Neuropsychological consequences of childhood medulloblastoma and possible interventions: A review.

BACKGROUND: Children who have been treated for a medulloblastoma often suffer long-term cognitive impairments that often negatively affect their academic performance and quality of life. In this article, we will review the neuropsychological consequences of childhood medulloblastoma and discuss the risk factors known to influence the presence and severity of these cognitive impairments and possible interventions to improve their quality of life. METHODS: This narrative review was based on electronic searches of PubMed to identify all relevant studies. RESULTS: Although many types of cognitive impairments often emerge during a child's subsequent development, the core cognitive domains that are most often affected in children treated for a medulloblastoma are processing speed, attention and working memory. The emergence and magnitude of these deficits varies greatly among patients. They are influenced by demographic (age at diagnosis, parental education), medical and treatment-related factors (perioperative complications, including posterior fossa syndrome, radiation therapy dose, etc.), and the quality of interventions such as school adaptations provided to the child or rehabilitation programs that focus on cognitive skills, behavior and psychosocial functioning. CONCLUSION: These patients require specialized and coordinated multidisciplinary rehabilitation follow-up that provides timely and adapted assessments and culminates in personalized intervention goals being set with the patient and the family. Follow-up should be continued until referral to adult services.

[Impact of prenatal corpus callosum agenesis diagnosis on pregnancy outcome. Evaluation of 155 cases between 2000 and 2006]. / Impact du dépistage anténatal des agénésies du corps calleux sur le devenir des grossesses. Etude de 155 dossiers de 2000 à 2006.

The purpose of this study was to investigate the changes between 2000 and 2006 in pregnancy outcome when a diagnosis of either isolated or associated fetal corpus callosum agenesis (CCA) was made, given that beginning in 2003, the information provided to couples facing this problem related a good prognosis in nearly 80 % of cases of isolated CCA and a poor prognosis in 20 % of cases. We retrospectively analyzed all pregnancies with a fetal diagnosis of CCA between 2000 and 2006 (n=155) and compared two periods: the first group from 2000 to June 2003, the second from July 2003 to 2006. For each group, we analyzed the type of CCA during pregnancy - either isolated or associated - and the outcome of pregnancy. We compared the rate of pregnancy termination before and after 2003 and analyzed the accuracy of the prenatal CCA diagnosis. Of the 155 patients studied, 62 terminated the pregnancy. The overall rate of termination decreased from 31/70 to 31/85. When CCA was said to be isolated prenatally, the rate of pregnancy termination fell from 13/35 to 9/44 (-17 %) (p<0.05). Nine diagnoses of CCA were corrected after birth or by postmortem examination. Improvement of prenatal diagnosis requires better quality of prenatal screening, with a more systematic study of dysmorphic features, a study of correlations between the type of CCA and the neurological prognosis, and more genetic studies.

[Childhood cerebral tumors: morbidity and follow-up into adulthood]. / Tumeurs cérébrales de l'enfant: morbidité et suivi à l'âge adulte.

This chapter presents guidelines for the follow-up of children with brain tumors, whether benign or malignant, in their transition to adulthood. The consequences of their disease and its treatment overlap greatly. The complications and long-term follow-up are detailed based on the specialists involved.

[A multidisciplinary consultation for children with brain tumors]. / Une consultation multidisciplinaire pour les enfants traités pour une tumeur cérébrale.

UNLABELLED: School achievement of children with brain tumors is hampered by progressive neurologic and cognitive sequelae. To help the children and their family, we have created in 1997 a multidisciplinary consultation together with Necker's hospital. MATERIAL AND METHODS: The study describes the organization of the consultation and analyses the files of 69 children seen between September 2001 and June 2002. RESULTS AND CONCLUSION: The authors conclude that this consultation is an irreplaceable mean to coordinate the complex rehabilitation process of a child treated for a brain tumor.

