Language and social communication in children with cerebellar dysgenesis

Objective: Acquired cerebellar lesions in children and adults may determine deficits of executive functions, visuoperceptual skills, expressive language and modulation of affect; a complex pattern termed 'cerebellar cognitive affective syndrome'. However, the long-term sequelae of malformative cerebellar lesions have yet to be systematically investigated, particularly in children. The purpose of this study was to present preliminary longitudinal data on the development of language and social communication skills in children with congenital malformations confined to the cerebellum. Patients and Methods: Five children ( 3 males, 2 females) with cerebellar malformations confined to the cerebellum were selected. Three patients presented with cerebellar hypoplasia involving the vermis and the hemispheres, while the remaining 2 had a malformation affecting only the cerebellar hemispheres. Neurobehavioral and language development were traced through access to available clinical data. Results: In the patients with cerebellar vermis malformation, language and social communicative skills were affected to a variable extent: 1 patient did not present with social disturbances during development. Those with hemispheric cerebellar lesions presented with selective linguistic impairments. Conclusions: The neurobehavioral profile of children with cerebellar malformations supports a key role of the cerebellum in language acquisition and affect regulation as distinguished functional domains. Copyright (c) 2007 S. Karger AG, Basel.