Skip to main content

Advertisement

Log in

Cerebellar Disorders in Childhood: Cognitive Problems

  • Published:
The Cerebellum Aims and scope Submit manuscript

Abstract

Over the last decade, increasing evidence of cognitive functions of the cerebellum during development and learning processes could be ascertained. Posterior fossa malformations such as cerebellar hypoplasia or Joubert syndrome are known to be related to developmental problems in a marked to moderate extent. More detailed analyses reveal special deficits in attention, processing speed, visuospatial functions, and language. A study about Dandy Walker syndrome states a relationship of abnormalities in vermis lobulation with developmental problems. Further lobulation or volume abnormalities of the cerebellum and/or vermis can be detected in disorders as fragile X syndrome, Downs’s syndrome, William’s syndrome, and autism. Neuropsychological studies reveal a relation of dyslexia and attention deficit disorder with cerebellar functions. These functional studies are supported by structural abnormalities in neuroimaging in these disorders. Acquired cerebellar or vermis atrophy was found in groups of children with developmental problems such as prenatal alcohol exposure or extreme prematurity. Also, focal lesions during childhood or adolescence such as cerebellar tumor or stroke are related with neuropsychological abnormalities, which are most pronounced in visuospatial, language, and memory functions. In addition, cerebellar atrophy was shown to be a bad prognostic factor considering cognitive outcome in children after brain trauma and leukemia. In ataxia teleangiectasia, a neurodegenerative disorder affecting primarily the cerebellar cortex, a reduced verbal intelligence quotient and problems of judgment of duration are a hint of the importance of the cerebellum in cognition. In conclusion, the cerebellum seems to play an important role in many higher cognitive functions, especially in learning. There is a suggestion that the earlier the incorrect influence, the more pronounced the problems.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Schmahmann JD, Sherman JC (1998) The cerebellar cognitive affective syndrome. Brain 121:561–567

    Article  PubMed  Google Scholar 

  2. Rapoport M, van Reekum R, Mayberg H (2000) The role of the cerebellum in cognition and behaviour: a selective review. J Neuropsychiatry Clin Neurosci 12:193–198

    PubMed  CAS  Google Scholar 

  3. Shevell MI, Majnemer A (1996) Clinical features of developmental disability associated with cerebellar hypoplasia. Pediatr Neurol 15:224–229

    Article  PubMed  CAS  Google Scholar 

  4. Steinlin M, Styger M, Boltshauer E (1999) Cognitive impairments in patients with congenital nonprogressive cerebellar ataxia. Neurology 53:966–973

    PubMed  CAS  Google Scholar 

  5. Steinlin M, Schmid M, Landau K, Boltshauser E (1997) Follow-up in children with Joubert syndrome. Neuropediatrics 28:204–211

    Article  PubMed  CAS  Google Scholar 

  6. Fenell EB, Gitten JC, Dede DE, Maria BL (1999) Cognition, behavior, and development in Joubert syndrome. J Child Neurol 14:592–596

    Article  Google Scholar 

  7. Barreirinho MS, Teixeira J, Moreira NG, Bastos S, Goncalvez S, Barbot MC (2001) Joubert’s syndrome: report of 12 cases. Rev Neurol 32:812–817

    PubMed  CAS  Google Scholar 

  8. Hodgkins PR, Harris CM, Shawkat FS, Thompson DA, Chong K, Timms C et al (2004) Joubert Syndrome: long term follow-up. Dev Med Child Neurol 46:694–699

    Article  PubMed  Google Scholar 

  9. Boddaert N, Klein O, Ferguson N, Sonigo P, Parisot D, Hertz-Pannier L et al (2003) Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology 45:320–324

    PubMed  CAS  Google Scholar 

  10. Gerszten PC, Albright AL (1995) Relationship between cerebellar appearance and function in children with Dandy-Walker syndrome. Paediatr Neurosrurg 23:86–92

    Article  CAS  Google Scholar 

  11. Mostofksy SH, Mazzocco MM, Aakalu G, Warsofsky IS, Denckla MG, Reiss AL (1998) Decreased cerebellar posterior vermis size in fragile X syndrome: correlation with neurocognitive performance. Neurology 50:121–130

    Google Scholar 

  12. Cornish K, Kogan C, Turk J, Manly T, James N, Mills A et al (2005) The emerging fragile X premutation phenotype: evidence from the domain of social cognition. Brain Cogn 57:53–60

