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Survivors of childhood cancer whose malignancy and/or treatment involved the central nervous system may demonstrate a consistent pattern of neurocognitive deficits. The present study evaluated a randomized clinical trial of the Cognitive Remediation Program (CRP). Participants were 6- to 17-year-old survivors of childhood cancer (N = 161; 35% female, 18% Hispanic, 10% African American, 64% Caucasian, 8% other) who were at least 1 year off treatment and who manifested an attentional deficit. They were enrolled at 7 sites nationwide. Two thirds of the participants were randomly assigned to cognitive remediation. All participants were assessed using a battery of academic achievement/neurocognitive tests and parent/teacher measures of attention. The CRP resulted in parent report of improved attention and statistically significant increases in academic achievement. Effect sizes were modest but were comparable with those for other clinical trials of brain injury rehabilitation and for psychological interventions in general. The CRP is presented as a potentially beneficial treatment for many survivors of pediatric cancer. Long-term clinical significance remains unproven. Further work is needed to improve effect sizes and treatment compliance and to address the needs of other populations with pediatric brain injury.

Mothers of children with serious illnesses have lower levels of well-being than mothers in the general population. Problem-solving therapy (PST), a cognitive-behavioral intervention, has been shown to be effective in treating negative affectivity (depression, anxiety) and other manifestations of reduced well-being. This report describes a problem-solving skills training (PSST) intervention, based on problem-solving therapy, for mothers of newly diagnosed pediatric cancer patients. Ninety-two mothers were randomly assigned to receive PSST or to receive standard psychosocial care (Control Group). After the 8-week intervention, mothers in the PSST Group had significantly enhanced problem-solving skills and significantly decreased negative affectivity compared with controls. Analysis revealed that changes in self-reports of problem-solving behaviors accounted for 40% of the difference in mood scores between the two groups. Interestingly, PSST had the greatest impact on improving constructive problem solving, whereas improvement in mood was most influenced by decreases in dysfunctional problem solving. The implications of these findings for refinement of the PSST intervention and for extension to other groups of children with serious illnesses are discussed.

It is now generally accepted that central nervous system treatments for childhood cancer can result in significant cognitive impairment, most commonly in the areas of attention/concentration. We review the literature on attentional and neurocognitive deficits in this population, and also efforts to remediate attentional deficits in other brain injured populations. It was our goal to develop an innovative, psychologically based outpatient rehabilitation program that would improve dysfunctional attentional processes and associated neuropsychological deficits. The characteristics of this program and a pilot study of its effectiveness are described. Participants were 31 off-therapy cancer survivors with documented attention deficits. Twenty-one completed the cognitive remediation program (CRP) and 10 served as comparisons. All participants completed a test of vigilance attention, 2 tests with an attentional component, and an arithmetic academic achievement measure. When the scores of the 2 groups were compared, the CRP group exhibited statistically significant improvement on all attentional measures. In contrast, the comparison group did not manifest any significant changes. Neither group demonstrated statistically significant changes on the arithmetic achievement test. We believe that the CRP has potential for improving attention/concentration, but generalization to academic achievement remains unproven. Phase 3 clinical trials and the documentation of long-term treatment gains are needed. Furthermore, it will be necessary to demonstrate the ecological validity of the CRP. With these caveats, this therapeutic approach may be helpful in other populations of cognitively impaired children and young adults, such as patients who have suffered traumatic brain injury. (JINS, 2002, 8, 115–124.)

PURPOSE: To assess the long-term neuropsychologic effects experienced by children who have tumors in the cerebellum that are diagnosed and treated during infancy. PATIENTS AND METHODS: Twenty-seven children with posterior fossa tumors diagnosed at less than 36 months of age were assessed prospectively with a comprehensive set of age-appropriate tests. Group means and SDs are reported for assessments conducted at diagnosis (analysis 1) and at the most recent follow-up appointment (analysis 2). Cognitive developmental growth curves were derived from the prospective data (analysis 3) using mixed model regression analyses and controlling for age at diagnosis and socioeconomic status. RESULTS: In the first analysis, eight of 11 infants at diagnosis scored within normal limits on all neuropsychologic domains, except for motor skills, which were impaired. In the second analysis, mean scores at the most recent follow-up of 21 of 27 patients were mostly in the normal range; however, group comparisons between those who had (n = 7) and had not (n = 14) been treated with cranial radiation therapy (CRT) showed that patients in the irradiated (CRT) group scored significantly lower than those in the nonirradiated (No-CRT) group on verbal intelligence quotient (IQ) and in the motor domain. In the third analysis (growth curves of CRT and No-CRT groups), statistically significant differences in slope were found on verbal IQ, performance IQ, perceptual-motor skills, language, and attention/executive skills. Slopes on the fine-motor domain were similar; both groups declined at approximately the same rate. CONCLUSION: Neurocognitive development and outcome of children with cerebellar tumors diagnosed in infancy is very positive among those who were treated with surgery and chemotherapy. Declines in performance across time were minimal, and scores tended to remain within normal limits. By itself, a cerebellar tumor in infancy does not seem to have a significant impact on children. However, those who received CRT as part of their treatment are likely to have neurocognitive and psychosocial deficits that require remediational interventions.

