Tavano Alessandro, Fabbro Franco, Borgatti Renato
Neurolinguistics Unit, San Vito al Tagliamento, Pordenone, Italy.
Folia Phoniatr Logop. 2007;59(4):201-9. doi: 10.1159/000102932.
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.
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.
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.
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.
儿童和成人后天性小脑病变可能导致执行功能、视觉感知技能、语言表达及情感调节方面的缺陷；这是一种被称为“小脑认知情感综合征”的复杂模式。然而，小脑发育畸形性病变的长期后遗症尚未得到系统研究，尤其是在儿童中。本研究的目的是呈现关于先天性小脑局限畸形患儿语言和社交沟通技能发展的初步纵向数据。
选取5例小脑局限畸形患儿（3例男性，2例女性）。3例患者表现为小脑蚓部及半球发育不全，其余2例仅小脑半球存在畸形。通过获取现有临床资料追踪神经行为和语言发育情况。
小脑蚓部畸形患者的语言和社交沟通技能受到不同程度影响：1例患者在发育过程中未出现社交障碍。小脑半球病变患者表现出选择性语言障碍。
小脑畸形患儿的神经行为特征支持小脑在语言习得和情感调节作为不同功能领域中起关键作用。
