Down's syndrome: Is there a decreased population of neurons?

Ross, Majorie H.; Galaburda, Albert M.; Kemper, Thomas L.

doi:10.1212/wnl.34.7.909

Cited by 191Down syndrome and intellectual disability researchGenetics and Neurodevelopmental DisordersWilliams Syndrome Research

Down's syndrome: Is there a decreased population of neurons?

Ross, Majorie H., Galaburda, Albert M., Kemper, Thomas L.·1984·Neurology

DOI 10.1212/wnl.34.7.909

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Abstract

Although gross abnormalities have been described in the brains of patients with Down's syndrome (DS), microscopic studies have revealed only minor and inconsistent findings. We compared two DS brains, in whole-brain serial sections, with similarly prepared age- and sex-matched normal controls. Architectonic abnormalities were noted, and cell counts revealed a significant poverty of granular cells in the DS brains, particularly in granular fields such as areas 3, 17, and 41. Golgi studies demonstrated all major cell types. A striking feature of the brain morphology in DS may be the curtailment of a specific cell type, most likely the aspinous stellate.

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The fetal cortex, cerebellum and hippocampus have all been shown to contain fewer neurons in individuals with Down syndrome than in controls, albeit based on small sample sizes.
“The fetal cortex, cerebellum and hippocampus have all been shown to contain fewer neurons in individuals with DS than in controls40–48, albeit based on small sample sizes17.”
From Consequences of trisomy 21 for brain development in Down syndrome (2024) · cites this paper as [46]
Several studies have shown that fewer interneurons are present in the cortex of individuals with Down syndrome than in controls, suggesting that reduced generation of interneurons contributes to the overall reduced neurogenesis in Down syndrome.
“Several studies have shown that fewer interneurons are present in the cortex of individuals with DS44,46,84 than in that of controls, suggesting that reduced generation of interneurons contributes to the overall reduced neurogenesis in DS.”
From Consequences of trisomy 21 for brain development in Down syndrome (2024) · cites this paper as [46]
Hypocellularity persists with fewer total neurons being identified in the superior temporal gyrus in adolescents and adults with Down syndrome (15-35 years).
“The hypocellularity persists with fewer total neurons being identified in the superior temporal gyrus in adolescents and adults with DS (15–35 years) (Ross et al., 1984; Giffin-Rao et al., 2020).”
From Neurodevelopment in Down syndrome: Concordance in humans and models (2022) · cites this paper as Ross et al., 1984
One study has identified a decreased number of aspinous stellate interneurons in young adults with Down syndrome.
“Broadly, one study has identified a decreased number of aspinous stellate interneurons in young adults with DS (Ross et al., 1984).”
From Neurodevelopment in Down syndrome: Concordance in humans and models (2022) · cites this paper as Ross et al., 1984

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Hinton, V. J., Brown, W. T., Wisniewski, K., & Rudelli, R. D. (1991) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320410306 · Cited by 527 · see citing works
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Mann, D. M. A. (1988) · Mechanisms of Ageing and Development · doi:10.1016/0047-6374(88)90041-3 · Cited by 346 · see citing works
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Belichenko, P. V., Masliah, E., Kleschevnikov, A. M., Villar, A. J., Epstein, C. J., Salehi, A., & Mobley, W. C. (2004) · Journal of Comparative Neurology · doi:10.1002/cne.20337 · Cited by 297 · see citing works
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Contestabile, A., Fila, T., Ceccarelli, C., Bonasoni, P., Bonapace, L., Santini, D., Bartesaghi, R., & Ciani, E. (2007) · Hippocampus · doi:10.1002/hipo.20308 · Cited by 281 · see citing works
