BACKGROUNDIncreased intensities of routinised and compulsive-like behaviours are seen in those with intellectual disabilities and have sometimes been shown to be associated with worries. We used the Childhood Routines Inventory (CRI, Evans et al., 1997) with two samples of children and adults with Down syndrome: (1) to determine whether routinised and compulsive-like behaviours were associated with mental health problems and (2) to determine the factor structure of the CRI.METHODParents or carers completed the CRI for (1) 125 adults with Down syndrome (aged 18-43 years) who had been assessed for mental health problems; worries and fears were also rated by parents/carers and (2) 206 individuals with Down syndrome (aged 4.5-43 years, with verbal mental ages of 2 years and above).RESULTS(1) People with a psychiatric diagnosis had significantly more worries and fears than those without such a diagnosis, but there was no significant difference in CRI scores. Logistic regression indicated that the fear rating was the only significant predictor of a diagnosis. (2) Exploratory and confirmatory analyses showed a three-factor model (Just right, Repetitive behaviour and Clothes sensitivity) to be the best solution. Those with psychiatric diagnoses had significantly higher ratings on the Repetitive behaviour factor.CONCLUSIONIncreased levels of routinised and compulsive-like behaviours were shown by individuals with Down syndrome of all ages, were not associated with mental health problems, but were associated with worries and fears. Factor analysis found three factors, two of which (Just right and Repetitive behaviours), were similar to those identified in typically developing samples. This suggests that the behaviours have similar adaptive functions in individuals with developmental delays.

Background. Individuals with Down's syndrome (DS) are at high risk of developing Alzheimer's disease (AD). However, few studies have investigated brain anatomy in DS individuals with AD.Method. We compared whole brain anatomy, as measured by volumetric magnetic resonance imaging (MRI), in DS individuals with and without AD. We also investigated whether volumetric differences Could reliably classify DS individuals according to AD status. We used volumetric MRI and manual tracing to examine regional brain anatomy in 19 DS adults with AD and 39 DS adults without AD.Results. DS individuals with AD had significantly smaller corrected volumes bilaterally of the hippocampus and caudate, and right amygdala and putamen, and a significantly larger corrected volume of left peripheral cerebrospinal fluid (CSF), compared to DS individuals without AD. The volume of the hippocampus and caudate nucleus correctly categorized 92% and 92% respectively of DS individuals Without AD, and 75% and 80%, respectively of DS individuals with AD.Conclusions. DS individuals with AD have significant medial temporal and striatal Volume reductions, and these may provide markers of clinical AD.

BackgroundPeople with Down's syndrome (DS) are at high risk for developing dementia in middle age. The biological basis for this is unknown. It has been proposed that non-demented adults with DS may undergo accelerated brain ageing.MethodWe used volumetric magnetic resonance imaging (MRI) and manual tracing to compare brain anatomy and ageing in 39 non-demented adults with DS and 42 healthy controls.ResultsIndividuals with DS had significant differences in brain anatomy. Furthermore, individuals with DS had a significantly greater age-related reduction in volume of frontal, temporal and parietal lobes, and a significantly greater age-related increase in volume of peripheral cerebrospinal fluid (CSF).ConclusionsNon-demented adults with DS have differences in brain anatomy and ‘accelerated’ ageing of some brain regions. This may increase their risk for age-related cognitive decline and Alzheimer's disease (AD).

There is a reported association between thyroid disorders and Down syndrome, but is this association based on valid and reliable research evidence? We evaluated thyroid function test results of 110 healthy adults with Down syndrome to determine biochemical thyroid status. Approximately two thirds were biochemically euthyroid when assessed by standard reference ranges for the general population. We believe that there is a need for revalidation of "normal" thyroid function tests parameters when applied to the Down syndrome population and that persons with Down syndrome are possibly being misdiagnosed and inappropriately treated for a nonexistent medical disorder.

