Teachers' report of learning and behavioural difficulties in children treated for cerebellar tumours.

PRIMARY OBJECTIVE: The aim of the study was the validation of the Deasy-Spinetta Questionnaire (DSQ) in 6-11 year olds with attention to the verification of three factors (learning difficulties, socialization and emotionality) and its application in children treated for cerebellar tumour. METHODS AND PROCEDURES: Twenty-nine children aged between 6-11 years were compared with 609 classmates. Teachers completed the DSQ. Patients were evaluated according to Wechsler Scales, the Purdue Pegboard and the International Cooperative Ataxia Rating scale. MAIN OUTCOMES AND RESULTS: In classmates, the DSQ factorial analysis showed three factors: learning, socialization difficulties and disturbing behaviour. Teachers reported more learning difficulties in patients than in classmates. Learning difficulties in patients were greater for mathematics and reasoning than for reading. Patients were described as less active, without evidence of autistic-like behaviour or irritability. The teachers' report of learning difficulties was significantly correlated with IQ scores, but not with neurological deficits. CONCLUSIONS: The proposed DSQ scores are interesting for the assessment of learning and behavioural difficulties in children treated for cerebellar tumours, as they provide complementary ecological information to that given by clinical and neuropsychological testing.

Cognitive and academic outcome after benign or malignant cerebellar tumor in children.

OBJECTIVE: To examine the impact of malignancy and location of the cerebellar tumor on motor, cognitive, and psychologic outcome. BACKGROUND: Although many studies focus on long-term outcome after cerebellar tumor treatment in childhood, the impact of its precise location remains unclear. PATIENTS AND METHODS: Children, aged from 6 to 13 years, with a cerebellar malignant tumor (MT; MT group, n=20) or a cerebellar benign tumor (BT; BT group, n=19) were examined at least 6 months after the end of treatment using the international cooperative ataxia rating scale, the Purdue pegboard for manual skill assessment and the age-adapted Weschler scale. Structural changes in brain anatomy were evaluated and parents and teachers answered 2 independent questionnaires. RESULTS: Parents and teachers reported high rate of learning and academic difficulties, but without any difference with respect to the type of tumor. However, children with cerebellar MT showed increased cognitive and motor difficulties compared with children with cerebellar BT. Cerebellar signs at clinical examination and manual skill impairment were strongly associated with cognitive difficulties. Both motor and cognitive impairments were found to be associated with extension of the lesion to the dentate nuclei. CONCLUSIONS: Dentate nuclei lesions are major risk factors of motor and cognitive impairments in both cerebellar BT and MT.

Injuries to inferior vermis and dentate nuclei predict poor neurological and neuropsychological outcome in children with malignant posterior fossa tumors.

BACKGROUND: Children treated for a malignant posterior fossa tumor (PFT) are at risk of intellectual impairment. Its severity is not explained by age and radiotherapy alone. The current study was designed to define the correlations between the anatomical damage and the neurological/neuropsychological deficits in children with a malignant PFT. METHODS: Sixty-one consecutive children (mean age, 6.0 years) treated for a malignant PFT with surgery, chemotherapy, and radiotherapy underwent a detailed neuropsychological evaluation, including a full-scale intelligence quotient (FSIQ), on average 5.6 years after the diagnosis. The neurological examination was recorded 1 month after surgery and at the time of the neuropsychological evaluation. Cerebellar and brain injuries were scored based on the magnetic resonance imaging (MRI). Correlation of these injuries with neurological and cognitive outcome were performed after adjustment for other potential risk factors (radiotherapy schedule, age, hydrocephalus, duration of symptoms, socioeconomic status, and surgical complications). RESULTS: Neurological deficits were strong predictors of low cognitive performances irrespective of the other risk factors. The extent of cerebellar deficits and fine motor dexterity impairment were correlated with the degree of damage to the dentate nuclei and inferior vermis. The IQ scores were inversely correlated with the severity of the damage to the dentate nuclei; mean FSIQ was 83 if they were both intact and 65 in the case of bilateral damage (P=.009). CONCLUSIONS: Damage to the dentate nuclei and to the inferior vermis, observed on MRI, predict the degree of impairment of neurological and neuropsychological functions in children treated for a malignant PFT.

Parental report of cognitive difficulties, quality of life and rehabilitation in children with epilepsy or treated for brain tumour.

PURPOSE: Paediatric neurological chronic conditions are often associated with physical, cognitive, psychological and behavioural difficulties that may affect quality of life (QOL) of children and their families. In this study, we compare parental report of difficulties and rehabilitation in children with various epileptic syndromes or treated for a benign or malignant brain tumour. METHOD: One hundred fifty-three children aged between 6 and 12 years were included, 119 with epilepsy (non-idiopathic generalized 31, non-idiopathic partial 62, idiopathic 26) and 34 treated for a brain tumour. Parents answered a multidimensional questionnaire on child's autonomy and cognitive or behavioural difficulties, impact of the illness on their own everyday life, and rehabilitation. RESULTS: Learning difficulties were reported by a majority of parents in all groups. Behavioural and autonomy problems were more often reported in the non-idiopathic generalized epilepsy group. Report of tiredness was more frequent and of disrupting behaviour less frequent in the tumour group than in epilepsy. Impact of the child's illness on parents' QOL was strong in all groups, and stronger in case of severe forms of epilepsy. CONCLUSIONS: Parental concerns are important to consider for rehabilitation programmes adapted to each child with these neurological conditions.

Vertical parasagittal hemispherotomy: surgical procedures and clinical long-term outcomes in a population of 83 children.

