A double‐blind, parallel, multicenter comparison of L‐acetylcarnitine with placebo on the attention deficit hyperactivity disorder in fragile X syndrome boys [L-acetylcarnitine treatment on fragile X patients hyperactive behaviour]. Language impairment in dementia: impact on symptoms and care needs in residential homes. Cognitive behavior therapy for comorbid obsessive-compulsive disorder in high-functioning autism spectrum disorders: a randomized controlled trial. Effects of intranasal oxytocin on social anxiety in males with fragile X syndrome The effectiveness of methylphenidate in the management of Attention Deficit Hyperactivity Disorder (ADHD) in people with intellectual disabilities: A systematic review. Outcome of teenage-onset anorexia nervosa in a Swedish community-based sample The efficacy of melatonin for sleep problems in children with autism, fragile X syndrome, or autism and fragile X syndrome. Theoretical exploration of the neural bases of behavioural disinhibition, apathy and executive dysfunction in preclinical Alzheimer's disease in people with Down's syndrome: potential involvement of multiple frontal-subcortical neuronal circuits. Using computers to teach people with intellectual disabilities to perform some of the tasks used within cognitive behavioural therapy: A randomised experiment. Manualised Individual Cognitive Behavioural Therapy for mood disorders in people with mild to moderate intellectual disability: a feasibility randomised controlled trial. Autism in adults: symptom patterns and early childhood predictors. Use of the DISCO in a community sample followed from childhood. Twenty-four-week treatment with extended release methylphenidate improves emotional symptoms in adult ADHD Impact of comorbid autism spectrum disorders on stimulant response in children with attention deficit hyperactivity disorder: a retrospective and prospective effectiveness study. Fetal alcohol spectrum disorders among children in a Brazilian orphanage. Group-based cognitive-behavioural anger management for people with mild to moderate intellectual disabilities: cluster randomised controlled trial. Adults with intellectual disabilities: prevalence, incidence and remission of self-injurious behaviour, and related factors. Does training improve understanding of core concepts in cognitive behaviour therapy by people with intellectual disabilities? A randomized experiment. Effects of a few food diet in attention deficit disorder. A prospective open-label study of aripiprazole in fragile X syndrome Managing behavioural and psychological symptoms in community dwelling older people with dementia: 1. A systematic review of the effectiveness of interventions Fifteen-year follow-up of 92 hospitalized adults with Down's syndrome: incidence of cognitive decline, its relationship to age and neuropathology. Emotion regulation as mediator of treatment outcome in therapy for deliberate self-harm.
Attention deficit hyperactivity disorder ( ADHD ) is a frequent behavioral problem in young boys with fragile X syndrome ( FXS ) , and its treatment is critical for improving social ability . The short‐term efficacy of stimulant medications like methylpheni date ( MPH ) is well established in children with ADHD . FXS boys treated with MPH have improved attention span and socialization skills ; however their mood becomes unstable at higher doses . Therefore , alternative pharmacological treatment of ADHD symptoms is desirable . A recent study showed that carnitine has a beneficial effect on the hyperactive – impulsive behavior in boys with ADHD without side effects . Our previous placebo‐controlled trial indicated that L‐acetylcarnitine ( LAC ) reduces hyperactivity in FXS boys . The objective of this study was to determine the efficacy of LAC in a larger sample of FXS boys with ADHD . The study design was r and omized , double blind placebo controlled , parallel , and multicenter ( with eight centers involved in Italy , France , and Spain ) . Sixty‐three FXS males with ADHD ( aged 6–13 years ) were enrolled ; 7 patients dropped out , 56 completed the one‐year treatment , and 51 were included in the statistical analysis . Both groups improved their behavior , showing that psychosocial intervention has a significant therapeutic effect . However , we observed a stronger reduction of hyperactivity and improvement of social behavior in patients treated with LAC , compared with the placebo group , as determined by the Conners ' Global Index Parents and the Vinel and Adaptive Behavior Scale . Our results show that LAC ( 20–50 mg/kg/day ) represents a safe alternative to the use of stimulant drugs for the treatment of ADHD in