areas on the medial surface of the hemisphere. J Neurosci 14:3284–3306
Heffner CD, Lumsden AGS, O’Leary DDM (1990) Target control of collateral extension and directional axon growth in the mammalian brain. Science 247:217–220
Humphrey T (1960) The development of the pyramidal tracts in human fetuses, correlated with cortical differentiation. In: Tower DB, Schadé JP (eds) Structure and Function of the Cerebral Cortex. Elsevier, Amsterdam, pp 93–103
Inoue K, Terashima T, Inoue Y (1991) The intracortical position of pyramidal tract neurons in the motor cortex of the reeler changes from postnatal day 10 to adulthood. Dev Brain Res 62:146–150
Janzer RC, Friede RL (1982) Dandy-Walker syndrome with atresia of the fourth ventricle and multiple rhombencephalic malformations. Acta Neuropathol (Berl) 58:81–86
Jones EG, Schreyer DJ, Wise SP (1982) Growth and maturation of the rat corticospinal tract. Prog Brain Res 57:361–379
Joosten EAJ, Gribnau AAM (1989) Astrocytes and guidance of outgrowing corticospinal tract axons in the rat. An immunocytochemical study using anti-vimentin and anti-glial fibrillary acidic protein. Neuroscience 31:439–452
Joosten EAJ, Gribnau AAM, Dederen PJWC (1987) An anterograde tracer study of the developing corticospinal tract in the rat: Three components. Dev Brain Res 36:121–130
Joosten EAJ, Gribnau AAM, Gorgels TGMF (1990) Immunoelectron microscopic localization of cell adhesion molecule L1 in developing rat pyramidal tract. Neuroscience 38:675–686
Joosten EAJ, van der Ven PFM, Hooiveld MHW, ten Donkelaar HJ (1991) Induction of corticospinal target finding by release of a diffusible, chemotropic factor in cervical spinal grey matter. Neurosci Lett 128:25–28
Kadhim H, Tabarki B, Verellen G, de Prez C, Rona A-M, Sébire G (2001) Inflammatory cytokines in the pathogenesis of periventricular leukomalacia. Neurology 56:1278–1284
Karch SB, Urich H (1972) Occipital encephalocele: A morphological study. J Neurol Sci 15:89–112
Kertesz A, Geschwind N (1971) Patterns of pyramidal decussation and their relationship to handedness. Arch Neurol 24:326–332
Killackey HP, Dehay C, Giroud P, Berland M, Kennedy H (1997) Distribution of corticospinal projection neurons in the neocortex of the fetal macaque monkey. Soc Neurosci Abstr 23:902
Kinney HC, Brody BA, Kloman AS, Gilles FH (1988) Sequence of central nervous system myelination in human infancy. II. Patterns of myelination in autopsied infants. J Neuropathol Exp Neurol 47:217–234
Krams M, Quinton R, Ashburner J, Friston KJ, Frackowiak RSJ, Bouloux PMG, Passingham RE (1999) Kallmann’s syndrome. Mirror movements associated with bilateral corticospinal tract hypertrophy. Neurology 52:816–822
Kullander K, Croll SD, Zimmer M, Pan L, McClain J, Hughes V, Zabski S, DeChiara TM, Klein R, Yancopoulos GD,Gale NW (2001) Ephrin-B3 is the midline barrier that prevents corticospinal tract axons from recrossing, allowing for unilateral motor control. Genes Dev 15:877–888
Kuypers HGJM (1962) Corticospinal connections. Postnatal development in the rhesus monkey. Science 138:678–680
Kuypers HGJM (1981) Anatomy of the descending pathways. In: Brooks VB, Brookhart JM, Mountcastle VB (eds) Handbook of Physiology—The Nervous System, Vol 2: Motor Systems. American Physiological Society, Bethesda, MD, pp 597–666
Kuzniecky RF, Andermann F, Guerrini R (1993) CBPS Multicenter collaborative study: Congenital bilateral perisylvian syndrome: Study of 31 patients. Lancet 341:608–612
Lagger RL (1979) Failure of pyramidal tract decussation in the Dandy-Walker syndrome. J Neurosurg 50:382–387
Lawrence DG, Hopkins DA (1976) The development of motor control in the rhesus monkey: evidence concerning the role of corticomotoneuronal connections. Brain 99:235–254
Lemire RJ, Loeser JD, Leech RW, Alvord EC Jr (1975) Normal and Abnormal Development of the Human Nervous System. Harper and Row, Hagerstown, MD