Abdominal and fetal echography tele-operated in several medical centres sites, from an expert center, using a robotic arm & telephone or satellite link.

OBJECTIVE: to design and validate a method for tele-operating (from an expert site) an echographic examination in an isolated site where the patient stays. METHOD: A dedicated robotic arm (ESTELE) holding a real ultrasound probe is remotely controlled from the expert site with a fictive probe, and reproduces on the real probe all the movements of the expert hand. The isolated places, are areas with reduced medical facilities, (secondary hospitals 20 to 100 km from the main hospital in Europ, dispensaries in Africa, Amazonia, the a rescue vehicles.... RESULTS: ESTELE was tested on 87 adults and 29 pregnant with ISDN or satellite lines. During fetal tele-operated echography the expert was able to perform appropriate views of the fetal structures in 95% of the cases. During exploration of adult abdomen the expert visualized the main organs in 87% of the cases. Presently the ESTELE system is installed in 4 secondary hospitals, 40 to 100 km from our University Hospital and tele-operated daily by our staff. CONCLUSION: Robotized tele-echography provide similar information as direct examination. No false diagnostic was reported. Moreover the patients were examined by an expert from the University Hospital while staying in the Medical center proximal to their home.

Neuropsychological outcome in children with optic pathway tumours when first-line treatment is chemotherapy.

Standard treatment of optic pathways gliomas consists of radiotherapy and surgery when feasible. Owing to the toxicity of irradiation, chemotherapy has emerged as an interesting therapeutic option, especially in young children. This study describes the neuropsychological profile of 27 children (aged between 1.5 and 15.7 years) with optic pathways gliomas treated with chemotherapy as first-line treatment. Eight of them also received radiotherapy as salvage treatment. Eight had neurofibromatosis type 1 (NF1). Intellectual outcome was preserved in children treated with chemotherapy only (mean=107+/-17) compared to children also receiving radiotherapy (mean IQ=88+/-24) or children having NF1 and treated with chemotherapy (mean IQ=80+/-13). Scores for abstract reasoning, mental arithmetic, chessboard/coding, perception, judgement of line orientation were lower in children irradiated than in those treated only by chemotherapy. Children with Nf1 showed subnormal IQ scores with marked impairment of short- and long-term memory. With respect to long-term neuropsychological outcome, our study shows that a chemotherapy-first strategy can preserve the intellectual outcome of these patients either by avoiding the need of radiotherapy or by delaying its use as much as possible.

Duodenogastric reflux on (99m)Tc-tetrofosmin myocardial SPECT mimics left ventricle inferior wall reverse redistribution and falsely decreases ejection fraction: a case report.

99mTc-labeled myocardial perfusion agents are excreted through the hepatobiliary system and can be used in the evaluation of the duodenogastric reflux that occurs during routine cardiac imaging. The resultant gastric activity can overlap the inferior wall of the left ventricle (LV) and can thus mimic reverse redistribution of the LV inferior wall on dual-isotope SPECT. We report a case of significant gastric activity, which leads to abnormally low LV ejection fraction and akinesis of the LV wall in addition to the appearance of reverse redistribution. This case report illustrates that care should be taken in the performance and interpretation of (99m)Tc-tetrofosmin SPECT in the presence of duodenal reflux. This condition could be mistaken for reverse redistribution in the inferior wall of the LV with concomitant underestimation of the LV and regional wall motion.

Epileptic syndromes, cognitive assessment and school placement: a study of 251 children.