FXS children . © 2008 Wiley‐Liss , Hyperactivity is a significant problem for almost all young males affected by fragile X syndrome ( FXS ) , the most common inherited disease causing mental retardation . Therapeutical approaches are actually based on Central Nervous System ( CNS ) stimulants lacking a well defined rationale and efficacy while they further decrease the patient 's limited attention span . A pilot study on 17 fragile X male treated with L-acetylcarnitine ( LAC ) over one year , showed a significant reduction of their hyperactivity behaviour tested by the Conners Abbreviated Parent-Teacher Question naire . LAC use in FXS patients derives from the hypothesis that the biochemical and physiological properties this substance has may preserve brain activity . LAC is a small , hydrosoluble molecule that easily diffuses in the extracellular space and enters any cell in the nervous system through specific transporters . Different cerebral areas use this molecule differently to metabolize glucose and lipids to provide for ATP and neurotrasmitters synthesis . The acetyl group LAC carriers represents a key metabolic signaling element possibly mediating its effect in the CNS . The exogenous administration of LAC may affect brain activity in FXS by : I ) modulation of fuel partitioning for energy production , which at the mithocondrial level is associated with the Kreb 's cycle metabolic role in neurotransmitter synthesis ; II ) remodelling of lipid membrane in terms of LAC actively determining the production of polyunsaturated fatty acids ; III ) preferential effect on the attention component of the cholinergic system which relies on its peculiar modality of communication in the CNS . Based on the above premises an explorative , double-blind , placebo controlled , multicenter study is ongoing . A total population of 160 children from nine European centers will be enrolled . The objective of this study is to determine the effect of LAC on the hyperactive behaviour of FXS children as evaluated by the administration of the Conners Abbreviated Parent Question naire BACKGROUND Impairment of language skills affects the level of functioning of an individual , interferes with effective communication and can result in development of disruptive behaviour . Social skills and capacity for self care may be compromised . Few studies have evaluated the impact of language problems on symptoms and socialization in people with dementia in care environments . METHOD 315 elderly residents with dementia ( 29 % living in nursing homes , 71 % in social care facilities ) were assessed using st and ardized psychiatric schedules including the Sheffield Screening Test for Acquired Language Disorders and Neuropsychiatric Inventory . Dementia Care Mapping was undertaken at r and om in at least 50 % of residents in each facility . RESULTS Expressive language impairment was associated with the presence of delusions even when severity of dementia was controlled for ( p=0.02 ) and showed a tendency of association with depression ( p=0.06 ) . Receptive language difficulties were strongly associated with presence of Aberrant Motor Behaviour , even controlling for severity of dementia ( p=0.04 ) . Decreased participation in social activities was correlated with both expressive ( p=0.048 ) and receptive aspects of language ( p<0.01 ) but social withdrawal was only correlated with receptive language difficulties ( p=0.01 ) . CONCLUSION Language disorders are associated with both behavioural and psychological symptoms of dementia even when severity of dementia is controlled for . Patients ' needs in communication skills should be addressed earlier to help them maintain social interactions and reduce the impact on behavioural problems and patients ' quality of life BACKGROUND High rates of anxiety disorders , particularly obsessive compulsive disorder ( OCD ) are reported in people with Autism spectrum disorders ( ASD ) .  Group cognitive behavioral treatment ( CBT ) has been found effective for anxiety in young people with ASD but not been OCD specific . One uncontrolled pilot study of individual CBT for OCD for adults with ASD showed good treatment efficacy . METHODS Forty-six adolescents and adults ( mean age 26.9 years , 35 Males ) with ASD and comorbid OCD were r and omized to CBT for OCD or anxiety management ( AM ) , a plausible control treatment . Treatments were matched in duration ( mean of 17.4 sessions CBT ; 14.4 sessions AM ) , the Yale-Brown Obsessive Compulsive Severity Scale ( YBOCS ) as primary outcome measure and evaluations blind to treatment group . Treatment response was defined as > 25 % reduction in YBOCS total severity