# Clinical, Cognitive and Behavioural Assessment in Children with Cerebellar Disorder
## 3 Developmental Cerebellar Cognitive-Affective Syndrome  
The Role of Cerebellum in Cognition and Emotion: Evidence from Acquired and Congenital Lesions in Children  
Over the last few decades, systematic investigation and substantial evidences have firmly established the role of the cerebellum in higher order functions, such as language, spatial orientation, the ability to organize symbolic functions in sequence, as well as complex emotional and social behaviors [2; 3; 23]. The relevant question today no longer pertains to whether the cerebellum plays a role in cognition and affect, rather how and through which mechanisms it is accomplished, even in developmental age [23; 24]. The first contribute was done by [PERSON] \"dysmetria of thought hypothesys\", followed by the conceptualization of the cerebellar cognitive-affective syndrome (CCAS) [1] described in adult patients with focal cerebellar lesions. CCAS is a clinical entity supporting the role of the cerebellum in cognition and affect and has been replicated across disease types and in patients of different ages (for the complete reference list, see [3]). The substrate of cerebellar role in cognition leads both in its architecture [25] and in the presence of highly organized and functionally specific heterogeneity of its connections with the spinal cord, brainstem and cerebral cortex [3]. Both animal tract tracing and human imaging evidence give insight on the anatomical and functional features of cerebellar connections. The sensorimotor cerebellum mostly in the anterior lobe, adjacent parts of lobule VI, and lobule VIII appears to be an integral part of the distributed neural circuits subserving the motor system (reciprocal connections with motor cortices via the feed-forward motor corticopontine projections and through feed-back to motor regions from cerebellar nuclei via the thalamus); whereas the cognitive cerebellum in the posterior lobe, and the limbic cerebellum (likely represented principally in the vermis), are incorporated into the distributed neural circuits necessary for cognition and emotion (reciprocal feed-forward and feed-back connections with the prefrontal cortex, posterior parietal cortex, superior temporal polymodal regions, cingulate gyrus and posterior parahippocampal area) (for a complete review see [3]). One primary reason for increased susceptibility of the cerebellum is its protracted developmental timeline compared with the neocortex, which expand the window of vulnerability to a spectrum of neurological and developmental insults, such as genetic mutations, toxic and vascular lesions [2; 26]. The cerebellum is among the first brain structures to begin cellular differentiation, and one of the last to fully mature. As such, the developing cerebellum is vulnerable to dysfunction due to genetic and epigenetic factors, toxic in utero environment, focal or global neonatal brain injury, or some combination of aforementionedstressors. This complexity of risk factors acting over the course of development thus results in a broad range of cellular, morphological, and circuit abnormalities [26]. Acquired focal lesions are a formidable study paradigm as they are discrete and can be precisely defined as for localization, facilitating the study of correlation between function and structure. In this line, since the CCAS definition, several evidences have been collected in children with cerebellar acute infections [27; 28], acquired strokes [29] and tumors rejection [30], supporting the cerebellar involvement in all the high cognitive functions. Studies in acquired lesions provides clinical evidence of functional somatotopic organization of the cerebellum, describing cognitive and executive symptoms associated with lesions to the posterolateral cerebellum and impaired regulation of affect particularly evident when the damage to the cerebellum included the vermis [6].