Luhan JA (1959) Long survival after unilateral stab wound of medulla with unusual pyramidal tract distribution. Arch Neurol 1:427–434
Maksem JA, Roessmann U (1979) Apert’s syndome with central nervous system anomalies. Acta Neuropathol (Berl) 48:59–61
Marín O, Baker J, Puelles L, Rubinstein JLR (2002) Patterning of the basal telencephalon and hypothalamus is essential for guidance of cortical projections. Development 129:761–773
Marín-Padilla M (1990) Origin, formation and perinatal maturation of the human cerebral cortex: An overview. J Craniofac Genet Dev Biol 10:137–146
Marín-Padilla M (1996) Developmental neuropathology and impact of perinatal brain damage. I. Hemorrhagic lesions of neocortex. J Neuropathol Exp Neurol 55:758–773
Marín-Padilla M (1997) Developmental neuropathology and impact of perinatal brain damage. II. White matter lesions of the neocortex. J Neuropathol Exp Neurol 56:219–235
Marín-Padilla M (1999) Developmental neuropathology and impact of perinatal brain damage. III. Gray matter lesions of the neocortex. J Neuropathol Exp Neurol 58:407–429
Métin C, Deléglise D, Serafini T, Kennedy TE, Tessier-Lavigne M (1997) A role for netrin-1 in the guidance of cortical efferents. Development 124:5063–5074
Miller MW (1987) The origin of corticospinal projection neurons in rat. Exp Brain Res 67:339–351
Molnár Z, Blakemore C (1995) How do thalamic axons find their way to the cortex? Trends Neurosci 18:389–397
Molnár Z, Cordery PM (1999) Connections between cells of the internal capsule, thalamus and cerebral cortex in embryonic rat. J Comp Neurol 413:1–25
Müller F, O’Rahilly R (1990a) The human brain at stages 21–23, with particular reference to the cerebral cortical plate and to the development of the cerebellum. Anat Embryol 182:375–400
Müller F, O’Rahilly R (1990b) The human rhombencephalon at the end of the embryonic period proper. Am J Anat 189:127–145
Müller K, Hömberg V, Lenard H-G (1991) Magnetic stimulation of motor cortex and nerve roots in children. Maturation of cortico-motoneuronal projections. Electroencephalogr Clin Neurophysiol 81:63–70
Müller K, Kass-Illiyya F, Reitz M (1997) Ontogeny of ipsilateral corticospinal projections: A developmental study with transcranial magnetic stimulation. Ann Neurol 42:705–711
Nathan PW, Smith MC (1955) Long descending tracts in man. I. Review of present knowledge. Brain 78:248–303
Nathan PW, Smith MC, Deacon P (1990) The corticospinal tracts in man. Course and location of fibres at different segmental levels. Brain 113:303–324
Nelson KB, Dambrosia JM, Grether JK, Phillips TM (1998) Neonatal cytokines and coagulation factors in children with cerebral palsy. Ann Neurol 44:665–675
Norman MG, McGillivray BC, Kalousek DK, Hill A, Poskin KJ (1995) Congenital Malformations of the Brain. Pathologic, embryologic, clinical, radiologic and genetic aspects. Oxford University Press, New York
Nudo RJ, Masterton RB (1990) Descending pathways to the spinal cord. III. Sites of origin of the corticospinal tract. J Comp Neurol 296:559–583
Nyberg-Hansen R, Rinvik E (1963) Some comments on the pyramidal tracts, with special reference to its individual variations in man. Acta Neurol Scand 39:1–30
O’Leary DDM, Koester SE (1993) Development of projection neuron types, axon pathways, and patterned projections of the mammalian cortex. Neuron 10:991–1006
Olivier E, Edgley SA, Armand J, Lemon RN (1997) An electrophysiological study of the postnatal development of the corticospinal system in the macaque monkey. J Neurosci 17:267–276
Ostrovskaya TI, Lazjuk GI (1988) Cerebral abnormalities in the Neu-Laxova syndrome. Am J Med Genet 30:747–756
Pang D, Dias MS, Ahdab-Barmada M (1992) Split notochord malformation. Part I: A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 31:451–480
