Cerebellar cognitive affective syndrome (CCAS) is a condition where cognitive and emotional abilities are affected due to cerebellar damage. Historically, the cerebellum was identified as the part of the brain involved in movement and balance, however, CCAS discovery has significantly extended its role in cognition and emotional regulation.
The cerebellar cognitive affective syndrome (CCAS) scale is a comprehensive clinical test to assess the presence and severity of CCAS in individuals with cerebellar disorders. The scale was designed to identify the cognitive and emotional impairments due to cerebellar dysfunction using a wide range of tests. The scale includes tests to look at working memory, planning, cognitive flexibility, and abstract reasoning, essential for goal-directed behavior and adaptive decision-making. Furthermore, the scale expands to tests which focus on visual-spatial organization, memory, and abilities, suggesting the cerebellum’s contribution to spatial processing (the ability to know to understand the location and orientation in space) and awareness.
The CCAS scale is a structured process involving quantitative and qualitative components. The quantitative element includes standardized tasks used to estimate specific spatial cognition, language, and memory. The assessment includes testing linguistic abilities such as verbal fluency, grammar, indicating cerebellum has a key function in language processing and communication. The scale’s qualitative approach involves clinical observations and subjective ratings of the individual’s mood disruptions, emotional ability, and social interactions. It offers advantages, including relatively short administration time, easier for the busy clinicians to test and report the cognitive and affective functioning in patients with cerebellar dysfunctions. Essentially, it monitors symptom progression over time, enabling timely intervention and rehabilitation approaches. Despite the advantages, the scale has limitations such as insufficient normative data due to the absence of data for specific groups of people. This includes children, older people, and populations from diverse cultural backgrounds.
In the future, more priority should be given to upgrading and validating the CCAS scale to cater to different populations and diverse clinical contexts. The CCAS scale systematically evaluates cognitive and emotional impairments from cerebellar damage. It is reliable, valid, and useful in both research and clinics.
If you would like to learn more about CCAS and the CCAS Scale, take a look at this article from the National Ataxia Foundation about the development of the CCAS scale.
Written by Prapti Chakraborty and edited by Asmer Aliyeva.
References
Ahmadian, N., Baarsen, K. van, Zandvoort, M. van, & Robe, P. A. (2019). The Cerebellar Cognitive Affective Syndrome—a Meta-analysis [Review of The Cerebellar Cognitive Affective Syndrome—a Meta-analysis]. The Cerebellum, 18(5), 941. Springer Science+Business Media. https://doi.org/10.1007/s12311-019-01060-2
Argyropoulos, G. P. D., Dun, K. van, Adamaszek, M., Leggio, M., Manto, M., Masciullo, M., Molinari, M., Stoodley, C. J., Overwalle, F. V., Ivry, R. B., & Schmahmann, J. D. (2019). The Cerebellar Cognitive Affective/Schmahmann Syndrome: a Task Force Paper [Review of The Cerebellar Cognitive Affective/Schmahmann Syndrome: a Task Force Paper]. The Cerebellum, 19(1), 102. Springer Science+Business Media. https://doi.org/10.1007/s12311-019-01068-8
Blanchard, J. J., Bradshaw, K., Garcia, C. P., Nasrallah, H. A., Harvey, P. D., Casey, D. E., Csoboth, C., Hudson, J. I., Julián, L., Lentz, E., Nuechterlein, K. H., Perkins, D. O., Skale, T. G., Snowden, L. R., Tandon, R., Tek, C., Velligan, D. I., Vinogradov, S., & O’Gorman, C. (2017). Examining the reliability and validity of the Clinical Assessment Interview for Negative Symptoms within the Management of Schizophrenia in Clinical Practice (MOSAIC) multisite national study. Schizophrenia Research, 185, 137. https://doi.org/10.1016/j.schres.2017.01.011
Clark, C. M., Belcheir, M. J., Strohfus, P. K., & Springer, P. J. (2011). Development and Description of the Culture/Climate Assessment Scale. Journal of Nursing Education, 51(2), 75. https://doi.org/10.3928/01484834-20111230-01
Hoche, F., Guell, X., Vangel, M., Sherman, J. C., & Schmahmann, J. D. (2017). The cerebellar cognitive affective/Schmahmann syndrome scale. Brain, 141(1), 248. https://doi.org/10.1093/brain/awx317
Maas, R. P. P. W. M., Killaars, S., Warrenburg, B. P. C. van de, & Schutter, D. J. L. G. (2021). The cerebellar cognitive affective syndrome scale reveals early neuropsychological deficits in SCA3 patients. Journal of Neurology. https://doi.org/10.1007/s00415-021-10516-7
Manto, M., & Mariën, P. (2015). Schmahmann’s syndrome – identification of the third cornerstone of clinical ataxiology. Cerebellum & Ataxias, 2(1). https://doi.org/10.1186/s40673-015-0023-1
Ocampo, A. C., Restubog, S. L. D., Liwag, M. E., Wang, L., & Petelczyc, C. (2018). My spouse is my strength: Interactive effects of perceived organizational and spousal support in predicting career adaptability and career outcomes. Journal of Vocational Behavior, 108, 165. https://doi.org/10.1016/j.jvb.2018.08.001
Schmahmann, J. D., & Sherman, J. C. (1998). The cerebellar cognitive affective syndrome. Brain, 121(4), 561. https://doi.org/10.1093/brain/121.4.561
Starowicz–Filip, A., Milczarek, O., Kwiatkowski, S., Bętkowska-Korpała, B., & Prochwicz, K. (2013). “Cerebellar cognitive affective syndrome CCAS – a case report.” Archives of Psychiatry and Psychotherapy, 15(3), 57. https://doi.org/10.12740/app/18666
Thieme, A., Roeske, S., Faber, J., Sulzer, P., Minnerop, M., Elben, S., Jacobi, H., Reetz, K., Dogan, I., Barkhoff, M., Konczak, J., Wondzinski, E., Siebler, M., Mueller, O., Sure, U., Schmahmann, J. D., Klockgether, T., Synofzik, M., & Timmann, D. (2020). Validation of a German version of the Cerebellar Cognitive Affective/ Schmahmann Syndrome Scale: preliminary version and study protocol. Neurological Research and Practice, 2(1). https://doi.org/10.1186/s42466-020-00071-3
Wan, N., Chen, Z., Wan, L., Tang, B., & Jiang, H. (2020). MR Imaging of SCA3/MJD [Review of MR Imaging of SCA3/MJD]. Frontiers in Neuroscience, 14. Frontiers Media. https://doi.org/10.3389/fnins.2020.00749
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