OBJECTIVE: Hemispherotomy techniques have been developed to reduce complication rates and achieve the best possible seizure control. We present the results of our pediatric patients who underwent vertical parasagittal hemispherotomy and evaluate the safety and global long-term outcome of this technique. METHODS: Eighty-three patients underwent vertical parasagittal hemispherotomy by the same neurosurgeon (OD) between 1990 and 2000. We reviewed all patients between 2001 and 2003 for a standard global evaluation. The general principle is to achieve, through a posterior frontal cortical window, the same line of disconnection as performed with the classic hemispherectomy, while leaving the majority of the hemisphere intact along with its afferent and efferent vascular supply. METHODS: Seventy-four percent of the patients were seizure-free; among them, 77% were seizure-free without further drug treatment. Twelve percent rarely had seizures (Engel Class II) and 14% continued to have seizures (Engel Class III or IV). The results varied according to the etiology, but this variation was not statistically significant. The early postoperative course was uneventful for 94% of the children, and shunt placement was necessary in 15%. We found a correlation between the preoperative delay and the Vineland Adaptive Behavior score: children with a longer duration of seizures had lower performances. CONCLUSION: Vertical parasagittal hemispherotomy is an effective surgical technique for hemispheric disconnection. It allows complete disconnection of the hemisphere through a cortical window with good results in terms of seizure outcome and a comparably low complication rate.

Therapeutic schedules influence the pattern of intellectual decline after irradiation of posterior fossa tumors.

BACKGROUND: To evaluate intellectual decline in children with posterior fossa (PF) tumors treated with different therapeutic protocols. PROCEDURE: Forty children had a complete neuropsychological evaluation prospectively twice, at least 6 months year (y) after the end of their treatment. Patients were classified into four groups according to treatment schedules: Group 1 (n = 7) PF radiotherapy (PFRT) alone at 50 Gy; Group 2 (n = 13) reduced-dose cranio-spinal irradiation (CSI) at 25 Gy with a PF boost; Group 3 (n = 9) standard CSI at 35 Gy and a PF boost; and Group 4 (n = 11) high-dose chemotherapy with stem cell support followed by PFRT at 50 Gy. RESULTS: At the first evaluation (mean interval since diagnosis 3.7 y), the mean Full-Scale Intellectual Quotient (FSIQ) was 80 (SD = 19). Only patients in Group 1 had a normal mean IQ score of 92 (SD = 14). At the second evaluation (mean interval since diagnosis 6.3 y), the mean FSIQ scores were significantly lower with a mean difference of 2.4 points, i.e., a yearly decline of one point. The magnitude of the FSIQ decline was positively correlated with the first IQ score (P = 0.0001) and inversely correlated with age at diagnosis (P = 0.0005). A FSIQ decline was observed in all treatment groups except Group 1 (P = 0.005). The differences in FSIQ observed initially between the four treatment groups persisted at the second evaluation. CONCLUSIONS: This study shows that FSIQ continues to decline more than 4 years after the diagnosis but this yearly decline seems to decrease with time from diagnosis. Therapeutic schedules influence the magnitude of this decline. Long-term follow-up into adulthood is necessary to effectively adapt patient rehabilitation.

Critical risk factors for intellectual impairment in children with posterior fossa tumors: the role of cerebellar damage.

OBJECT: Intellectual impairment is a major concern after treatment of malignant posterior fossa tumors in children. The effects of age at diagnosis and radiotherapy have been widely documented. Little is known, however, about perioperative factors, especially neurological damage to the cerebellum, the role of which in cognition and learning has been recently indicated. The authors studied the effects in 76 children treated for a malignant posterior fossa tumor in a cross-sectional study. METHODS: Two thirds of the tumors were medulloblastoma. Neuropsychological evaluation was performed at least 6 months after the end of treatment, and findings were correlated with clinical risk factors for intellectual impairment. The mean verbal intelligence quotient (VIQ) score was 87 +/- 19 (+/- standard deviation) and the mean performance IQ (PIQ) score was 76 +/- 17.5. A single neuropsychological test measuring hand skills (the Purdue Pegboard) was the strongest predictor of low IQ scores including items testing higher cognitive functions. A low VIQ was associated with impaired hand skills (p < 0.0001) and the presence of preoperative hydrocephalus (p = 0.02), whereas a low PIQ was associated with impaired hand skills (p < 0.0001) and incision of the vermis (p = 0.02). Impaired hand skills were associated with postoperative cerebellar mutism, oculomotor deficits, cerebellar syndrome, and therapeutic requirements. CONCLUSIONS: When treatment schedules are adapted to risk of disease and age, surgery-related risk factors then become critical for predicting intellectual impairment. Children with cerebellar damage are particularly at risk for long-term neuropsychological dysfunction and require active rehabilitation measures. Reducing surgery-related morbidity should be the next goal to reduce posterior fossa surgery-specific deficits.

Subjective experience after stroke.

To assess subjective experience after stroke, 214 patients completed the 63-item European Brain Injury Questionnaire. The same questionnaire was also completed by a close relative of each patient and by 214 control participants matched for gender and age. A principal component analysis showed 3 factors: depressive mood, cognitive difficulties, and difficulties in social interactions. In self-assessment, whereas the first 2 factors were scored higher by patients than by controls, the level of difficulty in social interactions did not differ between these 2 groups. In hetero-assessment, relatives scored patients' difficulties higher than patients did, especially for items suggesting the impact of stroke on themselves. Psychological difficulties of stroke patients and the consequences on their relatives need to be taken into account in rehabilitation programs.