# Clinical, Cognitive and Behavioural Assessment in Children with Cerebellar Disorder
## 4 Diagnostic Approach  
While the signs and symptoms of cerebellar involvement in adults are readily recognised, with a precise anatomo-functional correlation, the semiotics are less clear in children, and vary as a function of age. This is because the particular process of cerebellar maturation (unlike that of the other structures in the central nervous system) begins at a later stage of foetal development and takes longer to complete (after birth) [31]. The typical signs of any dysfunction will consequently only become apparent once the cerebellar functions have become integrated into the complex circuits of the central nervous system. As a result, poor motor coordination in the very early years of life is not necessarily evidence of a dysfunction; it can be seen in healthy children too. In cases where cerebellar damage does exist, the early signs may be very vague. This is partly due to spontaneous compensation phenomena, exploiting the vicarious role of the connecting structures (due to the brain's plasticity). On careful neurological assessment, clinicians are able to attribute a localising value to any signs and symptoms of cerebellar derangements: problems with balance when standing and an associated nystagmus point to damage to the archicerebellar system; asynergy of the limbs combined with ataxia on deambulation suggests that the paleocerebellar system is involved; and dysarthria with an altered spatio-temporal regulation of movements would indicate a lesion affecting the neocerebellar system. The objective neurological signs must be integrated with data from the child's medical history. It is important to seek any relevant family history pointing to a potentially genetic condition, to analyse the child's pre- and perinatal background, and to assess how the clinical condition evolves [72], particularly distinguishing between'static' situations which have likely been derived from malformative conditions and progressive situations characterised by developmental arrest or regression suggestive of degenerative diseases [73,74]. While the patient's family history can be important to identify a disorder of potentially genetic origin, it is equally essential to consider the child's physiological history, especially with regards to any significant pre- or perinatal events, and the age of onset of symptoms. The clinician should check for any changes in the patient's clinical condition, to ascertain whether it is static or progressive, thus the clinical follow-up should also include longitudinal observation and careful monitoring of how the child's development evolves. The availability of scales with validated gait and posture- biomarkers in children is important for diagnostic evaluation. In children with limited concentration and physical endurance, optimally applicable gait and posture biomarkers are characterized as: non-invasive, quick and easy. Until now, insight in the validity of clinically available gait and posture-biomarkers is incomplete. The Scale for the assessment and rating of ataxia (SARA) is an example described as a reliable, quickly assessable, and non-invasive rating scale for patients with ataxia, but there is a clear need to create a specific assessment scale for infant and toddler with items suitable for early developmental stages [75]. An accurate clinical assessment will enable appropriate further instrumental procedures to be arranged. It is common knowledge that the contribution of neuroimaging is crucial to the diagnosis of cerebellar disorders, and neurophysiological investigations can also have a significant role. Extensive and thorough neuroradiological characterisation with magnetic resonance of the brain is the most useful way to identify which further investigations are needed. MRI not only confirms the clinical suspicion of a cerebellar lesion, but also reveals its distribution, extent, and morphology [74]. Cerebellar lesions may occur alone or as part of a more complex malformation of the posterior cranial fossa, or they may be associated with other supratentorial anomalies.