Hugo R Van Dongen

The syndrome of 'cerebellar' mutism and subsequent dysarthria

“Cerebellar” mutism refers to a specific childhood disorder in which a complete but transient loss of speech, followed by dysarthria, occurs after removal of a cerebellar tumor. We present a consecutive series of 15 children with this disorder, which we prefer to designate “mutism and subsequent dysarthria.” The conditions in which it develops suggest also an extracerebellar component of cerebellar mutism. Hydrocephalus at presentation, localization of tumor adjacent to the fourth ventricle, and postsurgical edema of the pontine tegmentum are involved in its development.

Transient Loss of Speech Followed by Dysarthria after Removal of Posterior Fossa Tumour

The authors report three children who suffered transient loss of speech during six to eight weeks following removal of a large midline cerebellar tumour. None manifested speech difficulties immediately after surgery, but all developed mutism within 24 to 48 hours. The speech of all children slowly but completely recovered, after a period of severe dysarthria. The re‐organization of speech functions is discussed in relation to the functioning of musculature.

Dysarthria in Children With Cerebellar or Brainstem Tumors

Speech features were perceptually analyzed in two groups of children. The first group (n = 6) had undergone cerebellar tumor resection, and the second group (n = 6) included children with brainstem tumors. Children belonging to the first group became dysarthric after a postoperative mute phase. Slow speech rate was a specific feature, but scanning speech and irregular articulatory breakdown (i.e., prominent characteristics in adult ataxic dysarthria) were not observed. In the second group, hypernasality was a prominent characteristic and resembled flaccid dysarthria in adults. These findings suggest that acquired childhood dysarthria needs a proper classification.

Acquired childhood dysarthria: Review of its clinical presentation

The adult classification of dysarthria correlating with the pathophysiology of the motor systems is usually applied to classify acquired childhood dysarthria. However, the validity of this adult model for children has not been studied systematically. All studies pertaining to analysis of speech features in acquired childhood dysarthria published since 1980 were reviewed. Studies were classified on the basis of neuroradiologic evidence of lesion site and associated motor disorder. This review demonstrates that knowledge of acquired childhood dysarthria is based on a limited number of single case studies, most of which pertain to dysarthria occurring after resection of cerebellar tumor. Definite similarities to adult dysarthria were not evident. Some similarity to acquired childhood dysarthria due to basal ganglia lesions was detected. We conclude that acquired childhood dysarthria requires its own classification.

Review of research on the clinical presentation of acquired childhood aphasia

Aims– The traditional description of the clinical picture of acquired childhood aphasia (ACA) claims that ACA is invariably nonfluent, that recovery of language disorder is rapid and complete, and that ACA commonly occurs after right hemisphere damage. However, since the late 1970s the publication of several case studies has led to reject this longstanding standard doctrine. This review, which concerns the revised insights into ACA, aims at making an inventory of the recently described aphasic symptomatologies and neuroradiological data. Material– The literature on ACA with emphasis on the studies published since 1978. Results– Recent case studies show a great variety of aphasic symptomatologies including auditory comprehension disorders, paraphasias, neologisms, logorrhoea, jargon, impaired repetition abilities, and a host of linguistic deficits in reading and writing. Not only the typology of the aphasias but also the recently established clinicoradiological correlations appear to resemble those found in adults. Also, recovery from ACA shows to be less complete than previously thought. Conclusion– These findings bear consequences as to theories on cerebral organization of language in childhood. It appears that already in infancy the two cerebral hemispheres are no equal substrate for language representation. Therefore, prognosis and final outcome of ACA are not uniformly favourable.

Transient Cerebellar Eye Closure and Mutism after Cerebellar Tumor Surgery: Long-Term Clinical Follow-Up of Neurologic and Behavioral Disturbances in a 14-Year-Old Girl

Transient cerebellar eye closure (TCES) is a complication of cerebellar tumor surgery in children and is almost exclusively observed in the context of the syndrome of mutism and subsequent dysarthria. As knowledge about the course of transient cerebellar eye closure is absent, we describe in detail the clinical picture in a 14-year-old girl. The process of improvement of TCES is characterized by four distinct phases, can last more than 1 month and may be associated with severe persistent cerebellar dysfunction.

The many faces of acquired neurologic mutism in childhood

Acquired neurologic mutism in childhood is a complex phenomenon occurring in various neurologic conditions with different etiologies. We illustrate its clinical heterogeneity as reflected in a wide range of concomitant behavioral features by presenting 4 children with acquired neurologic mutism. Neuropsychologic examinations revealed differential patterns of defective or preserved phonation, orofacial movements, communicative behavior, and linguistic functions. We propose that detailed neuropsychologic analysis contributes to descriptions of the evolution of the speech impairment beyond the mute phase and the long-term disability. A framework for the clinical evaluation of children is therefore presented.