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Becker, L. E., Armstrong, D. L., & Chan, F. (1986) · Annals of Neurology · doi:10.1002/ana.410200413 · Cited by 279 · see citing works
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Guidi, S., Bonasoni, P., Ceccarelli, C., Santini, D., Gualtieri, F., Ciani, E., & Bartesaghi, R. (2008) · Brain Pathology · doi:10.1111/j.1750-3639.2007.00113.x · Cited by 276 · see citing works
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Ballard, C. G., Mobley, W. C., Hardy, J., Williams, G. H., & Corbett, A. (2016) · The Lancet Neurology · doi:10.1016/s1474-4422(16)00063-6 · Cited by 274 · see citing works
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Dossi, E., Vasile, F., & Rouach, N. (2018) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2017.02.001 · Cited by 250 · see citing works
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Coyle, J. T., Oster-Granite, M. L., & Gearhart, J. D. (1986) · Brain Research Bulletin · doi:10.1016/0361-9230(86)90074-2 · Cited by 248 · see citing works
Some morphometric observations on the cerebral cortex and hippocampus in presenile Alzheimer's disease, senile dementia of Alzheimer type and Down's syndrome in middle age
Mann, D. M. A., Yates, P. O., & Marcyniuk, B. (1985) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(85)90129-7 · Cited by 240 · see citing works
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Hof, P. R. (1995) · Archives of Neurology · doi:10.1001/archneur.1995.00540280065020 · Cited by 234 · see citing works
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Haydar, T. F. (1999) · Cerebral Cortex · doi:10.1093/cercor/9.6.621 · Cited by 237 · see citing works
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Bahn, S., Mimmack, M., Ryan, M., Caldwell, M. A., Jauniaux, E., Starkey, M., Svendsen, C. N., & Emson, P. (2002) · The Lancet · doi:10.1016/s0140-6736(02)07497-4 · Cited by 211 · see citing works
Deficits in human trisomy 21 iPSCs and neurons
Weick, J. P., Held, D. L., Bonadurer, G. F., Doers, M. E., Liu, Y., Maguire, C., Clark, A., Knackert, J. A., Molinarolo, K., Musser, M., et al. (2013) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1216575110 · Cited by 205 · see citing works
OLIG2 Drives Abnormal Neurodevelopmental Phenotypes in Human iPSC-Based Organoid and Chimeric Mouse Models of Down Syndrome
Xu, R., Brawner, A. T., Li, S., Liu, J.-J., Kim, H., Xue, H., Pang, Z. P., Kim, W.-Y., Hart, R. P., Liu, Y., et al. (2019) · Cell Stem Cell · doi:10.1016/j.stem.2019.04.014 · Cited by 193 · see citing works
Human Models Are Needed for Studying Human Neurodevelopmental Disorders
Zhao, X., & Bhattacharyya, A. (2018) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2018.10.009 · Cited by 192 · see citing works
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Zhou, C. J., Qiu, Y., Pereira, F. A., Crair, M. C., Tsai, S. Y., & Tsai, M. (1999) · Neuron · doi:10.1016/s0896-6273(00)81032-6 · Cited by 185 · see citing works
Implications for treatment: GABAA receptors in aging, Down syndrome and Alzheimer’s disease
Rissman, R. A., & Mobley, W. C. (2011) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2011.07237.x · Cited by 172 · see citing works
Widespread Proliferation Impairment and Hypocellularity in the Cerebellum of Fetuses with Down Syndrome
Guidi, S., Ciani, E., Bonasoni, P., Santini, D., & Bartesaghi, R. (2011) · Brain Pathology · doi:10.1111/j.1750-3639.2010.00459.x · Cited by 165 · see citing works
Age‐related cortical grey matter reductions in non‐demented Down’s syndrome adults determined by MRI with voxel‐based morphometry
Teipel, S. J., Alexander, G. E., Schapiro, M. B., Möller, H., Rapoport, S. I., & Hampel, H. J. (2004) · Brain · doi:10.1093/brain/awh101 · Cited by 154 · see citing works
Protocols to establish genotype-phenotype correlations in Down syndrome.