CONTEXT:Down syndrome (DS) is the most common genetic cause of mental retardation. However, the biological determinants of this are poorly understood. The serum sodium/myo-inositol cotransporter gene is located on chromosome 21, and myo-inositol affects neuronal survival and function. Nevertheless, few in vivo studies have examined the role of myo-inositol in DS.OBJECTIVE:To determine if people with DS have significant differences in brain myo-inositol concentration from controls and if, within people with DS, this is related to cognitive ability.DESIGN:A case-control study.SETTING:Outpatient.PARTICIPANTS:The sample was composed of 38 adults with DS without dementia (age range, 18-66 years) and 42 healthy controls (age range, 19-66 years). The DS and control groups did not differ significantly in age, sex, ethnic origin, apolipoprotein E status, or handedness.MAIN OUTCOME MEASURES:Hippocampal myo-inositol concentration and cognitive performance, as measured by the Cambridge Cognitive Examination.RESULTS:Hippocampal myo-inositol concentration was significantly higher in people with DS than in controls (P = .006), and within people with DS, increased myo-inositol concentration was significantly negatively correlated with overall cognitive ability (P = .04).CONCLUSIONS:Adults with DS have a significantly increased brain concentration of myo-inositol, and this is associated with reduced cognitive ability. Future studies are required to relate myo-inositol concentration in people with DS to brain development and increased risk for developing Alzheimer disease.

Journal of Applied Research in Intellectual DisabilitiesVolume 14, Issue 4 p. 413-414 Younger People with Dementia: Planning, Practice and Development V. Prasher, V. Prasher Department of Psychiatry, University of Birmingham, Birmingham, UKSearch for more papers by this author V. Prasher, V. Prasher Department of Psychiatry, University of Birmingham, Birmingham, UKSearch for more papers by this author First published: 10 September 2009 https://doi.org/10.1111/j.1468-3148.2001.00054.xRead the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat Volume14, Issue4December 2001Pages 413-414 RelatedInformation

There continues to be a steady growth in the published literature regarding issues for individuals with Down syndrome. This review highlights recent articles that contribute to the present scientific knowledge of the family, social, behavioural and health issues affecting both children and adults with Down syndrome. (C) 2001 Lippincott Williams & Wilkins.

Seven men with autistic disorders developed mood disorders or psychotic illnesses during adult life. This association has also been described in several previous reports. Although no firm conclusions can be drawn on the basis of case reports alone, it appears that autistic disorders may increase vulnerability to such psychoses. Possible reasons for the association are discussed, as is treatment.

In agreement with previous studies we show an age-related increase in serum levels of hyaluronic acid in healthy individuals. Levels in Down's syndrome persons were slightly higher, especially in those with hypothyroidism. This provides further evidence that the normal ageing process is accelerated in Down's syndrome.

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End‐stage dementia in adults with Down syndrome has not been fully investigated. Available information, 6 months prior to death, for 20 adults with Down syndrome who had died with Alzheimer's disease was reviewed. A terminal stage of severe intellectual deterioration, marked personality and mood changes, loss of sphincter control, seizure activity, immobility with hypertonia and complete loss of self‐care skills was found. These findings have important clinical and service implications.

ICD-10 Diagnostic Criteria for Research (DCR) based on the Clinical Descriptions and Diagnostic Guidelines for Mental and Behavioural Disorders were used to assign diagnoses to 38 people with mental retardation (MR) who had additional behavioural or psychiatric disorders. Problems were encountered with criteria for schizophrenia and related disorders. Recommendations were made regarding some diagnostic criteria, the method of coding diagnoses, and the need for additional guidance when classifying disorders associated with MR.Multiaxial classification during the field trial allowed mental and physical health problems and the severity of MR to be summarised in a standard format, assisting record keeping and the communication of information. The DCR represent an advance on previous diagnostic systems, and with additional axes (now being developed for use with a MR fascicle) will be a useful research tool. Psychiatric diagnoses and classificatory systems still suffer from the lack of external validating criteria or specific treatment responses for many disorders within them, and this poses special problems when the person with the disorder has difficulties with communication.

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The plasma distribution of gallium (as an analogue of aluminium) was investigated in patients with Alzheimer disease, Down syndrome, or stroke dementia, in subjects on haemodialysis for chronic renal failure, and in healthy controls. Gallium-transferrin binding was significantly lower in the Alzheimer (mean [SEM] 7.9 [1.1]%) and Down syndrome groups (6.9 [0.7]%) than in the controls (17.1 [1.6]%), whereas stroke dementia and haemodialysis patients had normal binding. There were no differences among the groups in plasma citrate concentration. The plasma transferrin concentration was slightly lower in the Alzheimer and Down syndrome groups than in the controls, but even lower in stroke dementia patients (1.74 [0.14] g/l vs 2.98 [0.18] g/l in controls). Transferrin iron saturation was higher in the Alzheimer (58.9%) and Down syndrome groups (81.6%) than in the controls (39.0%) or stroke dementia patients (33.4%). This deficiency of gallium/aluminium binding would leave more unbound aluminium which could move readily into the brain, where it has neurotoxic effects.