Possible association between the integrity of cerebellar pathways and neurocognitive performance in children with posterior fossa tumors.

BACKGROUND: Cerebellar tumor survivors often exhibit neuropsychological deficits that could be related to alterations in cerebro-cerebellar networks. This is a pilot study designed to understand if diffusion tensor imaging (DTI)-based tractography is able to identify possible correlations between cerebellar white matter structure and cognitive outcome in children on long-term follow-up for posterior fossa (PF) tumors who were thoroughly assessed for neuropsychological functioning. METHODS: DTI-based tractography was performed in pediatric patients with PF tumors. Fractional anisotropy (FA) and volumetric measurements of spinocerebellar, dentorubrothalamocortical and corticopontocerebellar tracts were analyzed. Cognitive and neuropsychological functioning was assessed by the Wechsler Intelligence Scale for Children-IV Edition (WISC-IV) and the Developmental Neuropsychological Assessment (NEPSY II). The associations between Full-Scale Intelligence Quotient (FSIQ), NEPSY-II scores, and fiber tracts were tested by the Spearman rank correlation coefficient. RESULTS: Seven patients (median age at diagnosis five years, range, 3-13) treated for medulloblastoma (2/7; 29%) and pilocytic astrocytoma (5/7; 71%) were retrospectively evaluated. All children had complete surgery. The median FSIQ was 84 (range, 67-93). Patients presented with several deficits on many NEPSY-II tasks; in particular, memory was impaired in nearly half of them. FSIQ and neurocognitive tasks significantly correlated with specific corticopontocerebellar tracts. CONCLUSION: Children on follow-up for PF tumor showed scattered cognitive impairments, including deficits in long-term and immediate memory. Tractography allowed us to describe a possible association between the integrity of cerebellar pathways and neurocognitive performance, suggesting that the myelinization of these fibers may represent an indicator for the development of long-term cognitive sequelae.

A multimodal approach to the study of children treated for posterior fossa tumor: A review of the literature and a pilot study.

OBJECTIVE: The aims of the present study were: (1) to review the literature on long-lasting cognitive sequelae in children treated for Posterior Fossa Tumor and (2) to investigate anatomic functional relations in a case series of 7 children treated for PFT using magnetic resonance imaging (MRI) post-processing methods. METHODS: We retrospectively analyzed MRIs of children who underwent complete surgical resection of PFT and performed extensive neuropsychological evaluation. Tumor, ventricular volumes, and VPS insertion site were drawn on T1 volumetric MRI scans and normalized to a pediatric template. Children showed worse performances on tasks tapping executive functions, memory, visuo-motor precision, and expressive language. RESULTS: Volumes of interest related to these functions showed a maximum overlap on the left vermis and the lateral ventricle enlargement, except for impaired narrative fluency -which was associated with left lateral ventricle enlargement- and narrative memory -which was related to the right vermis and the enlarged fourth ventricle. CONCLUSION: Results suggest that anatomic functional relations in children treated for PFT are related to a combination of different pathophysiological factors.

Toward a characterization of language development in children with congenital heart disease: A pilot study.

Congenital heart diseases (CHDs) represent one of the most common types of congenital abnormalities. More than 90% of children with critical heart defects achieve adulthood due to improvements in medical and surgical treatments. Nonetheless, survivors are at risk for neurodevelopmental disorders that may lead also to language impairments. The current study analyzed the linguistic profiles of a group of school-aged children treated surgically for CHD.Fifteen Children with CHDs (7 girls and 8 boys; mean age = 9.31 with SD = 2.10), without intellectual disability (i.e., IQ>70), who underwent cardiac surgery on cardiopulmonary bypass (CPB), were administered a selection of tests from the language assessment tool BVL 4-12. Their performance was compared with that of 15 healthy children matched for age and gender. As a result, children with CHDs scored significantly lower than healthy peers on tasks tapping lexical and grammatical processing as well as the episodic buffer component of working memory. Interestingly, the two groups did not differ on tasks assessing their lexical repertoire and phonological discrimination abilities. These findings are discussed in light of current theories of cognitive development and functioning.

Neuropsychological Impairment in Children With Class 1 Congenital Heart Disease.

Children with congenital heart disease (CHD) are at increased risk of neuropsychological impairments, but few studies are available on the nonlinguistic neuropsychological abilities of children with CHD. We conducted neuropsychological evaluations using the NEPSY II on a cohort of 17 school-age Italian children with diagnoses of Class 1 CHD, no genetic or chromosomal abnormalities, and normal intelligence scores, and we compared them with 34 matched controls. Children with CHD in this study had undergone at least one cardiac surgery with cardiopulmonary bypass support. We found that children with CHD scored significantly lower than their matched controls on memory and learning domain tasks, and they displayed subtle attention/executive dysfunctions and deficits in sensorimotor skills.

Productive and defective impairments in the neglect syndrome: graphic perseveration, drawing productions and optic prism exposure.

The effects of adaptation to prisms displacing rightwards the field of vision on omission errors, and on perseveration and other graphic productions in a line cancellation task, were assessed in nine right-brain-damaged patients with left unilateral spatial neglect. Prism adaptation improved both neglect, as indexed by omission errors, and perseveration behaviour, up to a delay of 60 min. No correlation was found between omission and perseveration errors in all assessments. The suggestion is made that perseveration and other complex graphic productions made by right brain-damaged-patients with left spatial unilateral neglect is due to a defective monitoring of complex motor behaviour, frequently associated to cerebral damage involving the right frontal lobe. Interpretations of perseveration behaviour in terms of allochiria and directional hypokinesia are considered, and their limits discussed.

Neuropsychological assessment of children with epilepsy and average intelligence using NEPSY II.

INTRODUCTION: Many studies have shown altered neuropsychological functioning of children with epilepsy even in the absence of intellectual disability, with notable concerns for both patients and their families. Although studies have described the cognitive profiles associated with specific epilepsy syndromes, there is incomplete agreement on the relation between spared and impaired abilities in different cognitive domains and on how deficits in one neuropsychological ability can secondarily affect performance in other cognitive domains. The aim of this study was to investigate the neuropsychological profile and vulnerabilities of children with epilepsy without intellectual disability or borderline intellectual functioning. METHOD: 23 children aged 7-15 years, with a diagnosis of epilepsy of genetic or unknown cause and average intellectual functioning (IQ >85), were administered all age-appropriate tests of the Italian-language version (Urgesi, Campanella, & Fabbro, 2011) of a comprehensive neuropsychological battery (NEPSY-II: A Developmental Neuropsychological Assessment, Second Edition; Korkman, Kirk, & Kemp, 2007). Their performance was compared with that of a control group matched for gender, age, handedness and education. RESULTS: Children with epilepsy showed significant impairments as compared to the control group in tests of attention and executive functions and sensorimotor skills. Notably, particular difficulties were observed also in social perception tasks that require affect recognition, an ability that has been so far poorly considered in children with epilepsy. CONCLUSIONS: The results highlight the importance of performing extensive evaluation of cognitive functions, including social cognition processes, in children with epilepsy with average intelligence in order to design appropriate interventions aimed at minimizing long-term consequences on educational and behavioral outcome.