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The Cerebellum

, Volume 15, Issue 3, pp 369–391 | Cite as

Consensus Paper: Revisiting the Symptoms and Signs of Cerebellar Syndrome

  • Florian Bodranghien
  • Amy Bastian
  • Carlo Casali
  • Mark Hallett
  • Elan D. Louis
  • Mario MantoEmail author
  • Peter Mariën
  • Dennis A. Nowak
  • Jeremy D. Schmahmann
  • Mariano Serrao
  • Katharina Marie Steiner
  • Michael Strupp
  • Caroline Tilikete
  • Dagmar Timmann
  • Kim van Dun
Consensus Paper

Abstract

The cerebellum is involved in sensorimotor operations, cognitive tasks and affective processes. Here, we revisit the concept of the cerebellar syndrome in the light of recent advances in our understanding of cerebellar operations. The key symptoms and signs of cerebellar dysfunction, often grouped under the generic term of ataxia, are discussed. Vertigo, dizziness, and imbalance are associated with lesions of the vestibulo-cerebellar, vestibulo-spinal, or cerebellar ocular motor systems. The cerebellum plays a major role in the online to long-term control of eye movements (control of calibration, reduction of eye instability, maintenance of ocular alignment). Ocular instability, nystagmus, saccadic intrusions, impaired smooth pursuit, impaired vestibulo-ocular reflex (VOR), and ocular misalignment are at the core of oculomotor cerebellar deficits. As a motor speech disorder, ataxic dysarthria is highly suggestive of cerebellar pathology. Regarding motor control of limbs, hypotonia, a- or dysdiadochokinesia, dysmetria, grasping deficits and various tremor phenomenologies are observed in cerebellar disorders to varying degrees. There is clear evidence that the cerebellum participates in force perception and proprioceptive sense during active movements. Gait is staggering with a wide base, and tandem gait is very often impaired in cerebellar disorders. In terms of cognitive and affective operations, impairments are found in executive functions, visual-spatial processing, linguistic function, and affective regulation (Schmahmann’s syndrome). Nonmotor linguistic deficits including disruption of articulatory and graphomotor planning, language dynamics, verbal fluency, phonological, and semantic word retrieval, expressive and receptive syntax, and various aspects of reading and writing may be impaired after cerebellar damage. The cerebellum is organized into (a) a primary sensorimotor region in the anterior lobe and adjacent part of lobule VI, (b) a second sensorimotor region in lobule VIII, and (c) cognitive and limbic regions located in the posterior lobe (lobule VI, lobule VIIA which includes crus I and crus II, and lobule VIIB). The limbic cerebellum is mainly represented in the posterior vermis. The cortico-ponto-cerebellar and cerebello-thalamo-cortical loops establish close functional connections between the cerebellum and the supratentorial motor, paralimbic and association cortices, and cerebellar symptoms are associated with a disruption of these loops.

Keywords

Cerebellum Ataxia Eye movements Dysmetria Tremor A- or Dysdiadochokinesia Hypotonia Speech Dysarthria Language Cognition Affect Loops Functional topography Cerebellar syndrome 

Notes

Conflicts Of Interest

The authors declare no relevant conflict of interest.

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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  • Florian Bodranghien
    • 1
  • Amy Bastian
    • 12
  • Carlo Casali
    • 13
  • Mark Hallett
    • 6
  • Elan D. Louis
    • 9
  • Mario Manto
    • 1
    Email author
  • Peter Mariën
    • 16
    • 17
  • Dennis A. Nowak
    • 10
    • 11
  • Jeremy D. Schmahmann
    • 15
  • Mariano Serrao
    • 13
    • 14
  • Katharina Marie Steiner
    • 7
    • 8
  • Michael Strupp
    • 2
  • Caroline Tilikete
    • 3
    • 4
    • 5
  • Dagmar Timmann
    • 7
    • 8
  • Kim van Dun
    • 17
  1. 1.FNRS ULB-Erasme, Unité d’Etude du MouvementBrusselsBelgium
  2. 2.University of MunichMunichGermany
  3. 3.CRNL INSERM U1028 CNRS UMR5292, Team ImpActBronFrance
  4. 4.Lyon I UniversityLyonFrance
  5. 5.Hospices Civils de Lyon, Neuro-Ophthalmology and Neurology D, Hôpital Neurologique Pierre WertheimerBronFrance
  6. 6.Human Motor Control Section, NINDSBethesdaUSA
  7. 7.Department of NeurologyUniversity Clinic EssenEssenGermany
  8. 8.Department of NeurologyUniversity of Duisburg-EssenEssenGermany
  9. 9.Department of NeurologyYale School of MedicineNew HavenUSA
  10. 10.Helios Klinik KipfenbergKipfenbergGermany
  11. 11.Neurologische Universitätsklinik, Philipps-Universität MarburgMarburgGermany
  12. 12.Kennedy Krieger InstituteBaltimoreUSA
  13. 13.Department of Medical and Surgical Sciences and BiotechnologiesRome Sapienza UniversityRomeItaly
  14. 14.Rehabilitation Centre, Movement Analysis LAB, Policlinico ItaliaRomeItaly
  15. 15.Ataxia Unit, Cognitive Behavioural Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology, Massachusetts General HospitalHarvard Medical SchoolBostonUSA
  16. 16.Clinical and Experimental Neurolinguistics, CLIN, Vrije Universiteit BrusselBrusselsBelgium
  17. 17.Department of Neurology and Memory ClinicZNA Middelheim General HospitalAntwerpBelgium

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