scores . RESULTS Both treatments produced a significant reduction in OCD symptoms , within-group effect sizes of 1.01 CBT group and 0.6 for the AM group . There were no statistically significant differences between the two groups at end of treatment , although more responders in the CBT group ( 45 versus 20 % ) . Effect sizes for self-rated improvement were small ( 0.33 CBT group ; -0.05 AM group ) . Mild symptom severity was associated with improvement in the AM but not the CBT group . Family/carer factors were important for both groups , in that increased family accommodation was associated with poorer outcome . CONCLUSIONS Evidence -based psychological interventions , both AM and CBT , were effective in treating comorbid OCD in young people and adults with ASD Fragile X syndrome ( FXS ) is a rare inherited genetic disorder causing severe intellectual disability and autistic-like symptoms . Individuals with FXS , males in particular , often exhibit extreme eye gaze avoidance and hyperarousal when they encounter stressful social situations . We investigated whether oxytocin ( OT ) , a hormone with prosocial and anxiolytic effects , could alleviate symptoms of social anxiety in this population . A r and omized double-blind placebo-controlled single-dose trial was performed with intranasal administration of placebo , 24 IU OT and 48 IU OT . Measures of eye gaze frequency , heart rate , respiratory sinus arrhythmia ( RSA ) , heart rate variability ( HRV ) and salivary cortisol were obtained during a structured social challenge conducted 50 min following OT administration . Ten low-functioning males with FXS ( aged 13 - 28 years ) traveled to Stanford for the initial visit : 8 completed the study .  Eye gaze frequency improved significantly in response to the 24 IU OT dose and salivary cortisol levels decreased significantly in response to the 48 IU OT dose . There was no effect of OT on heart rate , RSA or HRV although individual plots of the heart rate data suggested that OT increased heart rate in some participants and decreased heart rate in others . These findings suggest that intranasal administration of OT may ameliorate some symptoms of social anxiety in patients with FXS . Further double-blind placebo-controlled studies of OT , conducted in combination with behavioral treatment programs , may be warranted BACKGROUND The effectiveness of psychostimulants , primarily methylpheni date ( MPH ) , in the treatment of Attention Deficit Hyperactivity Disorder ( ADHD ) in the general population of typically growing children and adolescents is well established through many R and omised Controlled Trials ( RCTs ) . AIMS AND METHODS We carried out a systematic review of all the RCTs in people with intellectual disabilities ( ID ) that assessed effectiveness of MPH on the core ADHD symptoms . OUTCOMES AND RESULTS We included 15 papers from 13 studies that were all on children and adolescents with ID ( 315 participants were on MPH and placebo respectively ) , 12 of which used a cross over design , and one used a parallel design . On average around 40 - 50 % responded to MPH in the ID group whereas around 70 - 80 % response rate is reported among the non-ID children . Because of the heterogeneity of the outcome data it was not possible to carry out a meta- analysis . Significant adverse events included sleep difficulties and poor appetite along with weight loss and also irritability , social withdrawal and increased motor activities including tic . CONCLUSIONS AND IMPLEMENTATION On the basis of the poor quality evidence that is available , it seems that MPH may be effective in some but not all children and adolescents with ID and ADHD Abstract . In a prospect i ve long-term outcome study of a representative sample of teenage-onset anorexia nervosa ( AN ) , 51 individuals with AN , recruited after community screening , were contrasted with 51 matched comparison cases at a mean age of 24 years ( 10 years after AN onset ) . All 102 cases had been examined at age 16 and 21 years . At 24 years all prob and s were interviewed regarding mental and physical health , and overall outcome was assessed . Ten-year outcome of teenage-onset AN seemed to be relatively favourable in that half of all cases were free from eating disorder ( ED ) and other axis I disorder . There were no deaths . However , one in four in the AN group had a persisting ED , 3 of whom still had AN . Lifetime diagnoses of affective disorders and obsessive-compulsive disorder ( OCD ) were overrepresented in the AN group . Affective disorders coincided with the ED , and were not a problem after recovery from the ED . On the other h and , OCD , OCPD ( obsessive-compulsive personality disorder ) , and /or autism spectrum disorder continued to characterise more than one-third of the AN cases . One in six of the AN group had persistent problems with social interaction and obsessive compulsive behaviours from childhood into early adult years . Half the AN group had a poor overall outcome . These were subjects with either persisting ED or lifelong problems with social interaction and obsessive compulsive behaviour STUDY OBJECTIVE To determine the efficacy of melatonin on  sleep problems in children with autistic spectrum disorder ( ASD ) and fragile X syndrome ( FXS ) . METHODS A 4-week , r and omized , double blind , placebo-controlled , crossover design was conducted following a 1-week baseline period . Either melatonin , 3 mg , or placebo was given to participants for 2 weeks and then alternated for another 2 weeks . Sleep variables , including sleep duration , sleep-onset time , sleep-onset latency time , and the number of night awakenings , were recorded using an Actiwatch and from sleep diaries completed by parents . All participants had been thoroughly assessed for ASD and also had DNA testing for the diagnosis of FXS . RESULTS Data were successfully obtained from the 12 of 18 subjects who completed the study ( 11 males , age range 2 to 15.25 years , mean 5.47 , SD 3.6 ) . Five participants met diagnostic criteria for ASD , 3 for FXS alone , 3 for FXS and ASD , and 1 for fragile X premutation . Eight out of 12 had  melatonin  first . The conclusions from a nonparametric repeated- measures technique indicate that mean night sleep duration was longer on melatonin than placebo by 21 minutes ( p = .02 ) , mean sleep-onset latency was shorter by 28 minutes ( p = .0001 ) , and mean sleep-onset time was earlier by 42 minutes ( p = .02 ) . CONCLUSION The results of this study support the efficacy and tolerability of melatonin treatment for sleep problems in children with ASD and FXS BACKGROUND Recent research has suggested a specific impairment in frontal-lobe functioning in the pre clinical stages of Alzheimer 's disease ( AD ) in people with Down 's syndrome ( DS ) , characterised by prominent changes in personality or behaviour . The aim of the current paper is to explore whether particular kinds of change ( namely executive dysfunction ( EDF ) , disinhibition and apathy ) , associated in the literature with disruption of different underlying frontal-subcortical circuits , are a ) more or less frequently reported than others and b ) related to poor performance on tasks involving different cognitive processes . METHOD Seventy-eight participants ( mean age 47 years , range 36 - 72 ) with DS and mild to moderate intellectual disability ( based on ICD-10 criteria ) , without a diagnosis of dementia of Alzheimer 's type ( DAT ) or other psychiatric disorders , were selected from a larger sample of older adults with DS ( n = 122 ) . Dementia diagnosis was based on the CAMDEX informant interview , conducted with each participant 's main carer . Informant-reported changes in personality/behaviour and memory were recorded . Participants were scored based on symptoms falling into three behavioural domains and completed five executive function ( EF ) tasks , six memory tasks ( two of which also had a strong executive component ) and the BPVS ( as a measure of general intellectual ability ) . Multiple regression analyses were conducted to determine the degree to which the behavioural variables of ' EDF ' , ' disinhibition ' and ' apathy ' , along with informant-reported memory decline and antidepressant medication use , predicted performance on the cognitive tasks ( whilst controlling for the effects of age and general intellectual ability ) . RESULTS Strikingly , disinhibited behaviour was reported for 95.7 % of participants with one or more behavioural change ( n = 47 ) compared to 57.4 % with reported apathy and 36.2 % with reported EDF . ' Disinhibition ' score significantly predicted performance on three EF tasks ( design ed to measure planning , response inhibition and working memory ) and an object memory task , ( also thought to place high dem and s on working memory ) , while ' apathy ' score significantly predicted performance on two different tasks , those measuring spatial reversal and prospect i ve memory ( p < 0.05 ) . Informant reported memory decline was associated only with performance on a delayed recall task while antidepressant medication use was associated with better performance on a working memory task ( p < 0.05 ) . CONCLUSION Observed dissociation between performance on cognitive tasks associated with reported apathy and disinhibition