Subtle neuropsychological deficits in children with sickle cell disease.

Forty-three children and adolescents (8-16 years old) with sickle cell disease (SCD) and no overt neurologic dysfunction were evaluated with the Wechsler Intelligence Scale for Children-Revised (WISC-R), the Wide Range Achievement Test (WRAT), and an age-appropriate version of the Luria-Nebraska Neuropsychological Battery (LNNB). Thirty similar-aged siblings were controls. WISC-R performance and full-scale IQs were lower (p less than 0.05) for patients than sibling controls. No significant differences were seen on the WRAT. Patients with SCD missed more school days than controls (p less than 0.001) but did not differ from controls in academic performance or the proportion in special education. On the LNNB, scores for patients and controls 13 years and older did not differ. However, young patients had significantly elevated (abnormal) scores compared with controls on the following scales: expressive speech, writing, reading, arithmetic, memory, and pathognomonic. The possibility that subtle neuropsychological deficits in younger patients with SCD reflect either subclinical strokes or a predisposition for strokes merits further investigation in a large multi-institution study.

Acute psychosis in a patient with Epstein-Barr virus infection.

A case study is presented of an adolescent female who became psychotic and attempted suicide during an acute Epstein-Barr Virus infection. Resolution of the infection resulted in the patient returning to her previous normal mental status. The adolescent's ingestion of acetaminophen masked the signs and symptoms of infection and delayed proper diagnosis.

Social competence and behavioral adjustment of children who are long-term survivors of cancer.

Relatively little is known about the special needs of long-term survivors of childhood cancer or the factors that make this growing population vulnerable to chronic psychologic disorders. We therefore surveyed 183 children who had been treated for cancer at St Jude Children's Research Hospital and were free of the disease for greater than or equal to 2 years after completing therapy. Parental responses to the Child Behavior Checklist, a standardized inventory of social competence and behavioral problems, were analyzed in relation to demographic and medical variables, as well as the children's appearance and functional status. School-related problems and somatic complaints of undetermined origin were increased fourfold relative to age- and gender-adjusted rates for peer groups in the general population. The presence of functional but not cosmetic impairments increased the risk of academic and adjustment problems. An older age at evaluation, treatment with cranial irradiation, and residence in a single-parent household were also associated with an increased risk of psychologic problems. General pediatricians must eventually assume responsibility for the extended follow-up care of children who have survived a malignancy. Their awareness of the high-risk groups described in this report should facilitate more timely identification of problems and referral of the children to appropriate services.

Memory function in disease-free survivors of childhood acute lymphocytic leukemia given CNS prophylaxis with or without 1,800 cGy cranial irradiation.

Previous studies have found that CNS prophylaxis of children with leukemia, especially young children receiving cranial irradiation, causes neuropsychologic deficits. In the present study, 40 children in continuous complete remission from acute lymphocytic leukemia (ALL) were given a battery of tests to assess memory functioning 5 years after CNS prophylaxis. All children were free of CNS disease at diagnosis and had been randomly assigned to receive CNS prophylaxis with either 1,800 cGy cranial irradiation (CRT) plus intrathecal (IT) methotrexate (MTX) or IT MTX plus intravenous (IV) high-dose MTX (HDMTX). No treatment- or age-related differences were seen on 16 standardized memory measures. However, scores of the combined sample were significantly lower than age-corrected norms on a test of visual-spatial memory and on four scales of verbal memory. Differences in methods or intensity of CNS prophylaxis and study group selection criteria are proposed to explain our findings and to resolve discrepancies with previous reports. The long-term neuropsychological sequelae in these survivors of ALL may be attributable to some common factor, such as the disease itself or systemic and IT chemotherapy.

The psychological status of survivors of childhood/adolescent Hodgkin's disease.

To assess psychosocial late effects of childhood/adolescent cancer, semi-structured interviews were conducted with 40 subjects who had achieved complete remission from Hodgkin's disease and completed therapy at least five years previously. Mean ages were 12.8 years at diagnosis and 24.7 years at interview. Side effects of treatment were most often mentioned as the "worst thing" about having had Hodgkin's disease. Although subjects had missed a mean of six months of school, and 40% had reported unpleasant school experiences, their educational levels exceeded those expected in sex-, age-, and state-matched populations. Almost all subjects said that they had benefited in some way from the experience of having cancer. In contrast to the female subjects, male subjects expressed little interest in having their reproductive status assessed. Current concerns included discrimination in employment or in obtaining life or health insurance.

