ABSTRACT
Hematopoietic stem cell transplantation (HSCT) is a highly successful treatment for severe congenital immunodeficiencies. However, some studies have suggested that children may experience cognitive difficulties after HSCT. This large-scale study assessed cognitive and behavioral function for the cohort of children treated by HSCT at one center between 1979 and 2003 to determine the frequency and severity of problems and to identify risk factors. A total of 105 patients were assessed on standardized measures of cognitive and emotional and behavioral function together with a control group of unaffected siblings. The average IQ for the cohort was 85 (95% confidence interval, 81-90), significantly lower than both the population average of 100 (P < .001) and unaffected siblings. Multivariate analysis indicated that the underlying genetic defect, diagnosis of adenosine deaminase-deficient severe combined immunodeficiency, and consanguinity were associated with worse outcome but that age at transplantation and chemotherapy conditioning were not. Children treated by HSCT for severe immunodeficiency have an increased risk of long-term cognitive difficulties and associated emotional and behavioral difficulties. The specific genetic diagnosis, consanguinity, and severe clinical course are associated with poor outcome. Long-term follow-up of these patients should include screening to identify and manage these problems more effectively.
Subject(s)
Child Behavior Disorders/etiology , Cognition Disorders/etiology , Hematopoietic Stem Cell Transplantation/adverse effects , Immunologic Deficiency Syndromes/psychology , Immunologic Deficiency Syndromes/therapy , Adenosine Deaminase/deficiency , Adolescent , Adult , Affective Symptoms/etiology , Age Factors , Case-Control Studies , Child , Child, Preschool , Cohort Studies , Consanguinity , Female , Follow-Up Studies , Hematopoietic Stem Cell Transplantation/psychology , Humans , Immunologic Deficiency Syndromes/congenital , Immunologic Deficiency Syndromes/genetics , Intelligence , Male , Severe Combined Immunodeficiency/congenital , Severe Combined Immunodeficiency/genetics , Severe Combined Immunodeficiency/psychology , Severe Combined Immunodeficiency/therapy , Time FactorsABSTRACT
OBJECTIVES: The current study aimed to obtain older adult normative data on a neuropsychological battery in relation to functions underlying driving ability. The effect of age on performance on the battery was previously unknown; normative data revision was necessary to enable more appropriate use of the battery with older clients. DESIGN: Cross-sectional cohort study. METHODS: Volunteers were sought from healthy older people living independently in the community to complete the Rookwood Driving Battery. A group of 202 volunteers above the age of 70 were recruited from local social groups to complete the battery. Of these, 184 completed a screening test of cognitive integrity (Mini Mental State Examination, MMSE). The age ranged from 70 to 96 (mean age=81 years, SD=5.438). In the total sample, 155 (77%) were females. RESULTS: Results of the MMSE indicated that 161 (87.5%) of the group fell above a cut-off (25/30) typically used in epidemiological studies to identify age-related cognitive decline. Of these cognitively intact volunteers, performance was marked by higher battery error scores (mean=5.12, SD=3.75) than those observed in an earlier normative study using younger volunteers below 70 years of age (mean=1.41, SD=1.87). The two age groups differed significantly on all 10 battery subtests; in all cases the level of significance was .002 or less; for nine subtests, significance fell below .001. In the 'intact' older group, battery performance was observed to be closely related to score on the MMSE, a test of general cognitive integrity (r=.558, p=.01). CONCLUSIONS: Performance on the Rookwood Battery differs for the over 70s and under 70s. The authors suggest essential modifications in its use with older people.
