Assessment of Language Disorders in Children with Cerebellar Malformations

Abstract

Although more than 80% of the neurons of the brain are present with the cerebellum, no great attention was paid for the cognitive and linguistic functions that may be played by this “little brain” until recently. In the last few decades and due to the great advance in many fields such as the neuroimaging, neurogenetics and phoniatrics, many cognitive and linguistic functions of the cerebellum was confirmed, which change our question today from whether or not the cerebellum has cognitive function to which part of the cerebellum do what. Many hypotheses have been proposed as explanations for the cognitive and linguistic functions of the different parts of the cerebellum. However, till today, no universal agreement has been achieved. The neural cerebro-cerebellar circuits that link between the different parts of the cerebrum and the cerebellum are considered now as the basis for all potential cerebellar cognitive or linguistic functions. And it is assumed that any abnormality in this bidirectional circuits will cause the cerebellar cognitive affective syndrome “Schmahmann syndrome”. The cerebellum is vulnerable to wide spectrum of congenital malformations due to its protracted development. These malformations have major impacts on normal cognitive and linguistic development. Children with various types of cerebellar malformation almost always have Schmahmann syndrome in addition to the classical cerebellar motor manifestations. ¬The aim of this study was to investigate the impact of cerebellar malformations on the proper development of language and to have an insight into the role of each part the cerebellum in language development and the neuropathological mechanisms involved in the occurrence of language disorders subsequent to cerebellar malformations. This study was conducted on 15 children with various types of cerebellar malformations recruited from Brain Malformation Clinic, NRC. Their age ranged from 3 to 10 years. They were diagnosed according to their clinical-radiological features and were categorized into three groups. Seven patients had cerebellar hypoplasia with vermis mainly involvement (group A), four patients had cerebellovermian hypoplasia (group B), and four patients had pontocerebellar hypoplasia (group C). All the patients were subjected to the protocol of language assessment which was structured and applied in the Phoniatric Unit, Ain Shams University. Then the cases were statistically analyzed. Result showed that all the patients had delayed motor developmental milestones with various degrees of cerebellar motor manifestations. Cerebellar dysarthria was evident in speech of all patients whose verbal output allowed evaluation. The articulatory and prosodic features were more severely affected than voice quality and breathing. Impercised consonant, slow rate of speech, and excess equal stress were the main features. The cognitive development ranged from normal to profound ID. All the patients had their language development markedly below their chronological age for expressive and receptive domains. Eighty percent of the study population had the expressive language affected more than the receptive language, while twenty percent had the receptive language more affected due to dysmetria of attention and/or emotion. The syntax was more affect