Intellectual and academic achievement status after CNS relapse: a retrospective analysis of 40 children treated for acute lymphoblastic leukemia.

We determined the intellectual and academic status of 40 children with acute lymphoblastic leukemia who had experienced a primary isolated relapse in the CNS by analyzing the results of psychoeducational tests administered a median of 6.1 years after the relapse. Mean scores for full-scale IQ (87.5), verbal IQ (86.7), performance IQ (90.3), as well as academic achievement in reading (89.8), spelling (83.9), and mathematics (83.5) were significantly below normal expectations for age. Twenty percent of the group were mentally retarded and were receiving special educational assistance. The best clinical predictors of full-scale IQ were the number of radiation therapy courses, age, and the presence or absence of cerebral pathology as measured by computed tomography (CT). Children who were younger at the time of treatment, who received two courses of radiation therapy, and who had clinical seizures and structural abnormalities of the brain as detected by CT had the poorest psychological outcome. Although the psychoeducational consequences of CNS relapse and its attendant treatment are significant, these must be balanced by consideration of the relatively low probability of long-term survival without aggressive therapy. Recognition of this type of delayed morbidity with systematic surveillance and prompt attempts at rehabilitation may decrease or at least minimize these sequelae.

Serial neuropsychological studies of a child with acute lymphoblastic leukemia and subsequent glioblastoma multiforme.

A 10-year-old boy had a right posterior parietal glioblastoma 5 years after completing treatment for acute lymphoblastic leukemia. Interim findings included seizures, leukoencephalopathy, diffuse mineralizing microangiopathy, and abnormal changes in neuropsychological test performance, which, in retrospect, provided information about the location of the tumor. This case highlights unusual sequelae of childhood leukemia and its treatment, as well as the value of neuropsychological procedures in assessing functional status and integrity of the brain.

Munchausen syndrome by proxy simulating cystic fibrosis.

The case of a child with apparent cystic fibrosis whose many signs, symptoms, and laboratory results were convincingly portrayed by his mother is presented. The mother falsified the history, and cunningly altered sweat tests and stool fat analyses, and stole sputum from patients with cystic fibrosis to make her child appear to have cystic fibrosis. This case of Munchausen syndrome by proxy highlights the extent to which the diagnosis of cystic fibrosis rests on reliable history and laboratory data and emphasizes the extremes to which perpetrators of this form of child abuse may go to make their case. Medical personnel must educate civil authorities about this syndrome and the dangers it represents to the child.

A prospective study of the impact of home monitoring on the family.

Fourteen families whose infants required home monitoring for apnea were followed for approximately 5 years from the initial contact (1977). Each family received at least three psychiatric interviews and two follow-up contacts. Effects on the monitored infant, siblings, and parents were examined in open and semi-structured interviews. At first follow-up (mean of 21 months after monitoring discontinuance), seven of 14 monitored children were characterized as spoiled by parents. By the second follow-up, 2 1/2 years later, nine children showed speech, learning, and motor problems; five of these nine children had required resuscitation during their initial episode. Apnea severity was also related to both duration of monitoring and number of additional psychiatric interviews requested by parents or staff. Of 16 older siblings, 12 had psychological problems reported by their parents at first follow-up; these problems appeared to be largely resolved by the second follow-up, although three children were still having problems that caused parental concern. Monitoring, as well as the apnea itself, caused substantial distress manifested in depression, fatigue, and anxiety in many parents, particularly mothers. However, despite their considerable distress, none of the families discontinued monitoring prematurely. Results of the study indicated that the monitoring experience can be divided into four phases: doubt/acceptance, dependence/frustration, discontinuance, and late effects, with substantial differences in parents' responses occurring only in the last two phases.

Teaching psychosocial issues to pediatric house officers.

The most effective method of teaching psychosocial pediatrics to house officers apparently has not been ascertained. Therefore, in a study reported here, a controlled trial was performed to test whether a child psychiatrist accompanying a ward team on daily rounds would influence the attention given by the residents to psychosocial problems. The study population consisted of two ward teams over two months and the intervention limited to one team during the second month. The presence of a child psychiatrist on daily rounds did not influence the proportion of notes mentioning psychosocial problems nor the proportion of informative psychosocial notes in the intervention group. There was no significant relationship of notes to the age, sex, or payment status of the patient. However, the presence of the psychosocial notes and the information in them did vary with follow-up plans, type of consultations ordered, and admission and discharge diagnoses. In this article the authors discuss implications of the findings in the context of the dichotomy between the biomedical and biopsychosocial models of patient care.