Neurofibromatosis type 1 is a common autosomal dominant genetic disorder associated with numerous physical anomalies and an increased incidence of neuropsychological impairment. Tumors of the CNS occur in approximately 15% of children with neurofibromatosis, presenting additional risk for cognitive impairment. This study examines the impact of an additional diagnosis of brain tumor on the cognitive profile of children with neurofibromatosis. A comprehensive battery of neuropsychological tests was administered to 149 children with neurofibromatosis. Thirty-six of these children had a codiagnosis of brain tumor. A subset of 36 children with neurofibromatosis alone was matched with the group of children diagnosed with neurofibromatosis and brain tumor. Although mean scores of the neurofibromatosis plus brain tumor group were, in general, lower than those of the neurofibromatosis alone group, these differences were not statistically significant. Children in the neurofibromatosis plus brain tumor group who received cranial irradiation (n = 9) demonstrated weaker academic abilities than did children with brain tumor who had not received that treatment. These results suggest that neurofibromatosis is associated with impairments in cognitive functioning, but the severity of the problems is not significantly exacerbated by the codiagnosis of a brain tumor unless treatment includes cranial irradiation.

Summary This article describes a cognitive-behavioral therapy intervention for teaching mothers of newly diagnosed patients with pediatric cancer to solve their problems, problems related to their child's illness, and the family's emotional reactions to the illness. The authors discuss the challenges and effects of maternal adjustment throughout the various stages of the child's illness, review the characteristics of adult learners, describe a maternal problem-solving therapy program, and provide an overview of the structured educational process, followed by a detailed discussion of a six-step maternal problem-solving therapy intervention. Finally, they describe an inventory that measures the effects of the intervention, which is being tested in a randomized controlled trial.

This study examined the predicted utility of the Nonverbal Learning Disabilities syndrome (NLD) (Rourke, 1995) for characterizing neurocognitive and psychosocial outcomes in 123 children with brain tumors. Children with brain tumors were found to be at high risk of having a specific academic deficit, particularly in arithmetic. Children with arithmetic deficit evidenced a higher rate of impairment on nonverbal tasks than on verbal tasks, whereas children with reading deficit evidenced a higher rate of impairment on verbal tasks than on nonverbal tasks. However, significant differences between children with arithmetic and reading deficits were not found for all of the component features of the NLD syndrome, and arithmetic deficit was not related to treatment with irradiation.

Cross-cultural aspects of siblings' adaptation to childhood cancer were examined in two studies as part of a cooperative multi-institutional investigation in Israel and the United States. Study 1 compared the behavioral adaptation of Israeli siblings of patients with childhood cancer with matched clinically referred and normal Israeli children. Study 2 compared the behavioral adaptation of case-matched samples of Israeli and American siblings and assessed the contribution of family relations and parental coping to the siblings' behavioral adjustment. Israeli patients' siblings and normal samples scored significantly lower on the Child Behavior Checklist (CBCL) than the clinically referred sample did, indicating fewer behavior problems, with no differences between the sibling and normal samples. The CBCL scores of the Israeli and American samples did not differ significantly on the Externalizing and Internalizing subscales and the Total Behavior Problem score. Significant correlations were found in both the Israeli and American samples between the parental coping and family relations variables and the CBCL scores, indicating that greater internal family support and emotional expressiveness and lower conflict were associated with less behavior disturbance in the sibling. The authors consider cross-cultural and methodologic issues when interpreting similarities and differences among Israeli and American siblings of pediatric cancer patients.

Infants and young children who have brain tumors have a poor rateof survival and high treatment associated morbidity. A trial of mechlorethamine, vincristine (oncovin), procarbazine, and prednisone(MOPP) was performed to test the hypothesis that replacingradiotherapy with chemotherapy would improve survival and decreaselong term morbidity of infants who have brain tumors. Between 1976 and1988, 17 consecutive children less than 36 months old when diagnosed with medulloblastoma or ependymoma were treatedwith MOPP chemotherapy as primary therapy following surgical excision or biopsy of the tumor. Radiotherapy was reserved for recurrent disease. Ten of 17 children have survived without evidence of disease: medulloblastoma, eight of 12 with median survivaltime of 10.6 years (range, 6.2 to 15.2 yrs); and ependymoma, 2of 5 (at 13.0 and 16.0 yrs). Four of the 10 children with medulloblastomaand ependymoma who relapsed are now disease free at 7.5, 11.7, 12.2 and 13.5 yrs post relapse after receiving salvagetherapy with cisplatin (n = 1) or irradiation (n = 3). All relapsesoccurred within 26 months of diagnosis. Data on growth demonstrated heightless than the 5th percentile in all children who received cranial irradiation compared to 25 to 95th percentile for nonirradiated children. Intellectual ability for the groupwho did not require radiation was within normal range (mean IQ 100.1) and stable across annual assessments. Those who required radiation hadlower IQs which continued to decline over time (mean IQ 85 at mean age of 5.8 years, declining to 63 at 10 years).In young children with brain tumors, primary chemotherapy with MOPP, omitting radiotherapy, provides improved neurodevelopmental outcome and survival.