Two-hundred and fifty-one children (98 girls and 153 boys, aged from 3 to 17 years) with documented diagnosis of epileptic syndrome, IQ measurement, and information on school placement were included in this retrospective study. The relations between these three parameters as well as effects of age at onset and duration of epilepsy, seizure frequency, and number of antiepileptic drugs (AEDs) were analysed. Both IQ and schooling were univariately related to epileptic syndrome, age at onset and duration of epilepsy, and number of AEDs; seizure frequency was related to IQ but not to school placement. Multiple regression showed that IQ was independently related to epileptic syndrome and AED; multiple logistic regression showed that type of school (mainstream versus adapted or special) was independently related to IQ and AED. Children with idiopathic generalised or with localisation-related epilepsy had higher IQ scores and higher probability of mainstream schooling than those with symptomatic or cryptogenic generalised epilepsies or epileptic syndromes which were undetermined. Subtests profile of intelligence scale in localisation-related epilepsies showed different specific cognitive deficits, according to the location of the epileptic focus.

[Prognosis of isolated agenesis of the corpus callosum]. / Pronostic des agénésies isolées du corps calleux. Club Francophone de Médecine Foetale.

We report preliminary results of a three year follow-up of ten children affected with apparently isolated corpus callosum agenesis (prenatal diagnosis). This population was collected from a multicenter prospective study: annual survey included physical examination, developmental outcome and psychometric evaluation. Febril convulsions appeared to be more frequent than in the general population; developmental outcome was normal at the last evaluation. Follow-up has to be performed up to 10 years to determine more accurately prognosis of isolated corpus callosum agenesis.

Inherited microdeletion in Xp21.3-22.1 involved in non-specific mental retardation.

X-linked mental retardation (XLMR) is a genetically and clinically heterogeneous common disorder. A cumulative frequency of about 1/600 male births was estimated by different authors, including the fragile X syndrome, which affects 1/4000 males. Given this very high cumulative frequency, identification of genes and molecular mechanisms involved in other XLMRs, represents a challenging task of considerable medical importance. In this report we describe clinical and molecular investigations in the family of a mentally retarded boy for whom a microdeletion in Xp21.3-22.1 was detected within the frame of a previously reported systematic search for deletion using STS-PCR screening. Thorough clinical investigation of the sibling showed that two affected brothers exhibit a moderate non-specific mental retardation without any additional neurological impairment, statural growth deficiency or characteristic dysmorphy. Molecular analysis revealed that the microdeletion observed in this family is an inherited defect which cosegregates with mental retardation as an X-linked recessive condition, since both non-deleted boys and transmitting mother are normal. These results and the inherited microdeletion detected within the same region associated with non-specific MR, reported by Raeymaekers et al., suggest that Xp21.3 MR locus is prone to deletions. Therefore, search for microdeletions in the eight families assigned by linkage analysis to this region might allow a better definition of the critical region and an identification of the gene involved in this X-linked mental retardation.

[Neuropsychological sequelae of children treated for brain tumors: evaluation and risk factors]. / Séquelles neuropsychologiques des enfants traités pour tumeur cérébrale: évaluation et facteurs de risque.

The analysis of neuropsychological sequelae in children with brain tumors is a major concern in the debate for the definition of the therapeutic strategies. The authors review the recent literature in this field in the light of their own experience at the Gustave-Roussy Institute. Three main risk factors emerge from the analysis: radiation, age and surgical complications. The need for a longitudinal evaluation in these patients is emphasized in order to plan the subsequent appropriate readaptation.

Reduced day-to-day variation accompanies adaptive plasticity in the primate spinal stretch reflex.

Monkeys can change the amplitude of the spinal stretch reflex (SSR), or M1, when reward is made contingent on amplitude. The present study demonstrates that reduced SSR day-to-day variation accompanies such adaptive SSR change. This finding supports the assumption that initial, phase I, SSR change results from contingency-appropriate stabilization of tonic activity in relevant descending spinal cord pathways.

Adaptive plasticity in the spinal stretch reflex.

Monkeys can change the amplitude of the spinal stretch reflex without change in initial alpha motor neuron tone, as measured by EMG, or in initial muscle length. Change is apparent in 5-10 days, continues to develop over weeks, and persists during inactive periods. Spinal stretch reflex change may be a valuable system for studying the neuronal and synaptic bases of an adaptive change in primate CNS function.