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Clinical Features, Assessment, and Management of Patients with Developmental and Other Cerebellar Disorders

  • Michael S. SalmanEmail author
Chapter
Part of the Contemporary Clinical Neuroscience book series (CCNE)

Abstract

The cerebellum is essential for processing, modulating, and controlling movement, behavior, and cognitive functions. Cerebellar disorders cause tremor and incoordination, larger variability, and inaccuracy of movements during eye and limb movements, stance, and speech. Cerebellar dysfunction also results in impaired cognition and behavior. During the clinical assessment, details of the presenting complaints including onset and time course of ataxia, other symptoms, past medical history including developmental milestones, family history, and drug history are elicited. On examination emphasis is placed on examining the motor system especially speech and eye and limb movements. Other aspects include general examination, head size, dysmorphic features, neurocutaneous stigmata, and cognitive function assessment. A thorough exam of the cranial nerves, tone, strength, coordination, reflexes, gait, and sensation should be undertaken. A comprehensive assessment helps to narrow down the diagnostic possibilities and offers clues to specific disorders of the cerebellum. Management is guided by disease etiology.

Keywords

Cerebellum Motor coordination Eye movements Speech articulation Cognitive function 

References

  1. 1.
    Salman MS, Lee EJ, Tjahjadi A, Chodirker BN. The epidemiology of intermittent and chronic ataxia in children in Manitoba, Canada. Dev Med Child Neurol. 2013;55(4):341–7.CrossRefGoogle Scholar
  2. 2.
    Musselman KE, Stoyanov CT, Marasigan R, Jenkins ME, Konczak J, Morton SM, et al. Prevalence of ataxia in children: a systematic review. Neurology. 2014;82(1):80–9.CrossRefGoogle Scholar
  3. 3.
    Pandolfo M, Manto M. Cerebellar and afferent ataxias. Continuum (Minneap Minn). 2013;19(5):1312–43.Google Scholar
  4. 4.
    Prasad M, Ong MT, Setty G, Whitehouse WP. Fifteen-minute consultation: the child with acute ataxia. Arch Dis Child Educ Pract Ed. 2013;98(6):217–23.CrossRefGoogle Scholar
  5. 5.
    Bernard G, Shevell M. The wobbly child: an approach to inherited ataxias. Semin Pediatr Neurol. 2008;15(4):194–208.CrossRefGoogle Scholar
  6. 6.
    Holmes G. The Croonian lectures on the clinical symptoms of cerebellar disease and their interpretation. Lecture III. Lancet. 1922;200:59–65.CrossRefGoogle Scholar
  7. 7.
    Reeber SL, Otis TS, Sillitoe RV. New roles for the cerebellum in health and disease. Front Syst Neurosci. 2013;7:83.CrossRefGoogle Scholar
  8. 8.
    Bodranghien F, Bastian A, Casali C, Hallett M, Louis ED, Manto M, et al. Consensus paper: revisiting the symptoms and signs of cerebellar syndrome. Cerebellum. 2016;15(3):369–91.CrossRefGoogle Scholar
  9. 9.
    Manto M, Bower JM, Conforto AB, Delgado-García JM, da Guarda SN, Gerwig M, et al. Consensus paper: roles of the cerebellum in motor control – the diversity of ideas on cerebellar involvement in movement. Cerebellum. 2012;11(2):457–87.CrossRefGoogle Scholar
  10. 10.
    Marien P, Ackermann H, Adamaszek M, Barwood CH, Beaton A, Desmond J, et al. Consensus paper: language and the cerebellum: an ongoing enigma. Cerebellum. 2014;13(3):386–410.PubMedPubMedCentralGoogle Scholar
  11. 11.
    Salman MS, Tsai P. The role of the pediatric cerebellum in motor functions, cognition and behavior: a clinical perspective. Neuroimaging Clin N Am. 2016;26(3):317–29.CrossRefGoogle Scholar
  12. 12.
