Biomedical subjects

M D Ris

Publications and source records attributed to M D Ris.

9 recordsLinked to original sources

PubMed1999

Neurologic validity of the Wisconsin Card Sorting Test with a pediatric population.

This study investigated the neurologic validity of the Wisconsin Card Sorting Test (WCST) with a pediatric population; that is, the ability of the test to detect dysfunction in the frontal lobes. Fifty children with diverse etiologies of brain dysfunction were classified via EEG, MRI, or CT as having left hemisphere, right hemisphere, or bilateral frontal, extrafrontal, or multifocal/diffuse regions of brain dysfunction. Findings failed to support the hypotheses that WCST performance is more impaired in frontal lesions than extrafrontal or multifocal/diffuse lesions, or that WCST performance is more impaired in left hemisphere lesions than right. Although the WCST is not helpful in localizing cerebral area of dysfunction, it may still be a clinically useful test for examining processes that children use to solve complex problems.

Adolescent↗

PubMed1999

Treatment of children with medulloblastomas with reduced-dose craniospinal radiation therapy and adjuvant chemotherapy: A Children's Cancer Group Study.

PURPOSE: Medulloblastoma is the most common malignant brain tumor of childhood. After treatment with surgery and radiation therapy, approximately 60% of children with medulloblastoma are alive and free of progressive disease 5 years after diagnosis, but many have significant neurocognitive sequelae. This study was undertaken to determine the feasibility and efficacy of treating children with nondisseminated medulloblastoma with reduced-dose craniospinal radiotherapy plus adjuvant chemotherapy. PATIENTS AND METHODS: Over a 3-year period, 65 children between 3 and 10 years of age with nondisseminated medulloblastoma were treated with postoperative, reduced-dose craniospinal radiation therapy (23.4 Gy) and 55.8 Gy of local radiation therapy. Adjuvant vincristine chemotherapy was administered during radiotherapy, and lomustine, vincristine, and cisplatin chemotherapy was administered during and after radiation. RESULTS: Progression-free survival was 86% +/- 4% at 3 years and 79% +/- 7% at 5 years. Sites of relapse for the 14 patients who developed progressive disease included the local tumor site alone in two patients, local tumor site and disseminated disease in nine, and nonprimary sites in three. Brainstem involvement did not adversely affect outcome. Therapy was relatively well tolerated; however, the dose of cisplatin had to be modified in more than 50% of patients before the completion of treatment. One child died of pneumonitis and sepsis during treatment. CONCLUSION: These overall survival rates compare favorably to those obtained in studies using full-dose radiation therapy alone or radiation therapy plus chemotherapy. The results suggest that reduced-dose craniospinal radiation therapy and adjuvant chemotherapy during and after radiation is a feasible approach for children with nondisseminated medulloblastoma.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1998

Recovery in pediatric brain injury: is psychostimulant medication beneficial?

OBJECTIVE: To assess the effects of methylphenidate on attention, memory, behavior, processing speed, and psychomotor skills of children with closed head injuries. DESIGN: Double-blind, placebo-controlled, crossover design. SETTING: An outpatient facility of a children's hospital medical center. PATIENTS: Ten pediatric subjects identified through chart review. Subjects met baseline scores for hyperactivity (Conner's Hyperactivity Index greater than or equal to 60) and intellectual functioning (Verbal Intelligence Quotient greather than or equal to 70) and achieved minimal scores on two psychometric tests. All subjects evidenced head injury by focal lesions on computed tomography scan and/or sequelae reported at the time of injury. Severity of injury ranged from mild to severe. All subjects were medically stable at the time of testing. Mean time post injury was 2 years, 8 months. INTERVENTION: Administration of methylphenidate and placebo. MAIN OUTCOME MEASURES: Percentage change in scores was calculated to assess differences between baseline and end of methylphenidate/placebo trials. RESULTS: No significant differences between methylphenidate and placebo on measures assessing behavior, attention, memory, and processing speed. CONCLUSIONS: The results of the study call into question the effectiveness of methylphenidate in the pediatric head injury population.

Adolescent↗

PubMed1997

Adult psychosocial outcome in early-treated phenylketonuria.

Concerns about the psychosocial risk of adults with early-treated phenylketonuria (ETPKU) are predicated on four sources of scientific data: (1) consistent documentation of increased behavioural risk in children with ETPKU; (2) recent evidence of neurocognitive impairment in adults with ETPKU; (3) reports of neuroimaging abnormalities in adults with ETPKU; and (4) preliminary evidence of increased rates of psychiatric disturbance in this population. We studied the psychosocial adjustment of 25 patients, aged 18 years and older, with ETPKU. On most psychosocial outcome measures, patients were indistinguishable from 15 sibling controls. However, on a self-report inventory of psychiatric symptoms, 20% of the patients demonstrated significant morbidity. Psychosocial outcome of these patients was unrelated to concurrent or historical biological dietary disease factors, unlike neurocognitive outcome. A strong relationship was demonstrated, however, between neurocognitive measures and psychosocial morbidity. These findings indicate that a significant minority of patients with ETPKU develop psychosocial difficulties with multiple clinical elevations on a psychiatric inventory. However, most adults with ETPKU cope with the challenges of young adulthood with the same degree of success as their unaffected siblings. Neuropsychological surveillance during childhood and adolescence is important in identifying patients at risk for both neurocognitive and psychosocial morbidity.