Epstein, C. J., Korenberg, J. R., Annerén, G., Antonarakis, S. E., Aymé, S., Courchesne, E., Epstein, L. B., Fowler, A. E., Groner, Y., & Huret, J. L. (1991) · PubMed · Cited by 144 · see citing works
DSCAM/PAK1 pathway suppression reverses neurogenesis deficits in iPSC-derived cerebral organoids from patients with Down syndrome
Tang, X.-Y., Xu, L., Wang, J., Hong, Y., Wang, Y., Zhu, Q., Wang, D., Zhang, X.-Y., Liu, C.-Y., Fang, K.-H., et al. (2021) · Journal of Clinical Investigation · doi:10.1172/jci135763 · Cited by 143 · see citing works
The GABAergic Hypothesis for Cognitive Disabilities in Down Syndrome
Contestabile, A., Magara, S., & Cancedda, L. (2017) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2017.00054 · Cited by 141 · see citing works
Aneuploidy: From a Physiological Mechanism of Variance to Down Syndrome
Dierssen, M., Hérault, Y., & Estivill, X. (2009) · Physiological Reviews · doi:10.1152/physrev.00032.2007 · Cited by 132 · see citing works
Dendrites, dementia and the down syndrome
Takashima, S., Ieshima, A., Nakamura, H., & Becker, L. E. (1989) · Brain and Development · doi:10.1016/s0387-7604(89)80082-8 · Cited by 128 · see citing works
Dendritic and histochemical development and ageing in patients with Down's syndrome
TAKASHIMA, S., IIDA, K., MITO, T., & ARIMA, M. (1994) · Journal of Intellectual Disability Research · doi:10.1111/j.1365-2788.1994.tb00394.x · Cited by 126 · see citing works
Modeling Down Syndrome with Patient iPSCs Reveals Cellular and Migration Deficits of GABAergic Neurons
Huo, H.-Q., Qu, Z.-Y., Yuan, F., Ma, L., Yao, L., Xu, M., Hu, Y., Ji, J., Bhattacharyya, A., Zhang, S.-C., et al. (2018) · Stem Cell Reports · doi:10.1016/j.stemcr.2018.02.001 · Cited by 122 · see citing works
Neurogenesis impairment: An early developmental defect in Down syndrome
Stagni, F., Giacomini, A., Emili, M., Guidi, S., & Bartesaghi, R. (2018) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2017.07.026 · Cited by 120 · see citing works
Lifespan analysis of brain development, gene expression and behavioral phenotypes in the Ts1Cje, Ts65Dn and Dp(16)1/Yey mouse models of Down syndrome
Aziz, N. M., Guedj, F., Pennings, J. L. A., Olmos-Serrano, J. L., Siegel, A., Haydar, T. F., & Bianchi, D. W. (2018) · Disease Models & Mechanisms · doi:10.1242/dmm.031013 · Cited by 118 · see citing works
Genomic and functional profiling of human Down syndrome neural progenitors implicates S100B and aquaporin 4 in cell injury
Esposito, G., Imitola, J., Lu, J., De Filippis, D., Scuderi, C., Ganesh, V. S., Folkerth, R., Hecht, J., Shin, S., Iuvone, T., et al. (2008) · Human Molecular Genetics · doi:10.1093/hmg/ddm322 · Cited by 115 · see citing works
APP-dependent up-regulation of Ptch1 underlies proliferation impairment of neural precursors in Down syndrome
Trazzi, S., Mitrugno, V. M., Valli, E., Fuchs, C., Rizzi, S., Guidi, S., Perini, G., Bartesaghi, R., & Ciani, E. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr033 · Cited by 112 · see citing works
Down Syndrome: Advances in Molecular Biology and the Neurosciences
Capone, G. T. (2001) · Journal of Developmental & Behavioral Pediatrics · doi:10.1097/00004703-200102000-00007 · Cited by 110 · see citing works
SEIZURES IN CHILDREN WITH DOWN SYNDROME: ETIOLOGY, CHARACTERISTICS AND OUTCOME
Stafstrom, C. E., Patxot, O. F., Gilmore, H. E., & Wisniewski, K. E. (1991) · Developmental Medicine & Child Neurology · doi:10.1111/j.1469-8749.1991.tb05108.x · Cited by 106 · see citing works
INFANTILE SPASMS IN CHILDREN WITH DOWN SYNDROME
Stafstrom, C. E., & Konkol, R. J. (1994) · Developmental Medicine & Child Neurology · doi:10.1111/j.1469-8749.1994.tb11894.x · Cited by 105 · see citing works
Hyperkalemia during Anesthesia
Wisniewski, K. E., Laure‐Kamionowska, M., & Wisniewski, H. M. (1984) · New England Journal of Medicine · doi:10.1056/nejm198411013111818 · Cited by 105 · see citing works
Chapter 4 Alzheimer's Disease in Adults with Down Syndrome
Zigman, W. B., Devenny, D. A., Krinsky‐McHale, S. J., Jenkins, E. C., Urv, T. K., Węgiel, J., Schupf, N., & Silverman, W. P. (2008) · International review of research in mental retardation · doi:10.1016/s0074-7750(08)00004-9 · Cited by 99 · see citing works
VISUAL ACUITY IN INFANTS AND CHILDREN WITH DOWN SYNDROME
Courage, M. L., Adams, R. J., Reyno, S. M., & Kwa, P. G. (1994) · Developmental Medicine & Child Neurology · doi:10.1111/j.1469-8749.1994.tb11895.x · Cited by 94 · see citing works