Current trends in acquired childhood aphasia: An introduction

Abstract Although the interest in acquired childhood aphasia (ACA) arose about a century ago, it has received considerably less attention in the literature on language disorders in childhood than has developmental aphasia. Yet the study of ACA provides a unique opportunity to investigate brain/language relationships during the process of cerebral maturation. Hence, this paper aims to give an overview of recent advances in the study of ACA. The first part focuses on acquired aphasia with convulsive disorder, because most studies on ACA published in the past decade concern this intriguing syndrome. The second part concentrates on several aspects of ACA due to other aetiologies.

AN ANALYSIS OF SPONTANEOUS CONVERSATIONAL SPEECH FLUENCY IN CHILDREN WITH ACQUIRED APHASIA

We report on an instrumental analysis of spontaneous conversational speech (SCS) fluency in acquired childhood aphasia (ACA). Tape-recorded SCS samples of 25 children with ACA (clinical judgment: 12 nonfluent and 13 fluent), and of 12 dysarthric and 12 nonaphasic and nondysarthric right hemisphere injured children were analysed in order to: (1) investigate whether a more refined analysis can objectively contribute to the differentiation of patients who were labelled as fluent or nonfluent on the basis of a clinical judgment: (2) verify whether an instrumental analysis of phonation duration does confirm the subjective estimation of verbal rate (i.e. the number of words produced in a unit of time) in groups of children with acquired neurogenic speech/language disorders frequently met in clinical practice. The results are: (1) phonation rate (i.e. the vocalization percentage) seems to represent an adequate variable to distinguish clinically diagnosed nonfluent aphasic children from speech/language impaired children belonging to other clinical groups of acquired neurogenic speech/language disorders; (2) the verbal rate is highly correlated to the phonation rate in all investigated groups except the dysarthric one. We suggest the instrumental method discussed here might contribute to the differential diagnosis between dysarthric and aphasic disturbances in the acute stage of the disease. Concerning the study of ACA, the main issue of the present investigation is that an objective fluency measurement has succeeded in identifying aphasic children who obviously do not fit in with the standard doctrine on ACA, which claims that ACA is invariably nonfluent irrespective of lesion location.

Clinical Evaluation of Conversational Speech Fluency in the Acute Phase of Acquired Childhood Aphasia: Does a Fluency/Nonfluency Dichotomy Exist?

Traditional neurologic tenets claim that the clinical picture of acquired childhood aphasia is nonfluent irrespective of lesion location. In the past 20 years, however, several case studies have shown that fluent aphasic patterns can be observed in children with acquired childhood aphasia. But the question remains open as to whether the pattern of their speech characteristics is similar to the one described in adult aphasics as studies addressing spontaneous speech fluency characteristics in larger series of children with acquired childhood aphasia are scarce. The objective of this study was to investigate whether an analysis of spontaneous speech fluency as has previously been performed in adult aphasics by other investigators would also yield two distinct groups of aphasic children and, if so, whether the distribution of the different speech characteristics in both groups would reflect the rank order found in adults, that is, whether nonfluent verbal output characteristics would predominate in one group and fluent features in the other. Audiotaped and videotaped recordings of 24 cooperative children with acute acquired childhood aphasia unselected for age, gender, etiology, and aphasia severity ratings were analyzed according to 10 different speech characteristics. A cluster analysis (two-means clustering) was performed to seek the existence of two distinct groups of aphasic children. Results were confirmed, and exact P values were computed with Mann-Whitney U-tests. A two-means clustering created two distinct classes. Mann-Whitney U-tests ranked the speech characteristics according to their discriminating power between clusters. Comparing this rank order with the one previously found in adults revealed a high correlation (Spearmans rank correlation: rs = .915, P < < .005), thus indicating that the clusters we found were highly similar to the adult clusters. Thus, the use of the speech variables proposed to evaluate adult aphasic spontaneous speech enabled us to demonstrate a fluent/nonfluent dichotomy in a childhood aphasic population as well. This study shows that the traditional views on the uniformity of the clinical picture of acquired childhood aphasia are obsolete. Our findings corroborate data issued from several case reports of fluent acquired childhood aphasia and from the few studies focusing on speech fluency in acquired childhood aphasia, which all point to the existence of an adultlike heterogeneity of childhood aphasic syndromes. Current clinical evidence no longer supports the hypotheses of equipotentiality and progressive lateralization but favors the notion that the anatomic substrate for language representation in the child is similar to that in adults, even in young subjects. (J Child Neurol 2001;16:345-351).