Adult↗

PubMed1996

Mood and global-local visual processing.

Testing hypotheses derived from neuropsychological models of mood, as well as the association of mood states and personality characteristics with global-local visual processing, were examined. Fifty-nine men completed measures associated with depression and positive mood, and were administered a brief perceptual judgment task that assessed global-local visual processing biases. Additionally, 19 of these 59 subjects were administered measures of anxiety and optimism-pessimism and completed an expanded judgment task. Affective and personality variables were then correlated with judgment task performances. Consistent with predictions, positive mood and optimism were directly associated with a global bias and inversely related to a local bias. A converse pattern of findings was obtained with depression and trait anxiety. Implications for research concerning other aspects of visual processing are discussed.

Adult↗

PubMed1995

Bone marrow transplantation in a young child with sickle cell anemia.

Bone marrow transplantation (BMT) is the only curative therapy available for hemoglobinopathies. BMT was performed on a young child with sickle cell anemia (SCA) after approximately 9 months of transfusion therapy following her initial stroke. The patient received a matched sibling donor (sickle trait) BMT. The conditioning regimen consisted of busulfan 4 mg/kg/day x 4, cyclophosphamide 50 mg/kg/day x 4. Graft vs. host disease prophylaxis was daily cyclosporine for 6 months. There were no significant complications during BMT. Engraftment occurred on day +17 and the patient was transfusion independent since day +45. Pre-BMT cerebral arteriography showed multiple stenotic cerebral vessels and a moya-moya pattern. Perfusion MRI demonstrated reduced capillary perfusion. Approximately 170 days after BMT the patient experienced episodes of transient left-sided weakness and speech problems. Neuroimaging revealed progression of large vessel pathology by angiography despite significant improvement in cortical perfusion (MR perfusion scan). Molecular analysis by PCR and DNA fingerprinting confirmed absence of mixed mosaicism. Rheologic evaluation showed normal corrected bulk viscosity. It is possible that progression of large vessel pathology and return of clinical symptoms in the face of normal rheologic parameters may be due to worsening of the already damaged cerebral vessels by the BMT conditioning regimen. Further evaluations of patients with SCA undergoing BMT after a stroke are needed to answer this question.

Anemia, Sickle Cell↗

PubMed1994

Early-treated phenylketonuria: adult neuropsychologic outcome.

Twenty-five adults with phenylketonuria that was treated early were compared with 15 unaffected control siblings with respect to intellectual and neuropsychologic measures. Patients were found to have normal intelligence but were significantly lower than their control siblings on measures of intelligence, attention, and complex visuoconstructional ability. Stepwise multiple regression analyses found the patients' intellectual outcome to be best predicted by indexes reflecting early insult to the brain, whereas performance on a measure of novel problem solving was best predicted by concurrent serum phenylalanine level. Different pathophysiologic mechanisms may thus account for cognitive deficits in this population. These results provide further evidence of continuing benefits of dietary adherence into adulthood.

Adult↗

PubMed1994

Long-term neurobehavioral outcome in pediatric brain-tumor patients: review and methodological critique.

This paper provides an overview of pediatric brain tumors and reviews the literature on long-term neurobehavioral outcomes for these children. Progress in this area has been slow due to numerous methodological and practical complications. While some general conclusions can be drawn, the development of comprehensive models awaits more research into the multiplicity of biological and nonbiological events, and their interactions, potentially affecting outcome. Increased theoretical and methodological sophistication will be needed to address this complex classification of diseases. Barriers to research in this area are enumerated as are suggestions for future efforts.

Adolescent↗

PubMed1992

Social interactions between children with cancer or sickle cell disease and their peers: teacher ratings.

We evaluated the social reputation of children with cancer (nonprimary brain tumors) (n = 26) children with a primary malignancy involving the central nervous system (n = 15), and children with sickle cell disease (SCD) (n = 33) using a standardized measure of social reputation, the Revised Class Play (RCP). Each child's classroom teacher completed the RCP, an instrument designed to assess sociability-leadership, aggressive-disruptive, and sensitive-isolated interpersonal qualities. Matched-pairs analyses comparing the child with chronic illness to one peer in each class who was the same gender/race and the closest date of birth showed children with cancer were nominated more often for sociability-leadership roles and less often for aggressive-disruptive roles. Brain tumor survivors were nominated more often for sensitive-isolated roles. Children with SCD were not significantly different from peers. Findings suggest that the stressful life events associated with cancer or SCD did not have a significant negative impact on social adjustment for school-aged children with these diseases. The data demonstrate the critical need for appropriate controls when evaluating psychosocial morbidity and chronic illness. Better understanding of the processes of resilience for these children who are routinely exposed to heightened stress provides an opportunity to improve the competence of other high-risk youth.

Adolescent↗