Long-term sequelae in children after cerebellar astrocytoma surgery.

OBJECTIVE: To study long-term effects on neurologic, neuropsychological, and behavioral functioning in children treated for cerebellar pilocytic astrocytoma (CPA) without additional radio- and chemotherapy. METHODS: The authors assessed speech, language, nonverbal intelligence, attention, memory, executive skills, and visual (-spatial) functions in a consecutive series of 23 children. Neurologic and neuropsychological follow-up ranged from 1 year to 8 years and 10 months after resection. RESULTS: Long-term sequelae in the investigated domains were found in all children. Apraxia, motor neglect, and dysarthric features, as well as language, sustained attention, visual-spatial, executive, memory, and behavioral problems, were observed in various combinations and to different degrees. No clear pattern of neurocognitive disturbances could be discerned in this group. In addition, significant relationships were revealed between severity of preoperative hydrocephalus and visual-spatial skills. The high percentage of children who needed special education reflects the severity of the impairments. CONCLUSION: Despite the current opinion of a good quality of life after CPA treatment, careful long-term neurocognitive follow-up is needed in order to inform parents and teachers about the behavioral and cognitive sequelae and to contribute to timely social and educational intervention.

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 (Spearman's rank correlation: r = .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.

Tumour type and size are high risk factors for the syndrome of "cerebellar" mutism and subsequent dysarthria.

OBJECTIVE: "Cerebellar mutis" and subsequent dysarthria (MSD) is a documented complication of posterior fossa surgery in children. In this prospective study the following risk factors for MSD were assessed: type, size and site of the tumour; hydrocephalus at presentation and after surgery, cerebellar incision site, postoperative infection, and cerebellar swelling. METHODS: In a consecutive series of 42 children with a cerebellar tumour, speech and neuroradiological studies (CT and MRI) were systematically analysed preoperatively and postoperatively. Speech was assessed using the Mayo Clinic lists and the severity of dysarthria using the Michigan rating scale. RESULTS: Twelve children (29%) developed MSD postoperatively. The type of tumour, midline localisation, and vermal incision were significant single independent risk factors. In addition, an interdependency of possible risk factors (tumour>5 cm, medulloblastoma) was found. CONCLUSION: MSD often occurs after paediatric cerebellar tumour removal and is most likely after removal of a medulloblastoma with a maximum lesion diameter>5 cm.

Clinical utility of the judgment of line orientation test and facial recognition test in children with acquired unilateral cerebral lesions.

In unselected adult patients with brain damage, the Judgment of Line Orientation Test and the Facial Recognition Test are considered valid instruments for detecting right cerebral hemisphere lesions. It is unknown, however, whether this applies to children as well. Performance levels on the Judgment of Line Orientation Test and the Facial Recognition Test of 18 children with acquired left cerebral lesions and 14 children with acquired right cerebral lesions were reviewed. Subjects were unselected for age, sex, or etiology. Age-related norms were obtained in 81 normal controls, aged 7 to 14 years. Judgment of Line Orientation Test and Facial Recognition Test performance levels did not predict the presence of cerebral pathology per se in our unselected groups with demonstrated unilateral cerebral lesions, nor did they contribute to the prediction of the side of the lesion within the two groups with cerebral lesions. These results cast serious doubt on an important aspect of the clinical utility of both tests in children, namely their discriminative validity in the assessment of etiologically unselected populations with brain damage.

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.

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.

The Landau-Kleffner syndrome or 'acquired aphasia with convulsive disorder'. Long-term follow-up of six children and a review of the recent literature.

We present six patients with acquired aphasia with convulsive disorder (Landau-Kleffner syndrome) and distill the main clinical features from a review of the recent literature. Our series showed that the clinical picture can vary at onset, as well as during the course of the illness, and that the long-term outcome of the aphasia is quite unpredictable, despite the fact that epilepsy and electroencephalographic abnormalities usually regress or disappear with the years. We also call attention to the electroencephalographic phenomenon of electrical status epilepticus during slow sleep, and we suggest that the course of the aphasia may well be linked to the appearance and disappearance of electrical status epilepticus during slow sleep. Therefore, we recommend a sleep electroencephalogram in all children with Landau-Kleffner syndrome. Finally, our findings did not demonstrate the beneficial effect of treatment with anticonvulsants on the aphasia, but recent studies have shown that treatment with corticosteroids, whether combined with anticonvulsants, is effective.