Inattention, hyperactivity-impulsivity, academic skills and psychopathology in boys with and without haemophilia.

The aim of this study was to determine if symptoms of inattention (IN) and hyperactivity-impulsivity (HI) differ for boys with and without haemophilia and to determine if IN and HI are the essential behavioural dimensions on which the two groups differ. Using a quasi-experimental design, parents' and teachers' ratings of IN and HI for boys with and without haemophilia (ages 6-14 years) were compared. IN and HI were also assessed with a psychometric task, as were reading and math, psychopathology, and educational status via various techniques. Boys with haemophilia (n = 19) were rated higher on dimensions of HI and IN by teachers (P = 0.01, P = 0.02, respectively) but only on HI by parents (P = 0.01). In addition, the haemophilia group committed more impulsivity errors on a psychometric task (P = 0.01). Trends, but not statistically significant differences, were found on reading and math scores, and the haemophilia group had more special education participation. Compared to national norms, borderline range scores on the attention-deficit/hyperactivity disorder (ADHD)-related dimensions of HI and psychometrically measured impulsivity characterized the boys with haemophilia. Although not addressing formal diagnoses, this study found that boys with haemophilia risk ADHD-spectrum problems, especially HI, and special education participation, but not frank academic deficits.

Is attention deficit hyperactivity disorder over-represented among HIV-seronegative boys with haemophilia? Preliminary results from our centre.

Haemophilia care providers report anecdotally that many boys under their care bear the diagnosis of attention deficit hyperactivity disorder (ADHD). This study investigated the hypothesis that ADHD is over-represented among boys with haemophilia. All the boys with haemophilia, aged 5-14 years, who receive their comprehensive care at our centre were solicited to participate in this study. Of the 78 eligible boys, 34 (44%) were successfully contacted and agreed to participate. All participants were HIV-negative on both serological and PCR-based assays. The presence of ADHD symptoms was established via a parent- and teacher-completed standardized rating instrument. On the parent-rating scale, 26% of the participants exceeded the cut-off for inattentive ADHD, 18% for hyperactive/impulsive ADHD, and 18% for combined. On the teacher rating scale, 4% of the participants exceeded the cut-off for inattentive ADHD, but no participants were rated as having extreme hyperactive/impulsive or combined ADHD symptoms. Retrospectively, 29% of the participants had previously been diagnosed with ADHD, all treated with stimulant medications. Of note, 38% of our participants were enrolled in special education programmes. All of the above were more common in boys with haemophilia compared with national controls. A chart review of non-participating patients from the same clinic suggested that sampling bias is unlikely to account for these differences. These results provide the first empirical evidence that ADHD may be over-represented among boys with haemophilia.

Functional brain mapping using positron emission tomography scanning in preoperative neurosurgical planning for pediatric brain tumors.

OBJECT: The purpose of this report is to demonstrate the value of functional brain mapping using the positron emission tomography (PET) method for preoperative neurosurgical planning in children with brain tumors. Brain maps were used to characterize the relationship between potentially resectable tumors and functionally eloquent brain areas. METHODS: Five children, ranging in age from 3 to 13 years, with hemispheric brain tumors adjacent to eloquent cortex were studied. Magnetic resonance (MR) imaging was used to identify the brain tumors; PET imaging after injection of [18F] fluorodeoxyglucose (FDG), [11C]L-methionine (CMET), or a combination of the two was performed to grade the tumors; and a [15O] H2O uptake study was used to characterize the anatomical relationships of the tumors to functional cortex. The cortical activation maps were obtained during control periods and during behavioral tasks and were used to document motor, visual, and speech and language organizational areas. Wada tests were performed in two patients. Language and speech activation was concordant with the results of Wada testing. CONCLUSIONS: Functional brain mapping using PET scans and coregistered MR images provided the neurosurgeon with precise definitions of structural and functional cortical areas; this altered surgical management in some cases and/or was used to predict outcome. The combination of PET imaging with FDG and/or CMET and measurements of [15O] water uptake was useful in characterizing and grading tumors and instrumental in achieving effective neurosurgical planning. Postoperative results in the five cases suggest that preoperative functional brain mapping has the potential to improve outcome by defining a surgical plan to maximize resection and minimize the risk of neurological sequelae.

School psychologists: strategic allies in the contemporary practice of primary care pediatrics.

Immense changes in the developmental/behavioral aspects of primary care pediatrics have resulted from revisions in special education laws, introduction of managed care, widened dissemination of information about disabilities to parents, and the changing character of American society. Challenges associated with contemporary pediatric practice can be diminished by routine collaboration with school psychologists. An alliance with school-based psychologists permits pediatricians access to children in their natural environments and potential collaboration for preventing illness and emotional/behavior problems. Further, school psychologists can provide a partner for assessing and treating both common and low-incident disorders without the constraints imposed by managed care.

Tourette's syndrome and psychopathology in a child psychiatry setting.

