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DOI: 10.1055/s-0045-1811727
Cerebellar syndromes: clinical observations leading to the recognition of the three types
Authors
Funding The authors declare that the present study was supported by JSPS KAKENHI, under grant number 24K02668.

Abstract
Cerebellar syndrome is traditionally categorized into three primary types: cerebellar motor syndrome (CMS), vestibulocerebellar syndrome (VCS), and cerebellar cognitive affective syndrome (CCAS) or Schmahmann syndrome (SS). The first type is subdivided into five elemental features: dysmetria, kinetic tremor, asynergia, adiadochokinesis and dyschronometria. The second is characterized by dysmetria of saccades and jerky pursuit, as well as downbeat nystagmus and gaze-evoked nystagmus. And the third type is associated with a broader spectrum of cognitive and affective symptoms, including impairments in executive function, spatial cognition, language processing and emotional regulation. In its extreme form, cerebellar mutism can also develop during childhood following cerebellar vermis surgery. Recent physiological studies have shed light on the underlying neural mechanisms of these syndromes by identifying a common link of dysfunction within the cerebellum's internal forward model. This is essential to the prediction of the outcomes of motor and cognitive actions and underlines dysmetria as the core common element. Despite the diversity in clinical presentation, cerebellar syndromes can be understood as disruptions of a unified neural mechanism, providing a new framework for better understanding of cerebellar deficits.
Keywords
Cerebellum - Cerebellar Ataxia - Vestibular System - Cognitive Dysfunction - Mood dMsorders - Internal ModelsData Availability Statement
The concepts discussed in the present article are not based on raw data.
Authors' Contributions
Conceptualization: MM, HM; Writing – original draft: MM, HM; Writing – review & editing: MM, HM; Approval of the final version: MM, HM.
Editor-in-Chief: Hélio A. G. Teive https://orcid.org/0000-0003-2305-1073.
Associate Editor: Laura Silveira Moriyama https://orcid.org/0000-0003-2184-626X.
Publikationsverlauf
Eingereicht: 13. April 2025
Angenommen: 15. Juli 2025
Artikel online veröffentlicht:
19. September 2025
© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)
Thieme Revinter Publicações Ltda.
Rua Rego Freitas, 175, loja 1, República, São Paulo, SP, CEP 01220-010, Brazil
Mario Manto, Hiroshi Mitoma. Cerebellar syndromes: clinical observations leading to the recognition of the three types. Arq Neuropsiquiatr 2025; 83: s00451811727.
DOI: 10.1055/s-0045-1811727
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References
- 1 Garcin R. The ataxias. Handb Clin Neurol 1969; 1 (01) 309-355
- 2 Duchenne de Boulogne GBA. De l'ataxie locomotrice progressive. Recherches sur une maladie caractérisée spécialement par des troubles généraux de la coordination des mouvements. Arch Gén Méd 1859; 5 (13) 36-451
- 3 Babinski J. Del'asynergie cérébelleuse. Rev Neurol (Paris) 1899; 7: 806-816
- 4 Babinski J. Exposé des travaux scientifiques. Paris: Masson et Cie.; 1913
- 5 André-Thomas. La pathologie du cervelet. In: Roger GH, Widal F, Teissier PJ. Nouveau traité de médecine. Paris: Masson et Cie.; 1925. Vol. 39. , p. 755
- 6 Holmes G. The symptoms of acute cerebellar injuries due to gunshot injuries. Brain 1917; 40 (04) 461-535
- 7 Strick PL, Dum RP, Fiez JA. Cerebellum and nonmotor function. Annu Rev Neurosci 2009; 32: 413-434
- 8 Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain 1998; 121 (Pt 4): 561-579