Neuropsychological studies of children who have brain tumors have yielded diverse results with respect to identifying factors that contribute to poor intellectual outcome. The purpose of this study was to evaluate the relationship between pre- and perioperative events, tumor-related factors, and the neuropsychological status of children diagnosed with astrocytoma. Events that could potentially be detrimental to neuropsychological outcome were quantified utilizing a new "neurological severity score." The Neurological Severity Score was developed as a research tool to test our hypothesis that ultimate intellectual outcome is a result of cumulative, interactive insults on the central nervous system. This study constitutes a first step in examining the predictive value of the Neurological Severity Score by evaluating its correlation with baseline neuropsychological status. Fifty-nine children who had astrocytoma (36 supratentorial and 23 infratentorial) received complete neurological and neuropsychological evaluations within 3 months of diagnosis. Each child's neurological history and examination results were scored by an independent observer using the Neurological Severity Score. Neuroimages obtained at diagnosis and at the time of neuropsychological testing were evaluated as well. For the group as a whole, memory, attention, and motor abilities were significantly below age-appropriate norms, whereas intelligence, language, and academic skills were preserved. Patterns of deficits were identified and related to tumor site. There were no significant differences in mean neuropsychological domain scores between groups based on gender, pre- versus post-operative status, ethnicity, tumor grade, or abnormalities on magnetic resonance imaging (MRI). The Neurological Severity Score was significantly inversely correlated with visual-spatial skills, memory, attention, performance IQ, and global IQ. In conclusion, among all the medical and neurological factors present at diagnosis, the neurological severity score had the highest correlation with neuropsychological scores. This instrument has promise as a research tool in investigations of the psychological effects of cancer and its treatment on children. (J Child Neurol 1996;11:462-469).

Objective This seven-site study examined the overall health status, healthcare utilization, somatization, and health-risk behaviors of siblings of children with cancer compared to these factors in matched controls or normative data. The study also examined whether informants (i.e., siblings, parents, physicians) differed in their assessments of the above health domains. Design Subjects were 254 siblings of children with cancer from seven different pediatric oncology treatment centers that participated in the Sibling Adaptation to Childhood Cancer Collaborative study group. Predictors of the siblings' health status, healthcare utilization, somatization, and health-risk behaviors were identified, and the relationship between these health domains and the siblings' resiliency vs. dysfunctionality were explored via interviews. Results Overall, siblings were found to be moderately healthy, although siblings report significant problems with sleeping and eating. Healthcare utilization appears to be reduced for siblings. Most importantly, the parents of these siblings are less likely to seek medical help for a variety of conditions for which parents of control children would bring their children to a doctor. A pattern emerged of parental underreporting of sibling health variables when compared to what the siblings themselves reported. When the relationship between health outcomes and the siblings' adaptation to their sick sibling's illness was examined, the resilient and dysfunctional groups significantly differed from each other. It appears that health outcomes are related to sibling adaptation to the changes brought about by their sick sibling's cancer diagnosis and treatment. Conclusions The focus of care for families of children with cancer is often limited to the child with cancer. As indicated in this study, the “healthy” siblings may be overlooked in the process. While parents appear to recognize that their “healthy” children are complaining more about aches and pains, they may have little energy or time to attend to the needs of these other family members. It is the intent of this study to document what clinicians may expect and to highlight the need for evaluation of this otherwise neglected group. © 1996 Wiley-Liss, Inc.

Investigated responses of siblings (N = 254) of children with cancer to structured interviews designed to elicit thoughts and feelings about effects of cancer on self and family. Siblings indicated distress about family separations and disruptions, lack of attention, focus of family on the ill child, negative feelings in themselves and family members, cancer treatments and their effects, and fear of death. Siblings also reported becoming more compassionate, families becoming closer, and having experiences they otherwise would not have had. Age and gender differences in responses indicated distinctive perceptions of and vulnerability to the cancer experience. Older siblings were far more likely to report positive effects than younger siblings suggesting that level of maturity can moderate the stress of an ill child within the family.

Researchers conducting longitudinal studies with children or adults are inevitably confronted with problems of attrition and missing data. Missing data in longitudinal studies is frequently handled by excluding from analyses those cases for whom data are incomplete. This approach to missing data is not optimal. On the one hand, if data are missing at random, then dropping incomplete cases ignores information collected on those cases that could be used to improve estimates of population parameters (e.g., means, variances, covariances, and growth rates) and improve the power of significance tests of statistical hypotheses. On the other hand, if data are not missing at random, then dropping incomplete cases leads to biased parameter estimates and hypothesis tests that may be internally and externally invalid. This study uses three years of follow-up data from a longitudinal investigation of neuropsychological outcomes of cancer in children to demonstrate the problems presented by missing data in repeated measures designs and some solutions. In evaluating potential biasing effects of attrition, the study extends previous research on neuropsychological outcomes in pediatric cancer by inclusion of patients whose disease had relapsed, and by comparison of surviving and nonsurviving patients. Although the data presented have specific relevance to the study of neuropsychological outcome in pediatric cancer, the problems of missing data and the solutions presented are relevant to a wide variety of diseases and conditions of interest to researchers in child and adult neuropsychology.

