ContextBrain white matter hyperintensities are seen on routine clinical imaging in 46% of adults with congenital adrenal hyperplasia (CAH). The extent and functional relevance of these abnormalities have not been studied with quantitative magnetic resonance imaging (MRI) analysis.ObjectiveTo examine white matter microstructure, neural volumes, and central nervous system (CNS) metabolites in CAH due to 21-hydroxylase deficiency (21OHD) and to determine whether identified abnormalities are associated with cognition, glucocorticoid, and androgen exposure.Design, Setting, and ParticipantsA cross-sectional study at a tertiary hospital including 19 women (18 to 50 years) with 21OHD and 19 age-matched healthy women.Main Outcome MeasureRecruits underwent cognitive assessment and brain imaging, including diffusion weighted imaging of white matter, T1-weighted volumetry, and magnetic resonance spectroscopy for neural metabolites. We evaluated white matter microstructure by using tract-based spatial statistics. We compared cognitive scores, neural volumes, and metabolites between groups and relationships between glucocorticoid exposure, MRI, and neurologic outcomes.ResultsPatients with 21OHD had widespread reductions in white matter structural integrity, reduced volumes of right hippocampus, bilateral thalami, cerebellum, and brainstem, and reduced mesial temporal lobe total choline content. Working memory, processing speed, and digit span and matrix reasoning scores were reduced in patients with 21OHD, despite similar education and intelligence to controls. Patients with 21OHD exposed to higher glucocorticoid doses had greater abnormalities in white matter microstructure and cognitive performance.ConclusionWe demonstrate that 21OHD and current glucocorticoid replacement regimens have a profound impact on brain morphology and function. If reversible, these CNS markers are a potential target for treatment.

ABSTRACT. This study investigated emotional awareness, the recognition and understanding of different emotional states, among a non‐clinical population of adolescents and young adults with mental handicaps. While emotional awareness showed a high positive correlation with language comprehension, the results suggest that many of these individuals have specific emotional awareness deficits which are not in line with their language comprehension abilities. This finding suggests that the assessment of emotional awareness would be an important step in the choice of a self‐report measure to assess an individual's emotional state.

The issues arising from implementing an early intervention service, developed in the rural United States in the late 1960s in a range of different cultural contexts over a period of a quarter of a century, are explained. Services from India, Bangladesh, Jamaica and the United Kingdom are compared. As well as considering cross-cultural aspects of Portage, variability within one country, the United Kingdom, is considered by comparing one service in an inner-city area and one in a rural area.

This study investigated the use of an informant behaviour checklist to examine carestaff perceptions of sadness among a hospital population of people with mental handicaps. Using Cohen's kappa statistic there were only three checklist items which had acceptable levels of inter-rater agreement (physical aggression, crying and verbal abuse) with no significant variation in the incidence of those behaviours according to the severity of mental handicap. The flndings suggest that future research might attend to concepts of sadness among carestaff and their ability to identify sad emotional states in a reliable manner.

Boys and girls differed in their willingness to interact with unfamiliar peers with and without facial distinctions. Various explanations were given to explain causality of the anomaly. Findings lend some support to the proposal that high "background attractiveness" can overshadow the impact of a craniofacial anomaly.