DYRK1A-mediated cyclin D1 degradation in neural stem cells contributes to the neurogenic cortical defects in down syndrome
Najas, S., Arranz, J., Lochhead, P. A., Ashford, A. L., Oxley, D., Delabar, J. M., Cook, S. J., Barallobre, M. J., & Arbonés, M. L. (2015) · EBioMedicine · doi:10.1016/j.ebiom.2015.01.010 · Cited by 92 · see citing works
Silencing Trisomy 21 with XIST in Neural Stem Cells Promotes Neuronal Differentiation
Czerminski, J. T., & Lawrence, J. (2020) · Developmental Cell · doi:10.1016/j.devcel.2019.12.015 · Cited by 58 · see citing works
S100B and APP Promote a Gliocentric Shift and Impaired Neurogenesis in Down Syndrome Neural Progenitors
Lu, J., Esposito, G., Scuderi, C., Steardo, L., Delli-Bovi, L. C., Hecht, J. L., Dickinson, B. C., Chang, C. J., Mori, T., & Sheen, V. L. (2011) · PLoS ONE · doi:10.1371/journal.pone.0022126 · Cited by 89 · see citing works
Oxidative stress and neural dysfunction in Down Syndrome
Iannello, R. C., Crack, P. J., de Haan, J. B., & Kola, I. (1999) · Springer eBooks · doi:10.1007/978-3-7091-6380-1_17 · Cited by 89 · see citing works
Reduced cell number in the neocortical part of the human fetal brain in Down syndrome
Larsen, K. B., Laursen, H., Græm, N., Samuelsen, G. B., Bogdanovic, N., & Pakkenberg, B. (2008) · Annals of Anatomy - Anatomischer Anzeiger · doi:10.1016/j.aanat.2008.05.007 · Cited by 87 · see citing works
Morphological and biochemical assessment of DNA damage and apoptosis in Down syndrome and Alzheimer disease, and effect of postmortem tissue archival on TUNEL
Anderson, A. J., Stoltzner, S., Lai, F., Su, J. H., & Nixon, R. A. (2000) · Neurobiology of Aging · doi:10.1016/s0197-4580(00)00126-3 · Cited by 87 · see citing works
Excessive expression of synaptojanin in brains with Down syndrome
Arai, Y., Ijuin, T., Takenawa, T., Becker, L. E., & Takashima, S. (2002) · Brain and Development · doi:10.1016/s0387-7604(01)00405-3 · Cited by 86 · see citing works
Two key genes closely implicated with the neuropathological characteristics in Down syndrome: DYRK1A and RCAN1
Park, J., Oh, Y., & Chung, K. C. (2009) · BMB Reports · doi:10.5483/bmbrep.2009.42.1.006 · Cited by 86 · see citing works
Alteration of inhibitory circuits in the somatosensory cortex of Ts65Dn mice, a model for down’s syndrome.
Pérez-Cremades, D., Hernández, S., Blasco-Ibáñez, J. M., Crespo, C., Nacher, J., & Varea, E. (2010) · Journal of Neural Transmission · doi:10.1007/s00702-010-0376-9 · Cited by 85 · see citing works
Impaired myelination of the human hippocampal formation in Down syndrome
Ábrahám, H., Vincze, A., Veszprémi, B., Kravják, A., Gömöri, É., Kovács, G. G., & Seress, L. (2012) · International Journal of Developmental Neuroscience · doi:10.1016/j.ijdevneu.2011.11.005 · Cited by 85 · see citing works
Role of Founder Cell Deficit and Delayed Neuronogenesis in Microencephaly of the Trisomy 16 Mouse
Haydar, T. F., Nowakowski, R. S., Yarowsky, P., & Krueger, B. K. (2000) · Journal of Neuroscience · doi:10.1523/jneurosci.20-11-04156.2000 · Cited by 83 · see citing works
Evidence of Arrest of Neurogenesis and Synaptogenesis in Brains of Patients with Down's Syndrome
(1984) · New England Journal of Medicine · doi:10.1056/nejm198411013111819 · Cited by 82 · see citing works
Enhanced derivation of human pluripotent stem cell-derived cortical glutamatergic neurons by a small molecule
Cao, S.-Y., Hu, Y. F., Chen, C. Y., Yuan, F., Xu, M., Li, Q., Fang, K.-H., Chen, Y., & Liu, Y. (2017) · Scientific Reports · doi:10.1038/s41598-017-03519-w · Cited by 80 · see citing works
Discrepancy between Alzheimer‐Type Neuropathology and Dementia in Persons with Down's Syndromea
Wı́sniewski, H. M., & Rabe, A. (1986) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1986.tb40344.x · Cited by 81 · see citing works
Reflex Seizures are Frequent in Patients with Down Syndrome and Epilepsy
Guerrini, R., Genton, P., Bureau, M., Dravet, C., & Roger, J. (1990) · Epilepsia · doi:10.1111/j.1528-1157.1990.tb05496.x · Cited by 80 · see citing works
A Critical Period in Cortical Interneuron Neurogenesis in Down Syndrome Revealed by Human Neural Progenitor Cells
Bhattacharyya, A., McMillan, E., Chen, S. I., Wallace, K., & Svendsen, C. N. (2009) · Developmental Neuroscience · doi:10.1159/000236899 · Cited by 79 · see citing works
Total numbers of neurons and glial cells in cortex and basal ganglia of aged brains with Down syndrome-a stereological study.