Within the context of a coping model, the present study attempted to determine variables associated with good and poor adjustment for siblings (N = 129) of pediatric cancer patients. Family cohesion and adaptability were found to mediate the impact of pediatric cancer on the healthy sibling. Specifically, high levels of family cohesion and adaptability were associated with better adaptation for siblings. Some support also was found for Folkman, Schaefer, and Lazarus' (1979) coping model, as the following variables, proposed by the model, predicted sibling adjustment to pediatric cancer: health/energy/morale, social support, and utilitarian resources. Results from this study stress the need to examine the child in a variety of ecological contexts (e.g., parental and familial functioning; neighborhood/community support).

Reviewed 53 studies on the effects of central nervous system prophylactic treatment (CNSPT) on cognition and neuropsychological functioning. CNSPT involves irradiation and/or chemotherapy directed to the brain, and is frequently administered to children who have leukemia. The effects of CNSPT on neuropsychological functioning in long-term cancer survivors remains controversial. A number of studies failed to identify any neuropsychological impact secondary to CNSPT, whereas others reported deficits that appear to be a function of CNSPT. Several methodological issues that make it difficult to draw meaningful conclusions from this research literature are identified and discussed. Specific recommendations are made for improvements in research on CNSPT in children.

Identified types and frequencies of psychological difficulties manifested by pediatric oncology patients and child-, family-, and illness-related correlates of adjustment. Parents of 48 children with cancer, 4 to 17 years of age, completed the Personality Inventory for Children (PIC). Analysis of mean PIC scores indicated that the children had a high frequency of somatic concerns and problems in academic functioning. Similar mean PIC profiles were obtained for children across gender, age, and diagnostic groups. Overall, 52% of the children had profiles with two or more clinically significant problem areas. Children's adjustment was associated with gender, social competence, and parental coping. Boys exhibited significantly more problems than did girls. Children whom teachers rated as less socially competent and whose parents reported few effective coping responses exhibited greater difficulties in adjustment.

It is now generally accepted that the diagnosis of many pediatric cancers and their treatments result in significant and long-lasting neurocognitive, psychological, and psychosocial impairments and difficulties. The current status of research in this field has been addressed by other chapters in this text. We would, however, like to emphasize at the onset of our chapter that we firmly believe pediatric cancer is truly a family affair. The effects of the diagnosis of a life-threatening illness and its often-chronic treatment not only result in significant impact on the child’s or adolescent’s neuropsychological and psychological state, but also cause psychological ramifications for the parents, siblings, and extended family members. In healthy, well-functioning families, this major life obstacle can serve as an impetus to rally family members in support of the child. When this happens, interventions for late effects are beginning to be identified as effective and of potential benefit. This field, however, is clearly in its infancy. If the family is chaotic and struggling with relationship issues, the prognosis is less positive. Our clinical observations of these relationships are supported both by preliminary data from studies conducted by our research group and others, and by published manuscripts in the field of pediatric traumatic brain injury (Yeates et al., 1997, 2001). In one of the only studies investigating the impact of familial variables on psychosocial and neuropsychological outcome in pediatric brain tumor patients, the results are extremely consistent with the traumatic brain injury population (Carlson-Green, Morris, & Krawjecki, 1995). Reduced maternal dependence on external coping resources, higher parental socioeconomic status, dual-parent families, and familial cohesion were all identified as improving long-term outcome in this population, as documented by intellectual and behavioral integrity. The late effects of pediatric cancer and its treatment are physical, cognitive, psychological, and social. When multiple effects are present, they can be expected to result in a synergistic impact not only on the child, but also on other family members. The important point is that late effects should not be viewed in isolation or summated but should be appreciated for their interrelatedness.

The diagnosis of childhood cancer is a highly stressful event for all members of the family. This chapter focuses on the responses of mothers during the first few months after the diagnosis and describes an 8-week intervention, entitled problemsolving skills training (PSST), that is efficacious in reducing features of negative affectivity in mothers during the time of induction and early treatment. Studies examining the emotional well-being of parents of children with cancer suggested that mothers and fathers are at risk for symptoms of anxiety and depression during their child’s treatment (Fife, Norton, & Groom, 1987; Hughes & Lieberman, 1990; Noll et al., 1995). In addition, some evidence suggested that mothers are at increased risk for posttraumatic stress symptoms after treatment ends (Hall & Baum, 1995; Kazak et al., 1997; Manne, DuHamel, Gallelli, Sorgen, & Redd, 1998; Manne et al., 2001, 2002; Pelcovitz et al.,1996; Stuber, Christakis, Houskamp, & Kazak, 1996). These findings are not universal. Some researchers have reported a return to normal psychological functioning among parents within months after their child was diagnosed (Dahlquist, Czyzewski, & Jones, 1996; Kupst & Schulman, 1988; Kupst et al., 1995). Regardless, consensus exists that there is a period of time immediately after a child is diagnosed with cancer when caregivers are at risk for an increase in symptoms of depression and anxiety (Kazak et al., 2001). In addition to the specific stressors associated with a new diagnosis of cancer in her child, the wife/mother appears to be the key individual in maintaining the integrity of the entire family. For example, 74% of men reported that they talk primarily to their spouse when upset (Cutrona, 1996). Further, males will typically depend on their wives to maintain social contacts with friends, family, and the general community (Antonucci & Akiyama, 1987). Thus, mothers may be particularly vulnerable to distress because of the pivotal role they play in maintaining family social and emotional functioning. The experience of a cancer diagnosis can be easily understood as a major negative life stressor that can cause significant psychological distress.

