# Clinical, Cognitive and Behavioural Assessment in Children with Cerebellar Disorder
## 3 Developmental Cerebellar Cognitive-Affective Syndrome
### Premativity  
As stated before, cerebellar anomalies are an important cause of disability in premature children [6]. Damages involving the cerebellum can be either direct destructive (haemorrhage, stroke) or related to reduced development (underdevelopment). The latter seems to be particularly common and is linked to a particular vulnerability of the cerebellum in premature babies. With respect to this vulnerability, the rapid and complex changes that occur in the cerebellar structure during the fetal development play a central role, in particular between the 20 th and the 40 th gestational weeks when the process of cerebellar foliation is active to guarantee functional differentiation of the cerebellum, just like the convolutions of the cerebral hemispheres [67]. Up to 19% of premature babies with a gestational age of less than 32 weeks have damage to the cerebellum (see [68] for review) and even higher frequencies are found among babies with very low birth weight (<750 g). In particular, cerebellar haemorrhage represents the most commonly acquired lesion of the posterior fossa in preterm children affecting up to 37% of infants born less than 33 weeks' gestational age [34]. In her seminal studies, [PERSON] studied the prevalence, the neuropsychological profile and the long-term effects of cerebellar damage in premature children [69; 70]. It has been shown that cerebellar lesions in premature infants are followed by a significant growth deficit in the contralateral hemisphere evident both at the time of preterm birth and at the correct term age [69]. A cohort of 38 ex-preterm infants with isolated cerebellar damage evident on neonatal MRI, had MRI follow-up performed at the mean age of 35 \(\frac{1}{2}\) months (standard deviation: 13.8 months). Unilateral lesions of the cerebellum are associated with a significant reduction in the volume of gray matter in numerous cortical regions contralateral to the lesion (compared to ipsilateral), i.e., dorsolateral pre-frontal, premotor, sensorimotor, medio-temporal and premotor subcortical regions. On the contrary, in the cases of bilateral lesion, there were no interhemispheric differences in tissue volume for any of the regions considered. These results suggest that the growth deficit of brain regions is the result of the interruption of cerebellum-cortical connectivity in favor of the hypothesis of a single anatomical-functional system. A subsequent study demonstrated a relationship between the remote regional deficit of brain volumetric growth and domain-specific functional disabilities in 40 ex-preterm children with isolated cerebellar damage undergoing neurodevelopmental evaluation and quantitative MRI at the age of 34 months. The authors found significant associations between early signs of autism and volume of the dorsolateral prefrontal cortex, gross motor performance and cortical volumes of the sensorimotor areas, cognitive and language skills, and volumes of the middle premotor and temporal cortices [70]. A review by [PERSON] and colleagues [71] analysed 12 papers divided into three categories: studies on children with direct cerebellar damage, both haemorrhage and cerebellar stroke; studies on children with underdevelopment of the cerebellum due to the presence of a malfunction in another area in the cerebral hemispheres (diaschisis); studies on premature patients who are born with an underdeveloped cerebellum in the absence of direct cerebellar or cerebral lesions. The latter category is noteworthy since many children have cerebellar hypoplasia or hypertrophy linked to genetic and toxic factors that can cause prematurity; in these cases, therefore, it is not prematurity that causes the injury but vice-versa. The direct cerebellar insult, which is most concerning prematurity, may be obviously responsible for motor disorders, but also cognitive, socialization and behavioral disorders, revealing a long-term picture of developmental cognitive-affective cerebellar syndrome [71].