Karlsen, A. S., & Pakkenberg, B. (2011) · Cerebral Cortex · doi:10.1093/cercor/bhr033 · Cited by 75 · see citing works
Is it possible to improve neurodevelopmental abnormalities in Down syndrome?
Bartesaghi, R., Guidi, S., & Ciani, E. (2011) · Reviews in the Neurosciences · doi:10.1515/rns.2011.037 · Cited by 74 · see citing works
Neurogenesis of the basal forebrain in euploid and trisomy 16 mice: An animal model for developmental disorders in down syndrome
Sweeney, J. E., Höhmann, C. F., Oster‐Granite, M. L., & Coyle, J. T. (1989) · Neuroscience · doi:10.1016/0306-4522(89)90384-9 · Cited by 73 · see citing works
Can neural selectionism be applied to cognitive development and its disorders?
Johnson, M. H., & Karmiloff‐Smith, A. (1992) · New Ideas in Psychology · doi:10.1016/0732-118x(92)90046-3 · Cited by 74 · see citing works
Event-related brain potentials during an extended visual recognition memory task depict delayed development of cerebral inhibitory processes among 6-month-old infants with Down syndrome
Karrer, J. H., Karrer, R., Bloom, D., Chaney, L., & Davis, R. (1998) · International Journal of Psychophysiology · doi:10.1016/s0167-8760(98)00015-4 · Cited by 70 · see citing works
The NGF Metabolic Pathway in the CNS and its Dysregulation in Down Syndrome and Alzheimer's Disease
Iulita, M. F., & Cuello, A. C. (2015) · Current Alzheimer Research · doi:10.2174/1567205012666150921100030 · Cited by 67 · see citing works
Consequences of Trisomy 16 for Mouse Brain Development: Corticogenesis in a Model of Down Syndrome
Haydar, T. F., Blue, M. E., Molliver, M. E., Krueger, B. K., & Yarowsky, P. (1996) · Journal of Neuroscience · doi:10.1523/jneurosci.16-19-06175.1996 · Cited by 66 · see citing works
Regional Cerebral Glucose Metabolism is Normal in Young Adults with down Syndrome
Schapiro, M. B., Grady, C. L., Kumar, A., Herscovitch, P., Haxby, J. V., Moore, A. M., White, B. J., Friedland, R. P., & Rapoport, S. I. (1990) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.1990.35 · Cited by 65 · see citing works
GVS‐111 prevents oxidative damage and apoptosis in normal and Down's syndrome human cortical neurons
Pelsman, A., Hoyo‐Vadillo, C., Гудашева, Т. А., Середенин, С. Б., Ostrovskaya, R. U., & Busciglio, J. A. (2003) · International Journal of Developmental Neuroscience · doi:10.1016/s0736-5748(03)00031-5 · Cited by 65 · see citing works
The developmental and aging changes of Down's syndrome cell adhesion molecule expression in normal and Down's syndrome brains
Saito, Y., Oka, A., Mizuguchi, M., Motonaga, K., Mori, Y., Becker, L. E., Arima, K., Miyauchi, J., & Takashima, S. (2000) · Acta Neuropathologica · doi:10.1007/s004010000230 · Cited by 65 · see citing works
Neuronal Proliferation, Migration, Organization, and Myelination
Volpe, J. J. (2008) · Elsevier eBooks · doi:10.1016/b978-1-4160-3995-2.10002-0 · Cited by 65 · see citing works
Small planum temporale volume in Down's syndrome: a volumetric MRI study
Frangou, S., Aylward, E., Warren, A., Sharma, T., Barta, P. E., & Pearlson, G. D. (1997) · American Journal of Psychiatry · doi:10.1176/ajp.154.10.1424 · Cited by 65 · see citing works
GABAergic over-inhibition, a promising hypothesis for cognitive deficits in Down syndrome
Martín, J. Z. D. S., Delabar, J. M., Bacci, A., & Potier, M. (2017) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2017.10.002 · Cited by 64 · see citing works
Normal Neurogenesis but Abnormal Gene Expression in Human Fragile X Cortical Progenitor Cells