    Article  PubMed  Google Scholar 

  13. Jones W, Hesselink J, Courchesne E, Duncan T, Matsuda K, Bellugi U (2002) Cerebellar abnormalities in infants and toddlers with Williams syndrome. Dev Med Child Neurol 44:688–694

    Article  PubMed  Google Scholar 

  14. Rae C, Karmiloff-Smith A, Less MA, Dixon RM, Grant J, Blamire AM et al (1998) Brain biochemistry in Williams syndrome: evidence for a role of the cerebellum in cognition. Neurology 51:33–40

    Article  PubMed  CAS  Google Scholar 

  15. Nicolson R, Fawcett AJ, Dean P (2001) Dyslexia, development and the cerebellum. Trends Neurosci 24:515–516

    Article  PubMed  CAS  Google Scholar 

  16. Stoodley CJ, Harrison EP, Stein JF (2006) Implicit motor learning deficits in dyslexic adults. Neuropsychologia 44:795–798

    Article  PubMed  Google Scholar 

  17. Eckert MA, Leonard CM, Richards RL, Aylward EH, Thomson J, Berninger VW (2003) Anatomical correlates of dyslexia: frontal and cerebellar findings. Brain 126:482–494

    Article  PubMed  Google Scholar 

  18. Moretti R, Bava A, Torre P, Antonello RM, Cazzato G (2002) Reading errors in patients with cerebellar vermis lesions. J Neurol 249:461–468

    Article  PubMed  Google Scholar 

  19. Berquin PC, Gliedd JN, Jacobsen LK, Hamburger SD, Krain AL, Rapoport JL et al (1998) Cerebellum in attention-deficit hyperactivity disorder: a morphometric MRI study. Neurology 50:1087–1093

    PubMed  CAS  Google Scholar 

  20. Zang YF, Yong H, Chao-Zhe Z, Qing-Jiu C, Man-Qiu S, Meng L et al (2007) Altered baseline brain activity in children with ADHD revealed by resting-state functional MRI. Brain Dev 29:83–91

    Article  PubMed  Google Scholar 

  21. Hallahan B, Daly EM, McAlonan G, Loth E, Toal F, O’Brien F et al (2008) Brain morphometry volume in autistic spectrum disorder: a magnetic resonance imaging study of adults. Psychol Med 8:1–10

    Article  Google Scholar 

  22. Amaral DG, Schumann CM, Nordahl CW (2008) Neuroanatomy of autism. Trends Neurosci 31:137–145

    Article  PubMed  CAS  Google Scholar 

  23. Wills S, Cabanlit M, Bennett J, Asthwood P, Amaral DG, Van de4 Water J (2008) _Detection of autoantibodies to neural cells of the cerebellum in the plasma of subjects with autism spectrum disorders. Brain Behav Immun (in press). doi:10.1016/j.bbi.2008.07.007

  24. Rout UK, Dhossche DM (2008) A pathogenetic model of autism involving Purkinje cell loss through anti-GAD antibodies. Med Hypotheses 71:218–221

    Article  PubMed  CAS  Google Scholar 

  25. Sowell ER, Jernigan TL, Mattson SN, Riley EP, Sobel DF, Jones KL (1996) Abnormal development of the cerebellar vermis in children prenatally exposed to alcohol: size reduction in lobules I-V. Alcohol Clin Exp Res 20:31–34

    Article  PubMed  CAS  Google Scholar 

  26. Autti-Ramo I, Autti T, Korkman M, Kettunen S, Salonen O, Valanne L (2002) MRI findings in children with school problems who had been exposed prenatally to alcohol. Dev Med Child Neurol 44:98–106

    Article  PubMed  Google Scholar 

  27. Roebuck TM, Mattson SN, Riley EP (1998) A review of the neuroanatomical findings in children with fetal alcohol syndrome or prenatal exposure to alcohol. Alcohol Clin Exp Res 22:339–344

    Article  PubMed  CAS  Google Scholar 

  28. O’Hare ED, Kan E, Yoshii J, Mattson SN, Riley EP, Thompson PM et al (2005) Mapping cerebellar vermal morphology and cognitive correlates in prenatal alcohol exposure. Neuroreport 16:1285–1290

    Article  PubMed  Google Scholar 

  29. Krägeloh-Mann I, Toft P, Lunding J, Andresen J, Pryds O, Lou HC (1999) Brain lesions in preterms: origin, consequences and compensation. Acta Paediatr 88:897–908