UNLABELLED: Prior investigations of psychopathology among children with Tourette's syndrome (TS) have rarely used child psychiatry samples and sophisticated personality instruments. OBJECTIVE: To produce an objectively derived composite TS personality profile and to determine the rate of particular problems in a TS psychiatry sample compared with children with out TS from the same clinical practice. METHOD: Children (n = 33) referred to child psychiatrists because of emotional and behavior problems who were subsequently also found to meet DSM-III-R criteria for TS were assessed by the Personality Inventory for Children. RESULTS: Children with TS expressed high rates of psychopathology overall (composite 2.7 SD elevated) with depression, anxiety, and peculiar behavior having the highest values; depression occurred most frequently (73%), and attention-deficit hyperactivity disorder (55%) was no more common than among comparison group children and conduct problems (18%) were rarer. "Depression, anxiety, tension, and excessive worry" were characteristic of the actuarially derived modal TS personality. CONCLUSIONS: The prevalence and manifestations of psychopathology of children with TS in a child psychiatry practice are not identical with those reported in the literature. Child psychiatrists should be particularly vigilant of depressive symptoms and expect to encounter relatively few conduct problems compared with children without TS. Establishing "local prevalence rates" for children with TS seeking psychiatric evaluation can help guide the diagnostician and make diagnosis more assured.

Childhood Tourette's syndrome and the Thematic Apperception Test: is there a recognizable pattern?

26 children, one-half of whom were diagnosed with Tourette's syndrome, were administered the Thematic Apperception Test. A pilot investigation suggested that children with Tourette's syndrome produced high rates of responses in four categories: references to physical aggression, supernatural power, character names, and specific quantities. When compared with other children treated for emotional and behavioral problems at the same facility and matched on demographic variables and IQ, children with Tourette's syndrome were not more likely to produce Thematic Apperception Test responses in any of the four categories. This study shows that a recognizable pattern on projective testing was not easily established among these 13 children with Tourette's syndrome and further highlights the importance of using mental health comparison groups when investigating clinical disorders.

Use of positron emission tomography for presurgical localization of eloquent brain areas in children with seizures.

Successful surgical management of a neoplastic or nonneoplastic seizure focus in close proximity to or within eloquent brain areas relies on precise delineation of the relationship between the lesion and functional brain areas. The aim of this series was to validate the usefulness and test the efficacy of noninvasive presurgical PET mapping of eloquent brain areas to predict surgical morbidity and outcome in children with seizures. To identify eloquent brain areas in 15 children (6 female and 9 male; mean age 11 years) with epileptogenic lesions PET images of regional cerebral blood flow were performed following the administration of [(15)O]water during motor, visual, articulation, and receptive language tasks. These images with coregistered magnetic resonance (MR) images were then used to delineate the anatomic relationship of a seizure focus to eloquent brain areas. Additional PET images using [18F]fluoro-2-deoxy-D-glucose (FDG) and [11C]methionine (CMET) were acquired to help localize the seizure focus, as well as characterize the lesion. Patient surgical management decisions were based on PET mapping in combination with coregistered MR images, PET-FDG findings, and the anatomic characteristics of the lesion. At follow-up 1-26 months after surgery, all patients that underwent temporal lobectomy (9 patients) and extratemporal resection (4 patients) for a neoplastic or nonneoplastic seizure focus are seizure-free with minimal postoperative morbidity. Of prime importance, no child sustained a postoperative speech or language deficit. PET imaging was also well tolerated without procedural complications. Based on PET mapping, a nonoperative approach was used for 2 children and a biopsy only was used in one child. When cortical injury involved prenatally determined eloquent cortex, PET demonstrated reorganization of language areas to new adjacent areas or even to the contralateral hemisphere. Integration of anatomical and functional data enhanced the surgical safety, defined optimal surgical approach, delineated the seizure focus from eloquent brain areas, facilitated maximum resection and optimized the timing of surgery, thereby minimizing surgical morbidity while maximizing surgical goals. PET measurements of FDG and CMET uptake were also helpful in localizing the seizure focus and grading the tumors. PET used for brain mapping in children provides the surgeon with strategic preoperative information not readily attainable with traditional invasive Wada testing or intraoperative cortical stimulation. PET mapping may also improve the outcome of extratemporal resections by allowing aggressive seizure focus resection. In addition, serial brain maps may optimize timing for surgical intervention by demonstrating reorganization of eloquent cortex often seen in younger children after cortical injury. Our results suggest that noninvasive presurgical brain mapping has the potential to reduce risk and improve neurologic outcome.

Positron emission tomography in children with neurofibromatosis-1.

Neurofibromatosis-1 is an autosomal dominant genetic disorder commonly associated with neuropsychological complications. Focal areas of high signal intensity on magnetic resonance imaging (MRI) scans occur commonly but have shown inconsistent correlation with neuropsychological problems. Positron emission tomography (PET) scans utilizing [18F]fluoro-2-deoxy-D-glucose and MRI studies were performed on 10 children with neurofibromatosis-1 and multiple focal areas of high signal intensity to evaluate the regional cerebral metabolic rate for glucose of these lesions and other central nervous system structures. Co-registered PET and MRI studies confirmed reduced glucose metabolism of large focal areas of high signal intensity. Visual inspection and semiquantitative analysis of PET images demonstrated thalamic hypometabolism and varying degrees of cortical inhomogeneity in all cases of neurofibromatosis-1 compared to normal controls. Although a primary defect of the thalamus or cerebral cortex has not been defined, the metabolic abnormalities of this study suggest a potential relationship between these structures and the neuropsychological dysfunctions noted in neurofibromatosis-1.