Bhattacharyya, A., McMillan, E., Wallace, K., Tubon, T. C., Capowski, E. E., & Svendsen, C. N. (2008) · Stem Cells and Development · doi:10.1089/scd.2007.0073 · Cited by 61 · see citing works
Loss of neurones from cortical and subcortical areas in Down's syndrome patients at middle age
Mann, D. M. A., Yates, P. O., Marcyniuk, B., & Ravindra, C. (1987) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(87)90223-1 · Cited by 61 · see citing works
Magnetic resonance imaging evaluation of delayed myelination in Down syndrome: a case report and review of the literature
Koo, B. K. K., Blaser, S., Harwood-Nash, D., Becker, L. E., & Murphy, E. G. (1992) · Journal of Child Neurology · doi:10.1177/088307389200700417 · Cited by 59 · see citing works
Prenatal treatment with EGCG enriched green tea extract rescues GAD67 related developmental and cognitive defects in Down syndrome mouse models
Souchet, B., Duchon, A., Gu, Y., Dairou, J., Chevalier, C., Daubigney, F., Nalesso, V., Créau, N., Yu, Y., Janel, N., et al. (2019) · Scientific Reports · doi:10.1038/s41598-019-40328-9 · Cited by 54 · see citing works
Treating enhanced GABAergic inhibition in Down syndrome: Use of GABA α5-selective inverse agonists
Martínez-Cué, C., Delatour, B., & Potier, M.-C. (2014) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2013.12.008 · Cited by 58 · see citing works
Brain size and cerebral glucose metabolic rate in nonspecific mental retardation and down syndrome
Haier, R. J., Chueh, D., Touchette, P. E., Lott, I. T., Buchsbaum, M. S., MacMillan, D. L., Sandman, C. A., LaCasse, L., & Sosa, E. (1995) · Intelligence · doi:10.1016/0160-2896(95)90032-2 · Cited by 58 · see citing works
Newborn Basal Forebrain Lesions Disrupt Cortical Cytodifferentiation as Visualized by Rapid Golgi Staining
Höhmann, C. F., Kwiterovich, K. K., Oster‐Granite, M. L., & Coyle, J. T. (1991) · Cerebral Cortex · doi:10.1093/cercor/1.2.143 · Cited by 58 · see citing works
Untitled work
(2011) · PubMed · Cited by 57 · see citing works
Cortical differentiation and neurocognitive development: The parcellation conjecture
Johnson, M. H., & Vecera, S. P. (1996) · Behavioural Processes · doi:10.1016/0376-6357(95)00028-3 · Cited by 56 · see citing works
Mouse Models for Down Syndrome-Associated Developmental Cognitive Disabilities
Liu, C., Belichenko, P. V., Zhang, L., Fu, D., Kleschevnikov, A. M., Baldini, A., Antonarakis, S. E., Mobley, W. C., & Yu, Y. E. (2011) · Developmental Neuroscience · doi:10.1159/000329422 · Cited by 55 · see citing works
Ageing and Mental Handicap
Gath, A. (1986) · Developmental Medicine & Child Neurology · doi:10.1111/j.1469-8749.1986.tb14294.x · Cited by 53 · see citing works
Cerebral metabolic pattern in young adult Down's syndrome subjects: altered intercorrelations between regional rates of glucose utilization
Horwitz, B., Schapiro, M. B., GRADY, C. L., & Рапопорт, С. И. (1990) · Journal of Intellectual Disability Research · doi:10.1111/j.1365-2788.1990.tb01535.x · Cited by 51 · see citing works
Neurodevelopment in Down syndrome: Concordance in humans and models
Klein, J. A., & Haydar, T. F. (2022) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2022.941855 · Cited by 47 · see citing works
Recent Advances in the Genetics of Epilepsy: Insights from Human and Animal Studies
Prasad, A. N., Prasad, C., & Stafstrom, C. E. (1999) · Epilepsia · doi:10.1111/j.1528-1157.1999.tb02004.x · Cited by 47 · see citing works
Down Syndrome in Neurobiological Perspective: An Emphasis on Spatial Cognition
Uecker, A., Mangan, P. A., Obrzut, J. E., & Nadel, L. (1993) · Journal of Clinical Child Psychology · doi:10.1207/s15374424jccp2202_12 · Cited by 46 · see citing works
