Abstract
This report addresses behavioral abnormalities in children with cerebellar anomalies demonstrated on MRI scans. Published data are presented showing an interaction between cerebellar pathology and early childhood autism. The cerebellum is involved not only in movement coordination, but also in social adaptation and verbal communication. The genes expressed in the cerebellum during childhood are identical to those expressed in the hippocampus. We have observed 20 children with MRI-identified agenesis of the cerebellar vermis and behavioral abnormalities; children were aged 3–15 (mean 7.05) years and there were 12 males and eight females. A variety of autistic characteristics were identified in these children.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
L. L. Baxter, T. H. Moran, J. T. Richtsmeier, et al., “Discovery and genetic localization of Down syndrome cerebellar phenotypes using the Ts65Dn mouse,” Hum. Mol. Genet., 9, No. 2, 185–202 (2000).
Y. C. Chang, C. C. Huang, and S. C. Huang, “Volumetric neuroimaging in children with neurodevelopmental disorders-mapping the brain and behavior, Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi, 39, No. 5, 285–292 (1998).
W. S. Condon, “Sound-film microanalysis: A means for correlating brain and behavior,” in: Dyslexia: A Neuroscientific Approach to Clinical Evaluation, F. Duffy and N. Geschwind (eds.), Little, Brown and Co., Boston (1985).
M. Eckert, “Neuroanatomical markers for dyslexia: a review of dyslexia structural imaging studies,” Neuroscientist, 10, No. 4, 362–371 (2004).
F. Fabbro, R. Moretti, and A. Bava, “Language impairments in patients with cerebellar lesions,” J. Neurolinguistics, 13, No. 2–3, 173–188 (2000).
J. M. Fletcher, M. Barnes, and M. Dennis, “Language development in children with spina bifida,” Semin. Pediat. Neurol., 9, No. 3, 201–208 (2002).
I. Frampton, K. Rubia, X. Chitnis, et al., “Heterozygous PAX6_mutation, adult brain structure and fronto-striato-thalamic function in a human family,” Eur. J. Neurosci., 19, No. 6, 1505–1512 (2004).
I. Golani, “Homeostatic motor processes in mammalian interactions: A choreography of display,” in: Perspectives in Ethology, P. P. G. Bateson and P. H. Klopfer (eds.), Plenum Press, New York (1976).
S. Holroyd, A. L. Reiss, and R. N. Bryan, “Autistic features in Joubert syndrome: a genetic disorder with agenesis of the cerebellar vermis,” Biol. Psychiatr., 29, No. 3, 287–294 (1991).
P. S. Kang and V. J. Caride, “Functional brain imaging in a patient with giant cisterna magna,” Clin. Nucl. Med., 27, No. 11, 827–828 (2002).
Y. M. Lenhoff, P. P. Wang, F. Greenberg, and U. Bellugi, “Williams syndrome and the brain,” Sci. Am., 277, No. 6, 68–73 (1997).
A. Lincoln, Z. Lai, and W. Jones, “Shifting attention and joint attention dissociation in Williams syndrome: implications for the cerebellum and social deficits in autism,” Neurocase, 8, No. 3, 226–232 (2002).
C. Lopes, Z. Chettouh, J. M. Delabar, and M. Rachidi, “The differentially expressed C21orf5_gene in the medial temporal-lobe system could play a role in mental retardation in Down syndrome and transgenic mice,” Biochem. Biophys. Res. Commun., 305, No. 4, 915–924 (2003).
L. A. Martin, T. Escher, D. Goldowitz, and G. Mittleman, “A relationship between cerebellar Purkinje cells and spatial working memory demonstrated in a lurcher/chimera mouse model system,” Gen. Brain Behav., 3, No. 3, 158–166 (2004).
M. Martinez de Lagran, X. Altafaj, X. Gallego, et al., “Motor phenotypic alterations in TgDyrk1a transgenic mice implicate DYRK1A in Down syndrome motor dysfunction,” Neurobiol. Dis., 15, No. 1, 132–142 (2004).
K. Pierce and E. Courchesne, “Evidence for a cerebellar role in reduced exploration and stereotyped behavior in autism,” Biol. Psychiat., 49, No. 8, 655–664 (2001).
J. M. Prats-Vinas, “Does the cerebellum play a part in cognitive processes?” Rev. Neurol., 31, No. 4, 357–359 (2000).
A. L. Reiss, S. Patel, A. J. Kumar, and L. Freund, “Preliminary communication: neuroanatomical variations of the posterior fossa in men with the fragile X (Martin-Bell) syndrome,” Am. J. Med. Genet., 31, No. 2, 407–414 (1988).
M. C. Silveri and S. Misciagna, “Language, memory, and the cerebellum,” J. Neurolinguistics, 13, No. 2–3, 129–143 (2000).
J. E. Schmitt, E. Eliez, I. S. Warsofsky, et al., “Enlarged cerebellar vermis in Williams syndrome,” J. Psychiat. Res., 35, No. 4, 225–229 (2001).
Y. Tendler, G. Weisinger, R. Coleman, et al., “Tissue-specific p53 expression in the nervous system,” Brain Res. Mol. Brain Res., 72, No. 1, 40–46 (1999).
“The neuropsychiatry of limbic and subcortical disorders,” in: S. Salloway, P. Malloy, and J. L. Cummings (eds.), American Psychiatric Press, Washington, London (1997), pp. 3–18 and 133–143.
D. Vandeinse and J. E. Hornyak, “Linguistic and cognitive deficits associated with cerebellar mutism,” Pediat. Rehabil., 1, No. 1, 41–44 (1997).
C. A. Williams and J. L. Frias, “The Angelman (happy puppet) syndrome,” Am. J. Med. Genet., 11, No. 4, 453–460 (1982).
Author information
Authors and Affiliations
Additional information
__________
Translated from Zhurnal Nevrologii i Psikhiatrii imeni S. S. Korsakova, Vol. 106, No. 8, pp. 21–25, August, 2006.
Rights and permissions
About this article
Cite this article
Bobylova, M.Y., Petrukhin, A.S., Dunaevskaya, G.N. et al. Clinical-psychological characteristics of children with dysgenesis of the cerebellar vermis. Neurosci Behav Physiol 37, 755–759 (2007). https://doi.org/10.1007/s11055-007-0078-4
Received:
Issue Date:
DOI: https://doi.org/10.1007/s11055-007-0078-4