- 9 Daly MP, Sherman JC, Schmahmann JD. The Cerebellar Neuropsychiatric Rating Scale. Cerebellum 2025; 24 (02) 56
- 10 Fritze S, Ferrucci R, Manto M. The cerebellar neuropsychiatric rating scale: a new card in the deck. Cerebellum 2025; 24 (02) 57
- 11 Manto M, Mitoma H. Cerebellum: From the identification of the cerebellar motor syndrome to the internal models. Handb Clin Neurol 2023; 196: 159-174
- 12 Cabaraux P, Gandini J, Kakei S, Manto M, Mitoma H, Tanaka H. Dysmetria and errors in predictions: the role of internal forward model. Int J Mol Sci 2020; 21 (18) 6900
- 13 Mitoma H, Kakei S, Yamaguchi K, Manto M. Physiology of cerebellar reserve: redundancy and plasticity of a modular machine. Int J Mol Sci 2021; 22 (09) 4777
- 14 Schmahmann JD. The role of the cerebellum in affect and psychosis. J Neurolinguist 2000; 13 (2–3): 189-214
- 15 Schmahmann JD. The cerebellum and cognition. Neurosci Lett 2019; 688: 62-75
- 16 Manto M, Godaux E, Jacquy J. Cerebellar hypermetria is larger when the inertial load is artificially increased. Ann Neurol 1994; 35 (01) 45-52
- 17 Mitoma H, Hayashi R, Yanagisawa N, Tsukagoshi H. Characteristics of parkinsonian and ataxic gaits: a study using surface electromyograms, angular displacements and floor reaction forces. J Neurol Sci 2000; 174 (01) 22-39
- 18 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 (03) 369-391
- 19 Luciani L. Il cervelletto. Nuovi studi di fisiologia normale e patologica. Florence: Le Monnier; 1891
- 20 Holmes G. The Croonian lectures on the clinical symptoms of cerebellar disease and their interpretation. Lecture III. Lancet 1922; 200 (5158) 59-65
- 21 Hore J, Wild B, Diener HC. Cerebellar dysmetria at the elbow, wrist, and fingers. J Neurophysiol 1991; 65 (03) 563-571
- 22 Popa LS, Hewitt AL, Ebner TJ. Predictive and feedback performance errors are signaled in the simple spike discharge of individual Purkinje cells. J Neurosci 2012; 32 (44) 15345-15358
- 23 Tanaka H, Ishikawa T, Kakei S. Neural evidence of the cerebellum as a state predictor. Cerebellum 2019; 18 (03) 349-371
- 24 Murphy O, Kheradmand A. Cerebellum and oculomotor deficits. In: Gruol DL, Koibuchi N, Manto M, Molinari M, Schmahmann JD, Shen Y. editors. Essentials of Cerebellum and Cerebellar Disorders. 2nd edition.. Cham: Springer; 2023: 451-455
- 25 Kheradmand A, Zee DS. Cerebellum and ocular motor control. Front Neurol 2011; 2: 53
- 26 Zee DS, Shaikh A. Neurology of eye movements: From control systems models to ion channel and genetics to targeted pharmacology. In: Werner JS, Chalupa LM. editors. The new visual neuroscience. Cambridge, MA: MIT Press; 2013: 997-990
- 27 Schmahmann JD. The Cerebellar Cognitive Affective Syndrome and the Neuropsychiatry of the Cerebellum. In: Gruol DL, Koibuchi N, Manto M, Molinari M, Schmahmann JD, Shen Y. editors. Essentials of Cerebellum and Cerebellar Disorders. 2nd edition.. Cham: Springer; 2023: 485-492
- 28 Catsman-Berrevoets C, Patay Z. Cerebellar mutism syndrome. Handb Clin Neurol 2018; 155: 273-288
- 29 Frosch IR, Mittal VA, D'Mello AM. Cerebellar contributions to social cognition in ASD: A predictive processing framework. Front Integr Nuerosci 2022; 16: 810425
- 30 Koster-Hale J, Saxe R. Theory of mind: a neural prediction problem. Neuron 2013; 79 (05) 836-848
- 31 Leggio M, Molinari M. Cerebellar sequencing: a trick for predicting the future. Cerebellum 2015; 14 (01) 35-38
- 32 Mitoma H, Buffo A, Gelfo F, Guell X, Fucà E, Kakei S. et al. Consensus paper. Cerebellar reserve: From cerebellar physiology to cerebellar disorders. Cerebellum 2020; 19 (01) 131-153