Some morphometric observations on the brains of patients with Down's syndrome: their relationship to age and dementia
Mann, D. M. A., Royston, M. C., & Ravindra, C. (1990) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(90)90152-d · Cited by 46 · see citing works
The Amyloid Precursor Protein (APP) Triplicated Gene Impairs Neuronal Precursor Differentiation and Neurite Development through Two Different Domains in the Ts65Dn Mouse Model for Down Syndrome
Trazzi, S., Fuchs, C., Valli, E., Perini, G., Bartesaghi, R., & Ciani, E. (2013) · Journal of Biological Chemistry · doi:10.1074/jbc.m113.451088 · Cited by 45 · see citing works
Retracted
Developmental regulation of adult cortical morphology and behavior: An animal model for mental retardation
Bachman, E. S., Berger-Sweeney, J. E., Coyle, J. T., & Höhmann, C. F. (1994) · International Journal of Developmental Neuroscience · doi:10.1016/0736-5748(94)90071-x · Cited by 45 · see citing works
Altered Distribution of Hippocampal Interneurons in the Murine down Syndrome Model Ts65Dn.
Hernández-González, S., Ballestín, R., López-Hidalgo, R., Gilabert-Juan, J., Blasco-Ibáñez, J. M., Crespo, C., Nácher, J., & Varea, E. (2015) · Neurochemical Research · doi:10.1007/s11064-014-1479-8 · Cited by 41 · see citing works
Proliferation deficits and gene expression dysregulation in Down's syndrome (Ts1Cje) neural progenitor cells cultured from neurospheres
Moldrich, R. X., Dauphinot, L., Laffaire, J., Vitalis, T., Hérault, Y., Beart, P., Rossier, J., Vivien, D., Gehrig, C., Antonarakis, S., et al. (2009) · Journal of Neuroscience Research · doi:10.1002/jnr.22131 · Cited by 48 · see citing works
Decline in cerebral glucose utilisation and cognitive function with aging in Down's syndrome.
Schapiro, M. B., Haxby, J. V., Grady, C. L., Duara, R., Schlageter, N. L., White, B., Moore, A., Sundaram, M., Larson, S. M., & Рапопорт, С. И. (1987) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.50.6.766 · Cited by 41 · see citing works
Chapter 4 Age-related changes in Down syndrome brain and the cellular pathology of Alzheimer disease
Williams, R. S., & Matthysse, S. (1986) · Progress in brain research · doi:10.1016/s0079-6123(08)64297-1 · Cited by 39 · see citing works
Altered patterning of trisomy 21 interneuron progenitors
Giffin-Rao, Y., Sheng, J., Strand, B., Xu, K., Huang, L., Medo, M., Risgaard, K. A., Dantinne, S., Mohan, S., Keshan, A., et al. (2022) · Stem Cell Reports · doi:10.1016/j.stemcr.2022.05.001 · Cited by 38 · see citing works
Brain myo-inositol level is elevated in Ts65Dn mouse and reduced after lithium treatment
Huang, W., Galdzicki, Z., van Gelderen, P., Balbo, A., Chikhale, E. G., Schapiro, M. B., & Rapoport, S. I. (2000) · Neuroreport · doi:10.1097/00001756-200002280-00004 · Cited by 38 · see citing works
Developmental deficits and staging of dynamics of age associated Alzheimer’s disease neurodegeneration and neuronal loss in subjects with Down syndrome
Wegiel, J., Flory, M., Kuchna, I., Nowicki, K., Wegiel, J., Ma, S. Y., Zhong, N., Bobrowicz, T., de Leon, M. D., Lai, F., et al. (2022) · Acta Neuropathologica Communications · doi:10.1186/s40478-021-01300-9 · Cited by 27 · see citing works
Auditory evoked potentials in Down's syndrome
Dı́az, F., & Zurrón, M. (1995) · Electroencephalography and Clinical Neurophysiology/Evoked Potentials Section · doi:10.1016/0013-4694(95)00080-i · Cited by 35 · see citing works
Unbalanced dendritic inhibition of CA1 neurons drives spatial-memory deficits in the Ts2Cje Down syndrome model
