Language and Social Communication in Children with Cerebellar Dysgenesis

Abstract

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.

Efeitos de ensino envolvendo equivalência entre palavra ditada, palavra escrita e objeto sobre a inteligibilidade da fala em adolescente com hipoplasia cerebelar

“…The systematic planning and arrangement of environmental elements in the clinical and educational context can allow language/ speech professionals to analyze the variables (i.e., genetic and environmental) involved in each case and implement effective interventions that can alter behavioral speech patterns in patients with different types of disorders. 3,14,17 Among the possibilities of environmental conditions that involve speech and other symbolic behaviors (e.g., relating stimuli and reading, writing, and composing words), the equivalent relations model can operationally contribute to human speech interventions, 22,24,26 specifically with regard to intelligibility as demonstrated in the present study. The strengthening of a network of relations between dictated words, objects, printed words, the actions of reading, writing, and composing (using letters),and vocalization by teaching some of these relations gradually taught the subject to vocalize when presented with objects (naming) that corresponded to the phonemic conventions of the linguistic community (speech intelligibility) and allowed the subject to present meaning and comprehension that are shared by the community.…”

“…Significant impairment in the control of the phonoarticulatory system [9][10][11][12][13][14] and consequent behavioral deficits in speech in individuals with cerebellar hypoplasia are reflected by alterations in rhythm, intonation, and tone (i.e., dysprosody), 10,14,15 phonemic omissions/distortions, and agrammatism, 10,14,15 which are some of the possible speech disorders that can be related to cerebellar hypoplasia. This provides an important behavioral prognosis for professionals who work with this population in the language/speech field 3,4 .…”

“…However, different conditions can interfere with the way that behavioral phenotypes are expressed, such as the social circumstances in which speech occurs, the stimulation offered by the supporting network, the consequences of speech, educational exposure, and clinical interventions. Notably, the majority of studies on cerebellar hypoplasia investigate geneticclinical characteristics, 6,10,11,[13][14][15] with few studies that have reported strategies and therapeutics that are specific for this population 12 …”

Monitoring Maternal Beta Carotene and Retinol Consumption May Decrease the Incidence of Neurodevelopmental Disorders in Offspring

“…Patients who suffer from cerebellar dysgenesis demonstrate language and communication difficulties 14. Retinoic acid regulates cell differentiation within the central nervous system, and excessive levels of retinoic acid are well known to create abnormalities in the hindbrain of the developing primate embryo, including the cerebellum 15.…”

The Cerebellum and Language

“…There, several members of the Aphasia Committee graciously agreed to work during the intercongress period on the role of the cerebellum in language processes. Their commitment to publish their findings resulted in this collection of excellent papers on a difficult Tavano et al [7] present longitudinal data on the development of language and social communication skills in children with congenital malformations of the cerebellum. Their findings point to a specific role for the cerebellum in modulating linguistic and other cognitive abilities.…”