is in keeping with proposed differences underlying neural circuitry and supports the involvement of multiple frontal-subcortical circuits in the early stages of DAT in DS . However , the prominence of disinhibition in the behavioural profile ( which more closely resembles that of disinhibited subtype of DFT than that of AD in the general population ) leads us to postulate that the serotonergically mediated orbitofrontal circuit may be disproportionately affected . A speculative theory is developed regarding the biological basis for observed changes and discussion is focused on how this underst and ing may aid us in the development of treatments directly targeting underlying abnormalities AIMS Training has been shown to improve the ability of people with intellectual disabilities ( IDs ) to perform some cognitive behavioural therapy ( CBT ) tasks . This study used a computerised training paradigm with the aim of improving the ability of people with IDs to : a ) discriminate between behaviours , thoughts and feelings , and b ) link situations , thoughts and feelings . METHODS Fifty-five people with mild-to-moderate IDs were r and omly assigned to a training or attention-control condition in a single-blind mixed experimental design . Computerised tasks assessed the participants ' skills in : ( a ) discriminating between behaviours , thoughts and feelings ( separately and pooled together ) , and ( b ) cognitive mediation by selecting appropriate emotions as consequences to given thoughts , and appropriate thoughts as mediators of given emotions . RESULTS Training significantly improved ability to discriminate between behaviours , thoughts and feelings pooled together , compared to the attention-control condition , even when controlling for baseline scores and IQ . Large within-group improvements in the ability to identify behaviours and feelings were observed for the training condition , but not the attention-control group . There were no significant between-group differences in ability to identify thoughts , or on cognitive mediation skills . CONCLUSIONS A single session of computerised training can improve the ability of people with IDs to underst and and practise CBT tasks relating to behaviours and feelings . There is potential for computerised training to be used as a " primer " for CBT with people with IDs to improve engagement and outcomes , but further development on a specific computerised cognitive mediation task is needed BACKGROUND Evaluation of complex interventions , including st and ardisation of the intervention , types of outcomes selected and measures of change , is a fairly novel concept in the field of intellectual disabilities . Our aim was to explore these issues in a feasibility study of Manualised Individual Cognitive Behaviour Treatment ( M-iCBT ) compared to the treatment as usual alone ( TAU ) . METHODS Service users with mild to moderate intellectual disability experiencing a mood disorder or symptoms of depression and /or anxiety ( mini PAS-ADD total score > 10 or 7 respectively ) were r and omly assigned to either . RESULTS In total , 32 participants were r and omly assigned to 16 sessions of M-iCBT ( n=16 ) in addition to TAU or TAU alone ( n=16 ) . We explored recruitment and accrual rates , willingness to participate , acceptability of the intervention and suitability of assessment tools . Mean change ( 95 % CI ) in the Beck Depression Inventory-Youth ( BDI-Y ) score from baseline to the 16 week endpoint ( primary variable ) was 0.10 ( 95 % CI : -8.56 , 8.76 ) and in the Beck Anxiety Inventory-Youth ( BAI-Y ) 2.42 ( 95 % CI : -5.27 , 10.12 ) in favour of TAU . However , there was a clear trend in favour of CBT in depressed participants with or without anxiety . LIMITATIONS The intervention targeted both depression and anxiety following a transdiagnostic model . This may have impacted the anticipated size of change in the primary outcome . The precise impact of cognitive limitations on ability to use therapy effectively is not fully understood . CONCLUSIONS This study demonstrates that it is feasible to carry out a pragmatic r and omised controlled trial of M-iCBT for people with mild to moderate intellectual disability . However , uncertainties about its clinical and cost effectiveness can only be fully answered by further examination of its superiority against other treatments BACKGROUND Few studies have looked at the very long-term outcome of individuals with autism who were diagnosed in childhood . METHODS A longitudinal , prospect i ve , community-based follow-up study of adults who had received the diagnosis of autism ( classic and atypical ) in childhood ( n = 105 ) was conducted . A structured interview ( the Diagnostic Interview for Social and COmmunication disorders -- the DISCO ) was used in order to evaluate symptoms and symptom patterns 13 - 22 years after original diagnosis . Childhood measures , including IQ-level at time of childhood diagnosis and communicative speech registered before age 5 years , were studied in relation to the presence of autism symptoms at follow-up . RESULTS The classical and atypical autism groups were fairly homogeneously impaired in terms of symptoms in the social interaction category whereas other common childhood autism symptoms , including maladaptive and stereotyped behaviours , were more variable in the study group at follow-up . Odd responses to sensory stimuli were still extremely common . Speech before 5 years of age , IQ , gender , diagnosed medical disorder and onset of epilepsy before 5 years were variables that correlated to outcome on the DISCO algorithm for autistic spectrum disorders ( Wing & Gould , 1979 ) concerning style and quality of social interaction , communication style and pattern of self-chosen activities . CONCLUSIONS Social interaction problems were still present in the vast majority of adults with autism/atypical autism , but behavioural impairments were much more variable in adulthood . Almost all cases were reported to show persistent perceptual problems . Certain childhood measures were found to prospect ively predict adult social interaction style , communication type , and pattern of self-chosen activities , which still met diagnostic criteria for autism/atypical autism in adulthood Abstract Objectives . Treatment investigations with methylpheni date in adults with ADHD focus preferentially on the classical psychopathology : inattention , hyperactivity and impulsivity . ADHD-associated emotional symptoms , which are frequently present at least in ADHD sub population s , were studied rarely . The vast majority of the placebo-controlled trials had observation periods between 4 and 8 weeks . To assess the medium- to long-term effects of extended release methylpheni date ( MPH-ER ) on emotional symptoms and other psychopathology frequently seen in ADHD patients , we conducted a large-scale , multicenter treatment study . Methods . We performed a r and omised , 24-week , double-blind , placebo-controlled study in adults with ADHD . The diagnosis was made on the basis of the DSM-IV criteria , which were confirmed by clinical history and a structured psychopathological interview and the use of rating instruments . 363 patients were r and omized to MPH-ER or placebo at a ratio of 2:1 . The duration of the titration period was 5 weeks followed by a maintainance phase of 19 weeks . The efficacy measures were the observer rated 10-item Emotional Dysregulation Scale ( EDS ) derived from the Wender-Reimherr Adult Attention Deficit Disorder Scale ( WRAADDS ) and a self-report , six-item Emotional Lability Scale ( ELS ) extracted from the long version of the Conners Adult ADHD Self Report Scale ( CAARS : S : L ) . In addition we used the SCL-90-R for the assessment of ADHD associated and comorbid psychopathology . Results . MPH-ER was statistically superior to placebo in reducing emotional symptoms as assessed by the EDS and the ELS . Obsessive-compulsive symptoms and those of problems with self-concept declined until the end of the observation period . The decline was more pronounced in MPH-ER treated individuals . The effects remained robust during the entire maintenance period until week 24 . Symptoms of anxiety , depression , anger and hostility , phobia , paranoid ideations and psychoticism were not improved . Conclusions . MPH-ER appears to be an efficacious treatment for emotional symptoms with ADHD . Also obsessive-compulsive symptoms and problems with self-concept were affected positively BACKGROUND In the recent past , psychiatrists and paediatricians have avoided prescribing stimulant medication , such as methylpheni date and dexamphetamine to patients with autism spectrum disorders ( ASD ) because of both doubts about efficacy and concern that these medications make stereotypies worse . Recently , a number of small trials have suggested that methypheni date does have a role in the management of hyperactivity in children with autistic spectrum disorders . METHODS Children with ASD and attention deficit hyperactivity disorder ( ADHD ) , and children with ADHD without ASD received st and ard treatment with methypheni date from one specialist centre . A combination of st and ardized and novel outcome tools was used to allow both an exploratory retrospective study of 174 children and then a prospect i