Valbuena, S., García, Á., Mazier, W., Paternain, A. V., & Lerma, J. (2019) · Nature Communications · doi:10.1038/s41467-019-13004-9 · Cited by 32 · see citing works
Topography of nerve cell loss from the locus caeruleus in middle aged persons with Down's syndrome
Marcyniuk, B., Mann, D. M. A., Yates, P. O., & Ravindra, C. (1988) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(88)90016-0 · Cited by 32 · see citing works
The neuroanatomy of mental retardation in the white rat
Thompson, R. H., Huestis, P. W., Crinella, F. M., & Yu, J. (1986) · Neuroscience & Biobehavioral Reviews · doi:10.1016/0149-7634(86)90016-3 · Cited by 32 · see citing works
Use of toxins to disrupt neurotransmitter circuitry in the developing brain
Johnston, M. V., Barks, J., Greenamyre, T., & Silverstein, F. S. (1988) · Progress in brain research · doi:10.1016/s0079-6123(08)60519-1 · Cited by 32 · see citing works

Cellularity Defects Are Not Ubiquitous in the Brains of Fetuses With Down Syndrome
Emili, M., Stagni, F., Bonasoni, M., Guidi, S., & Bartesaghi, R. (2024) · Developmental Neurobiology · doi:10.1002/dneu.22953 · Cited by 3 · see citing works
Total numbers of neurons and glial cells in cortex and basal ganglia of aged brains with Down syndrome-a stereological study.
Karlsen, A. S., & Pakkenberg, B. (2011) · Cerebral Cortex · doi:10.1093/cercor/bhr033 · Cited by 75 · see citing works
Reduced cell number in the neocortical part of the human fetal brain in Down syndrome
Larsen, K. B., Laursen, H., Græm, N., Samuelsen, G. B., Bogdanovic, N., & Pakkenberg, B. (2008) · Annals of Anatomy - Anatomischer Anzeiger · doi:10.1016/j.aanat.2008.05.007 · Cited by 87 · see citing works
Quantitative comparison of radial cell columns in children with Down's syndrome and controls
Buxhoeveden, D. P., Fobbs, A. D., Roy, E., & Casanova, M. F. (2002) · Journal of Intellectual Disability Research · doi:10.1046/j.1365-2788.2002.00362.x · Cited by 29 · see citing works
Non‐neuronal cell responses differ between normal and Down syndrome developing brains
Kanaumi, T., Milenković, I., Adle‐Biassette, H., Aronica, E., & Kovács, G. G. (2013) · International Journal of Developmental Neuroscience · doi:10.1016/j.ijdevneu.2013.09.011 · Cited by 39 · see citing works

Authors: Ross, M. H., Galaburda, A. M., & Kemper, T. L.
Affiliations: Boston University (US)
Venue: Neurology
Publisher: Ovid Technologies (Wolters Kluwer Health)
Published: 1984-07
Volume: 34
Issue: 7
Pages: 909-909
ISSN: 0028-3878 (ISSN-L)
1526-632X
DOI: 10.1212/wnl.34.7.909
OpenAlex: W2054562862
Cited by: 191
References: 0
Citations by year: 2025: 5 · 2024: 4 · 2023: 5 · 2022: 9 · 2021: 4 · 2020: 2 · 2019: 5 · 2018: 6 · 2017: 7 · 2016: 2 · 2015: 5 · 2014: 3 · 2013: 2 · 2012: 2
FWCI: 12.82
Type: journal article
Language: en
Open access: closed
Locations: Neurology (publishedVersion)
PubMed (publishedVersion)
Topics: Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Down syndrome and intellectual disability research
Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
Life Sciences › Neuroscience › Developmental Neuroscience › Williams Syndrome Research
Keywords: Down syndrome, brain architecture, neuronal cell counts, granular cells, brain structure, Golgi staining, cerebral cortex
MeSH terms: Adult, Auditory Cortex, Brain, Child, Down Syndrome, Female, Humans, Male, Somatosensory Cortex, Visual Cortex

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Down's syndrome: Is there a decreased population of neurons?

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