# Clinical, Cognitive and Behavioural Assessment in Children with Cerebellar Disorder
###### Abstract  
Cerebellar disorders are characterised clinically by specific signs and symptoms, often associated with neurodevelopmental disorder. While the clinical signs of cerebellar disorders are clearly recognisable in adults and have a precise anatomo-functional correlation, in children the semiotics are less clear and vary with age because of the particular nature of the cerebellum's maturation. Unlike other structures of the central nervous system, this begins at a later stage of foetal development and extends over a longer period of time, even after birth. As a result, the typical signs of cerebellar dysfunction will only become evident when the cerebellar functions have become integrated into the complex circuits of the central nervous system. This means that poor motor coordination in the very early years of life may not necessarily correlate with cerebellar dysfunction, and this may also be encountered in healthy children. The cerebellum's role in cognitive and emotional functions relies on its structure and the complexity of its connections. Cognitive and behavioral impairment in cerebellar disorders can be the results of acquired lesions or the action of genetic and environmental risk factors, to which the cerebellum is particularly vulnerable considering its pattern of development. In the pathological setting, early evidence of cerebellar damage may be very vague, due, partly, to spontaneous compensation phenomena and the vicarious role of the connecting structures (an expression of the brain's plasticity). Careful clinical assessment will nonetheless enable appropriate instrumental procedures to be arranged. It is common knowledge that the contribution of neuroimaging is crucial for diagnosis of cerebellar conditions, and neurophysiological investigations can also have a significant role. The ultimate goal of clinicians is to combine clinical data and instrumental findings to formulate a precise diagnostic hypothesis, and thus request a specific genetic test in order to confirm their findings, wherever possible. ataxia; cerebellar hypoplasia; cerebellar atrophy; developmental delay +
Footnote †: journal: Medicalin processing peripheral information and in the elaboration and execution of motor actions. These areas include:  
* The vestibulocenebellum (flocculonodular lobe): this corresponds to the phylogenetically oldest area. The vestibulocenebellum receives information from the visual and vestibular systems, and its output returns to the vestibular nuclei. This cerebellar structure is essential in maintaining balance and coordinating eye movements with movements of the head and body axis. Lesions involving this structure give rise to ataxia and nystagmus (architerebellar syndrome);
* The spinocereblum: this includes the cerebellar vermis and the intermediate portion of the hemispheres. It receives sensory and proprioceptive inputs from the spinocerebellar pathways as well as the visual, auditory, and vestibular systems, and it sends messages through the deep cerebellar nuclei to the stations of the trunk and the descending systems (reticular substance, red nucleus, and vestibular nuclei) to the thalamus and the cortex. The structures forming the spinocereblum have a functional somatotopic representation with a prevalent representation of the trunk and axial musculature in the vermis, and of the musculature of the limbs in the intermediate area. The function of the spinocereblum is to control and monitor the performance of motor sequences, comparing the spinal marrow inputs and outputs, and modulating the direction and fluidity of the movement. It also has a role in regulating muscle tone by governing the activity of specific motor neurons in the spine. Lesions involving this structure consequently cause ataxia on deambulation, titubation, and limb asnergy, configuring the so-called 'paleocerebellar syndrome';
* The cerebrocereblum: this constitutes the phylogenetically most recent zone. It is represented by the lateral wall of the cerebellar hemispheres.