Picard N, Strick PL (1996) Motor areas of the medial wall: A review of their location and functional activation. Cerebral Cortex 6:342–353
Pinard J-M, Feydy A, Carlier R, Perez N, Pierot L, Burnod Y (2000) Functional MRI in double cortex: Functionality of heterotopia. Neurology 54:1531–1533
Polleux F, Giger RJ, Ginty DD, Kolodkin AL, Ghosh A (1998) Patterning of cortical efferent projections by semaphorin-neuropilin interactions. Science 282:1904–1906
Richards LJ, Koester SE, Tuttle R, O’Leary DD (1997) Directed growth of early cortical axons is influenced by a chemoattractant released from an intermediate target. J Neurosci 17:2445–2458
Robain O, Floquet C, Heldt N, Rozenberg F (1988) Hemimegalencephaly: A clinicopathological study of four cases. Neuropathol Appl Neurobiol 14:125–135
Roessmann U, Hori A (1985) Agyria (lissencephaly) with anomalous pyramidal crossing. Case report and review of literature. J Neurol Sci 69:357–364
Roland PE, Zilles K (1996) Functions and structures of the motor cortices in humans. Curr Opin Neurobiol 6:773–781
Rosenthal A, Jouet M, Kenwrick S (1992) Aberrant splicing of neural cell adhesion molecule L1 mRNA in a family with X-linked hydrocephalus. Nature Genet 2:107–112
Santavuori P, Somer H, Saino K, Rapola J, Kruus S, Nikitin T, Ketonen L, Leisti J (1989) Muscle-eye-brain disease (MEB). Brain Dev 11:147–153
Sarnat HB (2000) Molecular genetic classification of central nervous system malformations. J Child Neurol 15:675–687
Schottler F, Couture D, Rao A, Kahn H, Lee KS (1998) Subcortical connections of normotopic and heterotopic neurons in sensory and motor cortices of the tish mutant rat. J Comp Neurol 395:29–42
Schreyer DJ, Jones EG (1982) Growth and target finding by axons of the corticospinal tract in prenatal and postnatal rats. Neuroscience 7:1837–1853
Schreyer DJ, Jones EG (1988) Axon elimination in the developing corticospinal tract of the rat. Dev Brain Res 38:103–119
Schwab ME, Schnell L (1991) Channeling of developing rat corticospinal tract axons by myelin-associated neurite growth inhibitors. J Neurosci 11:709–721
Squier M, Chamberlain P, Zaiwalla Z, Anslow P, Oxbury J, Gould S, McShane MA (2000) Five cases of brain injury following amniocentesis in mid-term pregnancy. Dev Med Child Neurol 42:554–560
Squier W (2002) Pathology of fetal and neonatal brain damage: identifying the timing. In: Squier W (ed) Acquired Damage to the Developing Brain: Timing and causation. Arnold, London, pp 110–127
Stanfield BB (1992) The development of the corticospinal projection. Prog Neurobiol 38:169–202
Stanfield BB, O’Leary DDM (1985) The transient corticospinal projection from the occipital cortex during the postnatal development of the rat. J Comp Neurol 238:236–248
Staudt M, Niemann G, Grodd W, Krägeloh-Mann I (2000) The pyramidal tract in congenital hemiparesis: Relationship between morphology and function in periventricular lesions. Neuropediatrics 31:257–264
Staudt M, Grodd W, Gerloff C, Erb M, Stitz J, Krägeloh-Mann I (2002) Two types of ipsilateral reorganization in congenital hemiparesis. A TMS and fMRI study. Brain 125:2222–2237
Staudt M, Pavlova M, Böhm S, Grodd W, Krägeloh-Mann I (2003) Pyramidal tract damage correlates with motor dysfunction in bilateral periventricular leukomalacia (PVL). Neuropediatrics 34:182–188
ten Donkelaar HJ (2000) Development and regenerative capacity of descending supraspinal pathways in tetrapods: A comparative approach. Adv Anat Embryol Cell Biol 145:1–145
ten Donkelaar HJ, Wesseling P, Semmekrot BA, Liem KD, Tuerlings J, Cruysberg JRM, de Wit PEJ (1999) Severe, non X-linked congenital microcephaly with absence of the pyramidal tracts in two siblings. Acta Neuropathol (Berl) 98:203–211
ten Donkelaar HJ, Hoevenaars F, Wesseling P (2000) A case of Joubert’s syndrome with extensive cerebral malformations. Clin Neuropathol 19:85–93
