Abstract
The cerebellar cognitive affective syndrome (CCAS) includes disruption of linguistic processing such as verbal fluency, verbal working memory, grammar, and speech perception. We set out to examine linguistic capabilities in patients with cerebellar lesions to determine which domains are spared and which impaired and to evaluate the underlying cognitive structure of these deficits. Forty-four patients with cerebellar disease were compared to 40 healthy controls on the Oral Sentence Production Test (OSPT) which assesses production of sentences with correct syntactic structure and semantic quality. Twenty-five of these cerebellar patients and 25 controls received the Test of Language Competence-Expanded (TLC-E) that assesses metalinguistic ability. The OSPT failed to reveal differences between patients and controls. In contrast, all cerebellar patients were impaired on each of the four TLC-E subtests. Differences between isolated cerebellar and complex cerebrocerebellar patients were nonsignificant. These results confirm and extend prior observations of the TLC-E in patients with cerebellar lesions and suggest three separate but related language impairments following cerebellar dysfunction: (1) disruption in automatic adjustment of intact grammatical and semantic abilities to a linguistic context in sentence production, (2) disruption in automatic adjustment to a linguistic context in sentence interpretation, and (3) disruption of cognitive processes essential for linguistic skills, such as analysis and sequential logical reasoning. These findings are consistent with the unifying framework of the universal cerebellar transform and the dysmetria of thought theory and provide new insights into the nature of the cognitive impairments in patients with the CCAS.
Similar content being viewed by others
References
Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain. 1998;121:561–79.
Schmahmann JD, Pandya DN. The cerebrocerebellar system. In: schmahmann JD, ed. The cerebellum and cognition. San Diego, academic press. Int Rev Neurobiol. 1997;41:31–60.
Schmahmann JD. An emerging concept: the cerebellar contribution to higher function. Archiv Neurol. 1991;48:1178–87.
Schmahmann JD. The role of the cerebellum in cognition and emotion: personal reflections since 1982 on the dysmetria of thought hypothesis, and its historical evolution from theory to therapy. Neuropsychol Rev. 2010;20(3):236–60.
Mariën 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.
Stoodley CJ, Schmahmann JD. The cerebellum and language: evidence from patients with cerebellar degeneration. Brain Lang. 2009;110:149–53.
Desmond JE, Gabrieli FD, Glover GH. Dissociation of frontal and cerebellar activity in a cognitive task: evidence for a distinction between selection and search. Neuroimage. 1998;7:368–76.
Wiig E, Secord W. Test of Language Competence-Expanded Edition. 1st ed. Pearson: San Antonio; 1989.
Cook M, Murdoch B, Cahill L, Whelan BM. Higher-level language deficits resulting from left primary cerebellar lesions. Aphasiology. 2004;18:771–84.
Murdoch BE, Whelan BM. Language disorders subsequent to left cerebellar lesions: a case for bilateral cerebellar involvement in language? Folia Phoniatr Logo. 2007;59:184–9.
Caplan D, Hanna JE. Sentence production by aphasic patients in a constrained task. Brain Lang. 1998;63:184–218.
Koeppen AH. The cerebellum and its disorders. Manto MU, Pandolfo M, editors. Cambridge: Cambridge University Press, 2002, pp. 387–406.
Manto M, Gruol DL, Schmahmann JD, Koibuchi N, Rossi F. Handbook of the cerebellum and cerebellar disorders. 1st ed. New York: Springer; 2013.
Cohen J. Statistical power analysis for the behavioral sciences. 2nd ed. Hillsdale: Lawrence Erlbaum Associates; 1988.
Mariën P, Engelborghs S, Fabbro F, De Deyn PP. The lateralized linguistic cerebellum: a review and a new hypothesis. Brain Lang. 2001;79:580–600.
Stoodley CJ, Valera EM, Schmahmann JD. Functional topography of the cerebellum for motor and cognitive tasks: an fMRI study. Neuroimage. 2012;59:1560–70.
Acknowledgments
The authors gratefully acknowledge Casey Evans, BA for her blinded scoring of subject performance, and Mark Vangel, PhD, for statistical assistance. The assistance of Jason MacMore, BA and Marygrace Neal, MEd is also gratefully acknowledged. This work was supported in part by RO1-MH067980,the National Ataxia Foundation, and the Birmingham and MINDlink Foundations.
Conflict of Interest
The authors declare that there are no conflicts of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Guell, X., Hoche, F. & Schmahmann, J.D. Metalinguistic Deficits in Patients with Cerebellar Dysfunction: Empirical Support for the Dysmetria of Thought Theory. Cerebellum 14, 50–58 (2015). https://doi.org/10.1007/s12311-014-0630-z
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12311-014-0630-z