    Morton SM, Bastian AJ. Mechanisms of cerebellar gait ataxia. Cerebellum. 2007;6(1):79–86.CrossRefGoogle Scholar
  13. 13.
    Thach WT. Does the cerebellum initiate movement? Cerebellum. 2014;13(1):139–50.CrossRefGoogle Scholar
  14. 14.
    Bastian AJ. Learning to predict the future: the cerebellum adapts feedforward movement control. Curr Opin Neurobiol. 2006;16(6):645–9.CrossRefGoogle Scholar
  15. 15.
    Bhanpuri NH, Okamura AM, Bastian AJ. Predictive modeling by the cerebellum improves proprioception. J Neurosci. 2013;33(36):14301–6.CrossRefGoogle Scholar
  16. 16.
    Kheradmand A, Zee DS. Cerebellum and ocular motor control. Front Neurol. 2011;2:53.CrossRefGoogle Scholar
  17. 17.
    Urban PP, Marx J, Hunsche S, Gawehn J, Vucurevic G, Wicht S, et al. Cerebellar speech representation: lesion topography in dysarthria as derived from cerebellar ischemia and functional magnetic resonance imaging. Arch Neurol. 2003;60(7):965–72.CrossRefGoogle Scholar
  18. 18.
    Spencer KA, Slocomb DL. The neural basis of ataxic dysarthria. Cerebellum. 2007;6(1):58–65.CrossRefGoogle Scholar
  19. 19.
    Schmahmann JD, Sherman JC. Cerebellar cognitive affective syndrome. Int Rev Neurobiol. 1997;41:433–40.CrossRefGoogle Scholar
  20. 20.
    Marien P, Engelborghs S, Fabbro F, De Deyn PP. The lateralized linguistic cerebellum: a review and a new hypothesis. Brain Lang. 2001;79(3):580–600.CrossRefGoogle Scholar
  21. 21.
    Murdoch BE, Whelan BM. Language disorders subsequent to left cerebellar lesions: a case for bilateral cerebellar involvement in language? Folia Phoniatr Logop. 2007;59(4):184–9.CrossRefGoogle Scholar
  22. 22.
    Stoodley CJ, Schmahmann JD. Functional topography in the human cerebellum: a meta-analysis of neuroimaging studies. NeuroImage. 2009;44(2):489–501.CrossRefGoogle Scholar
  23. 23.
    De Smet HJ, Baillieux H, Wackenier P, De Praeter M, Engelborghs S, Paquier PF, et al. Long-term cognitive deficits following posterior fossa tumor resection: a neuropsychological and functional neuroimaging follow-up study. Neuropsychology. 2009;23(6):694–704.CrossRefGoogle Scholar
  24. 24.
    McAndrew S, Listernick R, Kuntz N. Cerebellar mutism in acute disseminating encephalomyelitis. Pediatr Neurol. 2014;50(5):511–4.CrossRefGoogle Scholar
  25. 25.
    Parrish JB, Weinstock-Guttman B, Yeh EA. Cerebellar mutism in pediatric acute disseminated encephalomyelitis. Pediatr Neurol. 2010;42(4):259–66.CrossRefGoogle Scholar
  26. 26.
    Weier K, Till C, Fonov V, Yeh EA, Arnold DL, Banwell B, et al. Contribution of the cerebellum to cognitive performance in children and adolescents with multiple sclerosis. Mult Scler. 2015;22(5):599–607.CrossRefGoogle Scholar
  27. 27.
    Bolduc ME, Du Plessis AJ, Sullivan N, Khwaja OS, Zhang X, Barnes K, et al. Spectrum of neurodevelopmental disabilities in children with cerebellar malformations. Dev Med Child Neurol. 2011;53(5):409–16.CrossRefGoogle Scholar
  28. 28.
    Bolduc ME, Limperopoulos C. Neurodevelopmental outcomes in children with cerebellar malformations: a systematic review. Dev Med Child Neurol. 2009;51(4):256–67.CrossRefGoogle Scholar
  29. 29.
    Hennes E, Zotter S, Dorninger L, Hartmann H, Häusler M, Huppke P, et al. Long-term outcome of children with acute cerebellitis. Neuropediatrics. 2012;43(5):240–8.CrossRefGoogle Scholar
  30. 30.
    Hoang DH, Pagnier A, Guichardet K, Dubois-Teklali F, Schiff I, Lyard G, et al. Cognitive disorders in pediatric medulloblastoma: what neuroimaging has to offer. J Neurosurg Pediatr. 2014;14(2):136–44.CrossRefGoogle Scholar
  31. 31.
    Hoche F, Frankenberg E, Rambow J, Theis M, Harding JA, Qirshi M, et al. Cognitive phenotype in ataxia-telangiectasia. Pediatr Neurol. 2014;51(3):297–310.CrossRefGoogle Scholar