    Article  PubMed  Google Scholar 

  30. Johnsen SD, Tarby TJ, Lewis KS, Bird R, Prenger E (2002) Cerebellar infarction: an unrecognized complication of very low birthweight. J Child Neurol 17:320–324

    Article  PubMed  Google Scholar 

  31. Johnsen SD, Bodensteiner JB, Lotze TE (2005) Frequency and nature of cerebellar injury in the extremely premature survivor with cerebral palsy. J Child Neurol 20:60–64

    Article  PubMed  Google Scholar 

  32. Limperopoulos C, Soul JS, Haidar H, Huppi PS, Bassan H, Warfield SK et al (2005) Impaired trophic interactions between the cerebellum and the cerebrum among preterm infants. Pediatrics 116:844–850

    Article  PubMed  Google Scholar 

  33. Limperopoulos C, Benson CB, Bassan H, Disalvo DN, Kinnamon DD, Moore M et al (2005) Cerebellar hemorrhage in the preterm infant: ultrasonographic findings and risk factors. Pediatrics M 116:717–724

    Article  Google Scholar 

  34. Levisohn L, Cronin-Golomb A, Schmahmann JD (2000) Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population. Brain 123:1041–1050

    Article  PubMed  Google Scholar 

  35. Riva D, Giorgi C (2000) The cerebellum contributes to higher functions during development: evidence from a series of children surgically treated for posterior fossa tumours. Brain 123:1051–1061

    Article  PubMed  Google Scholar 

  36. Steinlin M, Imfeld S, Zulauf P, Boltshauser E, Ridolfi-Lüthi A, Lövblad KO, Perrig W, Kaufmann F (2003) Neuropsychological long term sequelae after posterior fossa tumor resection during childhood. Brain 126:1998–2008

    Article  PubMed  Google Scholar 

  37. Beebe DW, Ris MD, Armstrong FD, Fontanesi J, Mulhern R, Holmes E (2005) Cognitive and adaptive outcome in low-grade paediatric cerebellar astrocytomas: evidence of diminished cognitive and adaptive functioning in National Collaborative Research Studies [CCG 9891/POG 9130]. J Clin Oncol 23(22):5198–5204

    Article  PubMed  Google Scholar 

  38. Scott RB, Stoodley CJ, Anslow P, Paul C, Stein JF, Sugden EM et al (2001) Lateralized cognitive deficits in children following cerebellar lesions. Dev Med Child Neurol 43:685–691

    Article  PubMed  CAS  Google Scholar 

  39. Paquier P, van Mourik M, van Dongen H, Catsman-Berrevoets C, Brison A (2003) Cerebellar mutism syndromes with subsequent dysarthria: a study of three children and a review of the literature. Rev Neurol 159:1017–1027

    PubMed  CAS  Google Scholar 

  40. Kursano Y, Tanaka Y, Takasanua H, Wada N, Tada T, Kakizawa Y, Hongo K (2006) Transient cerebellar mutism caused by bilateral damage to the dentate nuclei after the second posterior fossa surgery. J Neurosurg 104:329–331

    Article  Google Scholar 

  41. Papavasilou AS, Kotsalis C, Trakadas S (2004) Transient cerebellar mutism in the course of acute cerebellitis. Pediatr neruol 30:71–74

    Article  Google Scholar 

  42. Malm J, Kristensen B, Karlsson T, Carlberg B, Fagerlund M, Olsson T (1998) Cognitive impairment in young adults with infratentorial infarcts. Neurology 51:433–440

    PubMed  CAS  Google Scholar 

  43. Mostofsky SH, Kunze JC, Cutting LE, Lederman HM, Denckla MB (2000) Judgment of duration in individuals with ataxia-telangiectasia. Dev Neuropsychol 17:63–74

    Article  PubMed  CAS  Google Scholar 

  44. Soto Ares G, Vinchon M, Delmaire C, Abecidan E, Dheliemes P, Pruvo JP (2001) Cerebellar atrophy after severe traumatic head injury in children. Childs Nerv Syst 17:263–269

    Article  PubMed  CAS  Google Scholar 

  45. Ciesielski KT, Yanofsky R, Ludwig RN, Hill DE, Hart BL, Astur RS et al (1994) Hypoplasia of the cerebellar vermis and cognitive deficits in survivors of childhood leukemia. Arch Neurol 51:985–993

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Maja Steinlin.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Steinlin, M. Cerebellar Disorders in Childhood: Cognitive Problems. Cerebellum 7, 607–610 (2008). https://doi.org/10.1007/s12311-008-0083-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12311-008-0083-3

Keywords

Navigation