Mothers of children with cancer experience significant distress associated with their children's diagnosis and treatment. The efficacy of problem-solving skills training (PSST), a cognitive-behavioral intervention based on problem-solving therapy, was assessed among 430 English- and Spanish-speaking mothers of recently diagnosed patients. Participants were randomized to usual psychosocial care (UPC; n = 213) or UPC plus 8 sessions of PSST (PSST; n = 217). Compared with UPC mothers, PSST mothers reported significantly enhanced problem-solving skills and significantly decreased negative affectivity. Although effects were largest immediately after PSST, several differences in problem-solving skills and distress levels persisted to the 3-month follow-up. In general, efficacy for Spanish-speaking mothers exceeded that for English-speaking mothers. Findings also suggest young, single mothers profit most from PSST.

This chapter covers the approach taken by the M. D. Anderson Cancer Center Division of Pediatrics in the diagnosis, treatment, and supportive care of children with central nervous system (CNS) tumors. The unique aspects of state-of-the-art radiotherapy and neurosurgery techniques available to children are discussed as well as our comprehensive supportive-care program. The specialized care of children with neurofibromatosis and CNS tumors are described. Chemotherapy programs and clinical trials for children with CNS tumors are discussed in Chapter 4, “Brain Tumors: Chemotherapy and Investigational Therapy.”

Pediatric cancer is one of the most difficult challenges a family may face. The experience will have a lasting effect on the patient and all family members. There will be some personal and shared triumphs, but there will likely be some stressful situations and encounters that could impede the child’s natural developmental course and affect family dynamics. The behavioral medicine program in the Child and Adolescent Center at M. D. Anderson Cancer Center is a multidisciplinary service designed to address the many psychological, emotional, and psychosocial issues related to the experience of childhood cancer.

It is now generally accepted that central nervous system treatments for childhood cancer can result in significant cognitive impairment, most commonly in the areas of attention/concentration. We review the literature on attentional and neurocognitive deficits in this population, and also efforts to remediate attentional deficits in other brain injured populations. It was our goal to develop an innovative, psychologically based outpatient rehabilitation program that would improve dysfunctional attentional processes and associated neuropsychological deficits. The characteristics of this program and a pilot study of its effectiveness are described. Participants were 31 off-therapy cancer survivors with documented attention deficits. Twenty-one completed the cognitive remediation program (CRP) and 10 served as comparisons. All participants completed a test of vigilance attention, 2 tests with an attentional component, and an arithmetic academic achievement measure. When the scores of the 2 groups were compared, the CRP group exhibited statistically significant improvement on all attentional measures. In contrast, the comparison group did not manifest any significant changes. Neither group demonstrated statistically significant changes on the arithmetic achievement test. We believe that the CRP has potential for improving attention/concentration, but generalization to academic achievement remains unproven. Phase 3 clinical trials and the documentation of long-term treatment gains are needed. Furthermore, it will be necessary to demonstrate the ecological validity of the CRP. With these caveats, this therapeutic approach may be helpful in other populations of cognitively impaired children and young adults, such as patients who have suffered traumatic brain injury.

PURPOSE A prospective study was conducted to assess the effects of chemotherapy for cancer on children's long-term neuropsychologic status. PATIENTS AND METHODS Ninety-nine children who received no cranial radiation therapy (CRT) completed four annual neuropsychologic assessments. Fifty-one patients received intrathecal (IT) chemotherapy (ITC); 48 received no CNS treatment. These two groups were compared using repeated-measures analysis of variance on IQ, memory, language, freedom from distractibility, academic achievement, executive functions, and fine-motor, perceptual-motor, and tactile-spatial skills. In addition, 51 of the sample of 99 patients had been examined 5 to 11 years after diagnosis. Their data were analyzed to evaluate the longer-term effects of chemotherapy. The predictability of demographic and medical variables on neuropsychologic outcome at 3-year and long-term follow-up study were assessed using multiple regression techniques. RESULTS Overall, the effects of chemotherapy in the absence of CRT appear to be slight. Patients who received ITC and intravenous (IV) methotrexate declined slightly on perceptual-motor skills, but were still well within the normal range. Both groups, regardless of treatment, declined on academic achievement tests, although not to a statistically significant degree. Age effects were found on performance IQ (PIQ) and perceptual-motor skills. Socioeconomic status (SES) correlated with a large number of variables. Sex effects were not significant. CONCLUSION The present results are largely consistent with previous findings for nonirradiated groups. Treatment effects from ITC are slightly more apparent 5 to 11 years after diagnosis than at 3-year follow-up evaluation but this does not constitute a clinically meaningful difference. More noticeable are academic declines among all groups, regardless of treatment.

