# Clinical, Cognitive and Behavioural Assessment in Children with Cerebellar Disorder
## 2 Clinical Assessment  
The clinical picture of cerebellar disease as it appears in adults can't be identified in very early childhood because the cerebellar system is still incomplete at birth. As a result, even in normal children, cerebellar functions are only acquired during the early years of life and some characteristics of poor motor coordination are physiological in the early stages of development (in the first two years of life). This is why the typical signs of dysfunction (dysmetria and postural instability) only become apparent in a child after the neocereblar functions have normally integrated at higher system levels. The clinical signs of cerebellar dysfunctions may appear in various ways, depending on the prevalent site of the lesion and on the child's stage of motor development [6]. In children, there may also be some degree of compensation, depending on the severity of the lesion and the cognitive strategies implemented. In more detail, in the first year of life, the clinical picture is characterised by: global hypotonia with difficulties in invoking the osteotendinous reflexes; hypotonia that also involves the oral region, with gaping mouth; dribbling; and difficulty in sucking. In some cases, there may be very early signs of nystagmus (both horizontal and rotatory), often associated with ocular dysmetria and slow, irregular oscillatory movements. Already in the first two years of life, there is an evident delay in the acquisition of postural control, albeit to a variable degree, depending on the severity of the clinical picture and any coexisting cognitive retardation. In the second year, the clinical signs are more specific and become grossly obvious. At this age, the signs of oral involvement are more readily apparent (chewing difficulties, impaired movements of the tongue and lips, and limited vocal articulation). When the child attempts to remain seated, there is evidence of trunk ataxia, with oscillations in all directions (titubation). There are also early signs of hypermetria during efforts to move closer to objects, with oscillations of the upper limbs and difficulties with fine manipulation due to intentional tremor. The child will have poor temporal coordination in sequences of movements, accelerating, braking, and making abrupt adjustments (synergy). Defensive reactions are also detectable, but they appear to be slow and scarcely effective. Once the child can stand, the atax picture becomes more obvious; the child generally maintains a wider stance, with limbs rotated outwards, and tends to hold onto something to get about. Oscillations of the trunk and head are apparent, whether the child is standing still or attempting to move, and he/she will attempt to correct posture [7]. Children with cerebellar disorders learn to walk unassisted late, generally beyond three years of age, depending on the severity of the condition; in some cases, the child is never able to walk alone. When the child does succeed in walking, atax gait is characterized by a wider base, tilting in all directions, with adjustments and corrections making him/her appear to walk unevenly, in stops and starts, often with arms raised in a defensive gesture. Although the trunk ataxia is a permanent disability, it tends to improve slightly with time because the child adopts compensation strategies [8]. There may also be associated ocular motility disorders, as disruptions in rapid following movements. From the third year of life onwards, praxis and difficulties in manipulation become increasingly obvious and disabling (impairing the acquisition of personal autonomy), as do the associated the language disorders. In particular, verbal production may be retarded and characterised by dysarthria. Cerebellum has a key role in the eye movements control. It is involved in the mechanism of image fixation to the fovea to guarantee an optimal vision even when the body and/or the target is in movement [9].