ve study of a further 52 children to be carried out . RESULTS After treatment with stimulants , the subjects in both groups showed statistically significant improvements in target symptoms of ' hyperactivity ' , ' impulsivity ' , ' inattention ' , ' oppositionality ' , ' aggression ' and ' intermittent explosive rage ' . The Clinical Global Impression-Improvement and efficacy index measures also improved in each group . In both the retrospective and the prospect i ve studies , there was no statistically significant difference in the degree of improvements between each group . Importantly , neither tics nor repetitive behaviours worsened in either group . Children in the ' ADHD-only ' group who were prescribed stimulants experienced significant ' nausea ' , ' giddiness ' , ' headaches ' and ' sleep difficulties ' , whereas sleep difficulties were the only side effect that emerged in children in the ASD with ADHD group . CONCLUSIONS Both studies presented here support previous findings from smaller studies that show children with autism and ADHD can respond as well to stimulants as children with ADHD alone . Although r and omized controlled trials remain the gold st and ard for efficacy studies , systems like this that allow clinicians to continue rigorous and consistent monitoring for many years have a valuable role to play . Furthermore , such monitoring systems which now exist electronically can easily accumulate large data sets and reveal details about long-term effectiveness and long-term side effects of medication that are unlikely to be discovered in short-term trials BACKGROUND The objective was to investigate the frequency of fetal alcohol spectrum disorders ( FASD ) and ophthalmologic anomalies in orphanage children in Brazil . METHODS A prospect i ve study was performed on 94 children living in an orphanage in Brazil . The children were examined by a multidisciplinary team consisting of specialists in pediatrics , neurology , psychology , neuropsychiatry , and ophthalmology . RESULTS The main reasons for living in the orphanage , in 61 % of the children , were negligence , child abuse , and ab and onment . Of all the children studied , 50 % had mothers with known alcohol abuse and 47 % had one or more diagnoses of neurodevelopmental/behavioral and /or cognitive deficits . General developmental delay was found in 18 % , intellectual disability in 3 % , cognitive impairment in 27 % , attention-deficit/hyperactivity disorder in 14 % , and autism in 3 % . Altogether 17 % had FASD , comprising three children with fetal alcohol syndrome ( FAS ) , six with partial FAS , and seven with alcohol-related neurodevelopmental disorder . 16 % had ophthalmological findings such as poor vision , strabismus , and dysmorphology of the optic nerves . Twenty-eight children ( 30 % ) were adopted from the orphanage ; of these , six had FASD ( two FAS , three partial FAS , one alcohol-related neurodevelopmental disorder ) , five had attention-deficit/hyperactivity disorder , and eight had developmental delay . CONCLUSION Nearly half of the children living in the orphanage had neurodevelopmental disorders and a considerable number showed signs of damage from prenatal alcohol exposure . A broader look at the problem of FASD in Brazil and other South American countries is desirable to document the burden of disease and provide data for targeting prevention efforts BACKGROUND Many people with intellectual disabilities find it hard to control their anger and this often leads to aggression which can have serious consequences , such as exclusion from mainstream services and the need for potentially more expensive emergency placements . AIMS To evaluate the effectiveness of a cognitive-behavioural therapy ( CBT ) intervention for anger management in people with intellectual disabilities . METHOD A cluster-r and omised trial of group-based 12-week CBT , which took place in day services for people with intellectual disabilities and was delivered by care staff using a treatment manual . Participants were 179 service users identified as having problems with anger control r and omly assigned to either anger management or treatment as usual . Assessment s were conducted before the intervention , and at 16 weeks and 10 months after r and omisation ( trial registration : IS RCT N37509773 ) . RESULTS The intervention had only a small , and non-significant , effect on participants ' reports of anger on the Provocation Index , the primary outcome measure ( mean difference 2.8 , 95 % CI -1.7 to 7.4 at 10 months ) . However , keyworker Provocation Index ratings were significantly lower in both follow-up assessment s , as were service-user