Neurofibromatosis type 1, a common autosomal dominant genetic disorder, is associated with numerous physical and medical anomalies as well as an increased incidence of learning disability. Tumors of the central nervous system have been estimated to occur in 15%, but their contribution to neuropsychological status is unknown. This study examines the relative contribution of neurofibromatosis and brain tumor to the cognitive profile of children with neurofibromatosis. A comprehensive battery of neuropsychological and behavioral tests was administered to a group of 65 children with neurofibromatosis type 1. Fourteen were then matched on demographic variables with two other groups of children who had either a brain tumor in addition to neurofibromatosis or a brain tumor alone. The two brain tumor groups were also matched on tumor type, location, and therapy. Mean scores of the neurofibromatosis-brain tumor group were generally the lowest of the three groups; those of the brain tumor group were highest, and performance of the neurofibromatosis group was generally between the other two groups. These results suggest that neurofibromatosis is, by itself, associated with significant cognitive morbidity, but that the severity of the problems is increased somewhat if a brain tumor is also present. (J Child Neurol 1994;9:368-377).

Neuropsychological outcome of 28 patients with brain tumors diagnosed before the age of 36 months (mean, 19 months) was assessed using a comprehensive battery of tests. Elapsed time between diagnosis and testing averaged 6.2 years. Half the patients had received cranial radiation therapy and surgery, with and without chemotherapy, whereas the rest had received only surgery, with or without chemotherapy. Groups were comparable with respect to tumor diagnosis and location, age at diagnosis, race, and sex. Intellectual functioning was significantly lower in children whose treatment included cranial irradiation than in those treated without cranial irradiation, and this effect was more pronounced in nonverbal than in verbal intellectual abilities. Mean scores for the radiation group were lower than for the no-radiation group in all areas assessed and were significantly below age-based normative means in five of the eight cognitive areas: intellectual, memory, attention, motor, and visual-spatial skills. Mean scores for children in the no-radiation group were generally within the average range in all cognitive areas except visual-spatial skills, which were significantly below age-based normative means. Endocrine deficiencies and growth retardation were much more prevalent in patients treated with cranial irradiation. Because the immature brain is susceptible to treatment-related pathologic changes, infants are at greater risk than older children for significant, long-term neuropsychological, endocrine, and growth sequelae. In children treated without cranial irradiation, morbidity was minimized without an increased rate of mortality. ( J Child Neurol 1992;7:281-290).

We report on the neuropsychological status of 31 children with primary brain tumors who underwent assessment before receiving therapy. Overall, the children performed within normal limits in all test areas. The exception was the group with anterior hemispheric tumors who demonstrated deficits in executive cognitive functions. Also, when compared according to tumor type, children with midline tumors and hydrocephalus performed more poorly than others on measures of intelligence, executive abilities, visual-motor skills, and fine-motor functions. Although one-half to two-thirds of the children with supratentorial midline and infratentorial tumors had cranial nerve, oculomotor, or cerebellar deficits, only the latter were associated with specific neuropsychological deficits (poorer performance on fine-motor and visual-motor tests). Age did not appear to be a factor in these children's neuropsychological test performances.

This multi-institutional study investigated the association of behavioral/emotional adaptation among siblings of children with cancer with maternal general well-being, physical health, and resource use. One hundred seventy siblings and mothers completed standardized interviews and self-report measures 6 to 42 months after the cancer was diagnosed. As a group, mothers of children with cancer reported significantly lower levels of well-being than matched controls. When stratified according to the level of the sibling's behavioral/emotional adaptation, mothers of siblings in the Dysfunctional group (1) reported the lowest levels of well-being; (2) during the preceding year, were more likely to have sought professional services than mothers of children in the Resilient group; and (3) were least likely to have found social support helpful. Our results support an association between maternal well-being and sibling adjustment but show it is unlikely that nonspecific social support will improve adjustment. The rationale for problem-solving training for mothers is provided.

A multisite collaborative study assessed the frequency and intensity of emotional/behavioral distress in siblings of children with cancer. A sample of 254 siblings, aged 4 to 18 years, and their parents completed interviews and self-report measures 6 to 42 (average 22.5) months after diagnosis of cancer in a brother or sister. Matched controls were obtained from respondents to the Child Health Supplement of the National Health Interview Survey administered in 1988 (CHS88). Before diagnosis, the prevalence of parent-reported emotional/behavioral problems among siblings was similar to that in the general population (7.7% vs 6.3%; p = not significant). After diagnosis, prevalence rose to 18% among siblings. When siblings were grouped according to the presence or absence of problems exacerbated by and/or arising after diagnosis, four levels of adaptation, consistent with scores on the Behavior Problem Scales from the CHS88, emerged. This differentiation may help explain inconsistencies in sibling response reported previously and provides a framework for investigating factors that enhance adaptation.