  32. 32.
    Riva D, Cazzaniga F, Esposito S, Bulgheroni S. Executive functions and cerebellar development in children. Appl Neuropsychol Child. 2013;2(2):97–103.CrossRefGoogle Scholar
  33. 33.
    Catsman-Berrevoets CE, Aarsen FK. The spectrum of neurobehavioural deficits in the posterior fossa syndrome in children after cerebellar tumour surgery. Cortex. 2010;46(7):933–46.CrossRefGoogle Scholar
  34. 34.
    Tavano A, Grasso R, Gagliardi C, Triulzi F, Bresolin N, Fabbro F, et al. Disorders of cognitive and affective development in cerebellar malformations. Brain. 2007;130(Pt 10):2646–60.CrossRefGoogle Scholar
  35. 35.
    Poretti A, Benson JE, Huisman TA, Boltshauser E. Acute ataxia in children: approach to clinical presentation and role of additional investigations. Neuropediatrics. 2013;44(3):127–41.PubMedGoogle Scholar
  36. 36.
    Fogel BL. Childhood cerebellar ataxia. J Child Neurol. 2012;27(9):1138–45.CrossRefGoogle Scholar
  37. 37.
    Esscher E, Flodmark O, Hagberg G, Hagberg B. Non-progressive ataxia: origins, brain pathology and impairments in 78 swedish children. Dev Med Child Neurol. 1996;38(4):285–96.CrossRefGoogle Scholar
  38. 38.
    Steinlin M, Zangger B, Boltshauser E. Non-progressive congenital ataxia with or without cerebellar hypoplasia: a review of 34 subjects. Dev Med Child Neurol. 1998;40(3):148–54.CrossRefGoogle Scholar
  39. 39.
    Wassmer E, Davies P, Whitehouse WP, Green SH. Clinical spectrum associated with cerebellar hypoplasia. Pediatr Neurol. 2003;28(5):347–51.CrossRefGoogle Scholar
  40. 40.
    Shevell MI, Majnemer A. Clinical features of developmental disability associated with cerebellar hypoplasia. Pediatr Neurol. 1996;15(3):224–9.CrossRefGoogle Scholar
  41. 41.
    Fekete R. Ataxia. In: Jankovic J, Greenamyre JT, editors. MedLink neurology. San Diego: MedLink Corporation. Available at www.medlink.com. Last updated: 28th October 2015.
  42. 42.
    Salman MS, Chodirker BN. Neuro-ophthalmological findings in children and adolescents with chronic ataxia. Neuro-Ophthalmology. 2015;39(3):125–31.CrossRefGoogle Scholar
  43. 43.
    Cassidy L, Taylor D, Harris C. Abnormal supranuclear eye movements in the child: a practical guide to examination and interpretation. Surv Ophthalmol. 2000;44(6):479–506.CrossRefGoogle Scholar
  44. 44.
    Salman MS, Chodirker BN, Bunge M. Neuroimaging findings and repeat neuroimaging value in pediatric chronic ataxia. Can J Neurol Sci. 2016;43(6):824–32.CrossRefGoogle Scholar
  45. 45.
    Klassen S, Dufault B, Salman MS. Can latent class analysis be used to improve the diagnostic process in pediatric patients with chronic ataxia? Cerebellum. 2017;16(2):348–57.CrossRefGoogle Scholar
  46. 46.
    Dichgans J. Clinical symptoms of cerebellar dysfunction and their topodiagnostical significance. Hum Neurobiol. 1984;2(4):269–79.PubMedGoogle Scholar
  47. 47.
    Doherty D, Millen KJ, Barkovich AJ. Midbrain and hindbrain malformations: advances in clinical diagnosis, imaging, and genetics. Lancet Neurol. 2013;12(4):381–93.CrossRefGoogle Scholar
  48. 48.
    Ilg W, Bastian AJ, Boesch S, Burciu RG, Celnik P, Claaßen J, et al. Consensus paper: management of degenerative cerebellar disorders. Cerebellum. 2014;13(2):248–68.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Section of Pediatric NeurologyChildren’s HospitalWinnipegCanada
  2. 2.Department of Pediatrics and Child Health, Max Rady College of Medicine, Rady Faculty of Health SciencesUniversity of ManitobaWinnipegCanada

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