Cognitive impairment in cerebellar malformations : a logi model based on cognitive testing

Abstract EN

Background: Though the cerebellum has long been recognized long time ago for its major role in motor functions and coordination, recently confirmed that the cerebellum has great rule in higher order functions, it shapes and dominates the functions of many brain areas, especially cognition and behavior, through different connections and tracts between the cerebellum and cerebral cortex.

The complex development of the cerebellum together with the high number of involved genes and the long embryonic development of the cerebellum make the cerebellum vulnerable to a broad spectrum of malformations and disruption.

Neuroimaging plays a key role in the diagnosis of posterior fossa malformations.

Objective: Our scope was to determine to what extent cerebellar malformations affect behavior, cognition, motor functions and quality of life.

Methodology: A total of 60 patients proven with cerebellar malformations were enrolled and classified into five groups according to MRI finding.

Patients were underwent brain MRI, behavioural, cognitive, motor and quality of life assessment by, (CBCL) child behavior check list, Wechsler intelligent quotient test, (SARA) scale for the assessment and rating ataxia and quality of life scoring respectively.

Results: Our finding highlighted that children with cerebellar malformations experience high prevalence of Cogntive, as well as poor quality of life due to functional disabilities, except patients with Dandly walker malformations, although they have favourable negro-developmental outcome but this does not prevent them from experiencing poor quality of life.

Conclusion: Cerebellar malformation is heavily connected to cognition, behavior and affect in addition to impairment in the motor function, especially patients with joubert syndrome they experienced high level of behavior and affect abnormalities so, It can be stated that different types of congenital cerebellar malformations lead to a complex picture that can be incorporated under the picture of cognitive syndrome.

Recommendations: Further studies need to be done in order to confirm role of cerebellum in higher orders functions like behavior, emotion processing and cognition.