Thirty-two long-term survivors of childhood leukemia who were followed up at the University of Texas M. D. Anderson Hospital were evaluated with a battery of 17 neuropsychologic tests. These tests were selected to assess the development of cognitive skills and functions associated with brain impairment in children. Statistically significant differences were found between the group of children given CNS irradiation and the nonirradiated group on full-scale IQ and verbal IQ scores, mathematics skills, constructional skills, and memory for spatial material. Of particular interest was the absence of differences in language-based measures of verbal memory and the presence of group differences on measures of memory for spatial material. While the sample size was small, the findings delineate specific areas likely to be affected. These results indicate the need for caution when including cranial irradiation in CNS prophylaxis. When any CNS treatment is given, it seems appropriate that provisions be made for assessment and remediation of affected skills.

This article reviews 41 studies of the effects of prophylactic CNS treatment on the neurobehavioral development of children with cancer. This research is classified according to studies of (a) children in treatment; (b) long-term survivors; and (c) longitudinal follow-ups of children from the time of diagnosis. Studies vary considerably in design, sample, and outcome variables, so firm conclusions regarding the morbidity of CNS prophylaxis are not currently possible. However, the studies do suggest that CNS prophylaxis does impair cognitive development, particularly when cranial radiation therapy is part of the treatment. There is also evidence of greater impairment in younger children and some suggestion of more frequent impairment of non-language skills relative to language skills. The possible relationships among age, radiation, and non-language cognitive skills may be linked to disruption of white matter CNS structures apparent on autopsy and cerebral tomography following treatment.

The development of two brief self-report measures for assessing parent-child communication specific to pediatric cancer is reported The instruments assess the quality of parent-child communication with their ill child and the sibling of the ill child The measures have good internal consistency and are significantly related to the overall level of healthy sibling adjustment.

The present paper reviews the literature on object relations theory with respect to the borderline patient and, within this perspective, discusses its application to hypnotherapy. Emphasis is placed on developmental aspects of the attachment-separation-individuation process that relate to certain characteristics of the hypnotic experience and the therapeutic relationship, including transference, regression, and the internalization of positive aspects of the therapeutic alliance. The therapeutic process as described parallels normal developmental phases. Specific techniques to facilitate therapeutic integration are outlined and illustrated with case examples. These help to structure the internalization of the therapeutic relationship as a foundation to restructuralization of the patient's representational world.

Surgery and radiotherapy are the primary modalities of treatment for pediatric brain tumors. Despite the widespread use of these treatments, little is known of their acute effects (within one year posttreatment) on neuropsychological functions. An understanding of acute treatment effects may provide valuable feedback to neurosurgeons and a baseline against which delayed sequelae may be evaluated. This study compares pre- and posttherapy neuropsychological test performance of pediatric brain tumor patients categorized into two groups on the basis of treatment modalities: surgery (n = 7) and radiotherapy (n = 7). Treatment groups were composed of children aged 56 to 196 months at the time of evaluation with heterogeneous tumor diagnoses and locations. Comparisons of pretherapy findings with normative values using confidence intervals indicated that both groups performed within the average range on most measures. Outstanding deficits at baseline were observed on tests of fine-motor, psychomotor, and timed language skills, and are likely to be attributable to tumor-related effects. Comparisons of pre- versus posttherapy neuropsychological test findings indicated no significant interval changes for either group. Results suggest that surgery and radiotherapy are not associated with acute effects on neuropsychological functions. ( J Child Neurol 1988;3:63-68).

Historical foundations of quality of life (QL) assessment, including those in adult oncology, are reviewed in the context of the current need for a developmental measure for clinical pediatric research. QL measures that can be applied to the assessment of children with cancer and other chronic and life-threatening diseases are urgently needed

The changes that occur in a child's life following the diagnosis of cancer extend to other members of the family and, through time, far beyond the biologic cure of the disease. The responses of the family to this stress and the ways in which they cope with it will depend partially on their own resources and partially on the severity of the disease and its treatment. This paper describes the factors that influence the family's adaptation to illness and presents a case example that illustrates criteria useful in assessing the need for psychologic consultation in childhood cancer patients and their families. Also presented are guidelines for the physician in his/her continuing observation and follow-up of these patients and families.

This study examines the neuropsychological effects of intrathecal and intravenous chemotherapies, specifies the relationship between drug administrations and test performance, and addresses differential effects of chemotherapy related to treatment age. Participants were 19 children under 6 years of age who were receiving intrathecal and intravenous chemotherapy for the treatment of acute lymphocytic leukemia (n = 15) or Burkitt's lymphoma (n = 4). Neuropsychological test performances obtained within 3 months of diagnosis and 1 year later were compared. Stability of performance was indicated on measures of higher order cognitive abilities and gross‐motor skills. Deficits in fine‐motor skills and abnormal deep tendon reflexes observed at baseline showed significant improvement 1 year later. Recovery was associated with discontinuation of systemic vincristine and attributed to rebound from chemotherapy‐induced peripheral neuropathy.