ten Donkelaar HJ, Willemsen MAAP, van der Heijden I, Beems T, Wesseling P (2002) A spinal intradural enterogenous cyst with well-differentiated muscularis propria. Acta Neuropathol (Berl)104:538–542
Terashima T (1995) Course and collaterals of corticospinal fibers arising from the sensorimotor cortex in the reeler mouse. Dev Neurosci 17:8–19
van der Knaap MS, Valk J (1995) Magnetic Resonance of Myelin, Myelination and Myelin Disorders. Second edition. Springer, Berlin-Heidelberg-New York
Verhaart WJC (1950) Hypertrophy of pes pedunculi and pyramid as result of degeneration of contralateral corticofugal fiber tracts. J Comp Neurol 92:1–15
Verhaart WJC, Kramer W (1952) The uncrossed pyramidal tract. Acta Psychiatr Neurol Scand 27:181–200
Vogel H, Halpert D, Horoupian DS (1990) Hypoplasia of posterior spinal roots and dorsal spinal tracts with arthrogryposis multiplex congenita. Acta Neuropathol (Berl)79:692–696
Volpe JJ (2001) Hypoxic-ischemic encephalopathy: Neuropathology and pathogenesis. In: Volpe JJ. Neurology of the Newborn. Fourth edition. Saunders, Philadelphia, pp 296–330
Volpe JJ (2001) Neurobiology of periventricular leukomalacia in the premature infant. Pediatr Res 50:553–563
Volpe JJ, Adams RD (1972) Cerebro-hepato-renal syndrome of Zellweger: An inherited disorder of neuronal migration. Acta Neuropathol (Berl) 20:175–198
Weimann JM, Zhang YA, Levin ME, Devine WP, Brûlet P, McConnell SK (1999) Cortical neurons require Otx1 for the refinement of exuberant axonal projections to subcortical targets. Neuron 24:819–831
Williams RS, Swisher CN, Jennings M, Ambler M, Caviness VS Jr (1984) Cerebro-ocular dysgenesis (Walker-Warburg syndrome): Neuropathologic and etiologic analysis. Neurology 34:1531–1541
Wózniak W, O’Rahilly R (1982) An electron microscopic study of myelination of pyramidal fibres at the level of the pyramidal decussation in the human fetus. J Hirnforsch 23:331–342
Yachnis AT, Rorke LB (1999) Neuropathology of Joubert’s syndrome. J Child Neurol 14:655–659
Yakovlev PI, Lecours AR (1967) The myelogenetic cycles of regional maturation of the brain. In: Minkowski A (ed) Regional Development of the Brain in Early Life. Blackwell, Oxford, pp 3–70
Yakovlev PI, Rakic P (1966) Patterns of decussation of bulbar pyramids and distribution of pyramidal tracts on two sides of the spinal cord. Trans Am Neurol Assoc 91:366–367
Yamamoto T, Sakakibara S, Mikoshiba K, Terashima T (2003) Ectopic corticospinal tract and corticothalamic tract neurons in the cerebral cortex of yotari and reeler mice. J Comp Neurol 461:61–75
Yokoyama N, Romero MI, Cowan CA, Galvan P, Heimbacher F, Charnay P, Parada LF, Henkemeyer M (2001) Forward signaling mediated by Ephrin-B3 prevents contralateral corticospinal axons from recrossing the spinal cord midline. Neuron 29:85–97
Jen JC, Chan W-M, Bosley TM, Wan J, Carr JR, Rüb U, Shattuck D, Salamon G, Kudo LC, Ou J, Lin DDM, Salih MAM, Kansu T, al Dhalaan H, al Zayed Z, MacDonald DB, Stigsby B, Plaitakis A, Dretakis EK, Gottlob I, Pieh C, Traboulsi EI, Wang Q, Wang L, Andrews C,Yamada K, Demer JL, Karim S, Alger JR, Geschwind DH, Deller T, Sicotte NL, Nelson SF, Baloh RW, Engle EC (2004) Mutations in a human ROBO gene disrupt hindbrain axon pathway crossing and morphogenesis. Science 304:1509–1513
Sabatier C, Plump AS, Ma L, Brose K, Tamada A, Murakami F, Lee EY-HP, Tessier-Lavigne M (2004) The divergent Robo family protein Rig-1/Robo3 is a negative regulator of slit responsiveness required for midline crossing by commissural axons. Cell 117:157–169
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ten Donkelaar, H.J., Lammens, M., Wesseling, P. et al. Development and malformations of the human pyramidal tract. J Neurol 251, 1429–1442 (2004). https://doi.org/10.1007/s00415-004-0653-3
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DOI: https://doi.org/10.1007/s00415-004-0653-3