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Cerebellar cognitive affective syndrome
Cerebellar cognitive affective syndrome (CCAS), also called "Schmahmann's syndrome"〔Manto M, Mariën P. Schmahmann’s syndrome - identification of the third cornerstone of clinical ataxiology. Cerebellum and Ataxias 2015, 2:2 〕 is a condition that follows from lesions (damage) to the cerebellum of the brain. This syndrome, described by Dr. Jeremy Schmahmann and his colleagues refers to a constellation of deficits in the cognitive domains of executive function, spatial cognition, language, and affect resulting from damage to the cerebellum.〔Schmahmann, J. D. & Sherman, J. C. (1998). The cerebellar cognitive affective syndrome. ''Brain, 121,'' 561-579.〕〔Levisohn, L., Cronin-Golomb, A. & Schmahmann, J. D. (2000). Neuropsychological consequences of cerebellar tumor resection in children. ''Brain, 123,'' 1041-1050.〕〔Schmahmann, J. D. (2001). The cerebellar cognitive affective syndrome: Clinical correlations of the dysmetria of thought hypothesis. ''International Review of Psychiatry, 13,'' 313-322.〕〔Schmahmann, J. D. (2010). 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. ''Neuropsychological Review, 20,'' 236-260.〕〔Wolf, U., Rapoport, M. J., & Schweizer, T. A. (2009). Evaluating the affective component of the cerebellar cognitive affective syndrome. ''Journal of Neuropsychiatry and Clinical Neurosciences, 21,'' 245-253.〕 Impairments of executive function include problems with planning, set-shifting, abstract reasoning, verbal fluency, and working memory, and there is often perseveration, distractibility and inattention. Language problems include dysprosodia, agrammatism and mild anomia. Deficits in spatial cognition produce visual–spatial disorganization and impaired visual–spatial memory. Personality changes manifest as blunting of affect or disinhibited and inappropriate behavior. These cognitive impairments result in an overall lowering of intellectual function.〔〔 CCAS challenges the traditional view of the cerebellum being responsible solely for regulation of motor functions. It is now thought that the cerebellum is responsible for monitoring both motor and nonmotor functions. The nonmotor deficits described in CCAS are believed to be caused by dysfunction in cerebellar connections to the cerebral cortex and limbic system.〔〔〔Schmahmann, J. (1991). An emerging concept. The cerebellar contribution to higher function. ''Archives of Neurology, 48,'' 1178-1187.〕
==Symptoms==
The CCAS has been described in both adults and children.〔 The precise manifestations may vary on an individual basis, likely reflecting the precise location of the injury in the cerebellum.〔 These investigators〔 subsequently elaborated on the affective component of the CCAS, i.e., the neuropsychiatric phenomena. They reported that patients with injury isolated to the cerebellum may demonstrate distractibility, hyperactivity, impulsiveness, disinhibition, anxiety, ritualistic and stereotypical behaviors, illogical thought and lack of empathy, aggression, irritability, ruminative and obsessive behaviors, dysphoria and depression, tactile defensiveness and sensory overload, apathy, childlike behavior, and inability to comprehend social boundaries and assign ulterior motives.〔
The CCAS can be recognized by the pattern of deficits involving executive function, visual-spatial cognition, linguistic performance and changes in emotion and personality. Underdiagnosis may reflect lack of familiarity of this syndrome in the scientific and medical community. The nature and variety of the symptoms may also prove challenging. Levels of depression, anxiety, lack of emotion, and affect deregulation can vary between patients.〔 The symptoms of CCAS are often moderately severe following acute injury in adults and children, but tend to lessen with time. This supports the view that the cerebellum is involved with the regulation of cognitive processes.〔Courchesne, E. & Allen, G. (1997). Prediction and preparation, fundamental functions of the cerebellum. ''Learning & Memory, 4,'' 1-35.〕〔Hokkanen, L. S. K., Kauranen, V., Roine, R. O., Salonen, O., & Kotila, M. (2006). Subtle cognitive deficits after cerebellar infarcts. ''European Journal of Neurology, 13,'' 161-170.〕
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