This group discussion centered on psychosocial factors in the reintegration of the medically exceptional child. The issue of labeling was the focus. Several times allusions were made to Dr. Bartholome’s point that health care providers create labels for patients. These labels, terms that emphasize different needs or lend themselves to stereotyping, can be barriers to assimilating the student into the school environment. When labels are overemphasized, children and their families experience the discomfort of enforced social distance, though the enforcement may be subtle. School personnel, despite specialized training, may create this distance as a result of the anxiety attached to their perception of these labels. This may impede their ability to help the classmates of the patient/student to accept him as an individual unencumbered by labels.

This study investigates by written questionnaire the information sources preferred by 63 adolescent cancer patients. Physicians were the most common source. Over half preferred private discussions with a health professional over other formats, and 68% indicated physicians as the health professional of choice. Sixty-eight percent wanted parents included. Only 35% indicated that additional information would be personally helpful, while 42% and 45%, respectively, stated that families and friends should have more information. No relationship between diagnosis or sex and the patients' responses was found. Newly diagnosed patients were less likely to perceive physicians as their main information sources or want additional information. Patients in active illness phases were also less desirous of additional information. Younger patients were more likely to prefer information only from parents, to avoid group discussions, and to negate wanting friends to receive additional information. Hispanics were most likely to want parents to have additional information and to request parental inclusion in discussions.

As survival time has been extended for young cancer patients, their informational needs have also increased. Using written questionnaires, we surveyed 63 adolescent patients, 60 parents, and 53 physicians to compare their perceptions of patient-communication patterns and current and preferred information sources. Physicians were the main current and preferred information sources noted by all three groups. Group discussions with patients the same age, films and television programs, and books were ranked as acceptable but less preferable sources by more than half of each group. More than 90% of the patients were usually accompanied by a parent. Patients most frequently sought their parents to discuss general concerns and cited parents as their second most frequently used source of disease-related information. Adolescents qualified their parental discussions more than was perceived by their parents (P less than 0.01) and viewed themselves as discussion initiators more frequently than did their parents (P less than 0.05). Parents were more likely than patients to prefer physicians as their child's information source (P less than 0.05). Physicians consistently underestimated their own and the parents' importance, believing instead that the patients relied more on peers. Fewer physicians than patients or parents favored including parents in discussion with health professionals (P less than 0.001).

Cranial radiation therapy (CRT) has been associated with an increased incidence of neuropsychological impairments and pathologic changes in the CNS among children. However, findings regarding a causal relationship between CRT and neurobehavioral impairments and the differential impact of CRT as a function of treatment age have been equivocal. Inconsistent findings may be attributed to the current research focus on description of impairments to the neglect of a larger theoretical framework and the failure of investigators to integrate findings from the various disciplines involved in assessing CRT effects. Two theories regarding the etiology of CRT effects on neuropsychological functions have been proposed. The myelination hypothesis suggests that CRT effects are attributable to direct effects on myelin synthesis. Findings indicating that the child is in a state of particular vulnerability to teratogens due to the rapid growth phase of myelin during the first 48 months of life provide the basis for this hypothesis. The myelination hypothesis predicts a differential effect for CRT as a function of age/maturation. The vascular hypothesis proposes that CRT effects are due to pathological changes in vascular tissues. Results indicating prominent white matter changes among some CRT recipients provide the basis for this hypothesis. The vascular hypothesis predicts no age effect or an inverse age effect; it places more emphasis on the relationship between indices of cerebral blood flow and neuropsychological test performance. Two basic mechanisms underlying the effects of CRT are outlined to provide a theoretical framework on which future research may be based.(ABSTRACT TRUNCATED AT 250 WORDS)

A number of psychosocial issues are evolving as childhood cancer becomes a chronic rather than a fatal condition. These include issues associated with extended hospitalization and social isolation, as well as long-term adjustment to survival that may involve recurrence of disease. Primary emphasis in current research and intervention is placed on how children and their families adapt to long-term survival. This article reviews the scope and limitations of previous research in this area, which focuses on the individual with little reference to the family, school, or social networks or to the interactions occurring among these arenas. An ecological model is suggested as the most useful perspective for enhancing our understanding of the impact of childhood cancer on the child, family, and community. Much of what is already known about how cancer and its treatment affect children and families is consistent with the propositions of an ecological model. The transactional, reciprocal, and interdependent nature of coping behaviors within a family system is perhaps the most obvious indicant of the need for multiple perspectives for understanding how families respond to the pressures of a chronic, but possibly life-threatening, illness. An ecological approach is timely, therefore, in that there is a need for psychological research and intervention directed beyond the immediate context of treatment to extended levels of social interaction and social systems.

It is clear from the passage of Public Law 94–142 (Education for All Handicapped Children Act, 1975) that legislators, and presumably the public whom they represent, have some awareness of the special educational needs of exceptional children. The law requires that all children, regardless of handicapping condition, be provided a free, appropriate education, and that the schools make available “related services” (physical therapy, occupational therapy, counseling, social work, and specialized supportive services) to those children who would not otherwise benefit from their educational programs. This brief and general statement may very well reflect the full extent of most people’s understanding of the law and its implications. There are many facets of the law that are either unknown or misunderstood by many, and because the law has such broad implications for schools and society, there is a real need to educate the public.