ratings on another self-report anger measure based on personally salient triggers . Both service users and their keyworkers reported greater usage of anger coping skills at both follow-up assessment s and keyworkers and home carers reported lower levels of challenging behaviour . CONCLUSIONS The intervention was effective in improving anger control by people with intellectual disabilities . It provides evidence of the effectiveness of a CBT intervention for this client group and demonstrates that the staff who work with them can be trained and supervised to deliver such an intervention with reasonable fidelity BACKGROUND Self-injurious behaviour ( SIB ) is a serious condition , with implication s for the person , their family and financial costs to the state providing care . The previously reported prevalence of SIB has ranged from 1.7 % to 41 % , or 1.7%-23.7 % in community studies . There has been little study of remission rate , and incidence has not previously been reported . SIB has been reported to be individually associated with lower ability , autism and communication impairments , but given the inter-relationships between these three factors , it is not known whether they are independently associated with SIB . This study investigates the point prevalence , incidence and remission rates of SIB among the adult population with intellectual disabilities ( ID ) , and explores which factors are independently associated with SIB . METHOD A prospect i ve cohort study design was used in a general community setting . The participants were all adults ( 16 years and over ) with ID in a defined geographical area .  Individual assessment s were conducted with all participants . RESULTS The point prevalence of SIB ( as defined by DC-LD ) was 4.9 % , the two-year incidence was 0.6 % , and two-year remission rate was 38.2 % . Independently related to SIB were : lower ability level , not living with a family carer , having attention deficit hyperactivity disorder , visual impairment , and not having Down syndrome . Other factors , including communication impairment , autism , and level of deprivation of the area resided within , were not related . CONCLUSIONS SIB is not as enduring and persistent as previously thought ; a significant proportion gains remission in this time period . This should provide hope for families , paid carers and professionals , and reduce therapeutic nihilism . Our study is a first tentative step towards identifying risk-markers for SIB , and developing aetiological hypotheses for subsequent testing . The extent to which SIB may be a relapsing-remitting ( episodic ) condition requires further investigation , so does further hypothesis-based investigation of factors that might be predictive of incidence of , and remission from , SIB BACKGROUND People with intellectual disabilities ( ID ) experience similar or even higher rates of mental health problems than the general population and there is a need to develop appropriate treatments . Cognitive behaviour therapy ( CBT ) is effective for a wide range of disorders in the general population . However , there is some evidence that people with ID may lack the cognitive skills needed to take part in CBT . AIMS To test if people with ID can learn skills required for CBT , specifically the ability to distinguish between thoughts , feelings , and behaviours and to link thoughts and feelings ( cognitive mediation ) . METHOD A r and omized independent groups design was used to examine the effect of training in CBT on two tasks measuring  CBT skills .  Thirty-four adults with ID were r and omly allocated to the experimental condition ( N=18 ) or to the control condition ( N=16 ) .  CBT skills were assessed blind at baseline and after the intervention . RESULTS The training led to significant improvements in participants ' ability to link thoughts and feelings , and this skill was generalized to new material . There was no effect of training on participants ' ability to distinguish amongst thoughts , feelings , and behaviours .  People with ID can , therefore , learn some skills required for CBT . This implies that preparatory training for CBT might be useful for people with ID . The results might be applicable to other groups who find aspects of CBT difficult Seventy-eight children , referred to a diet clinic because of hyperactive behaviour , were placed on a ' few foods ' elimination diet . Fifty nine improved in behaviour during this open trial . For 19 of these children it was possible to disguise foods or additives , or both , that reliably provoked behavioural problems by mixing them with other tolerated foods and to test their effect in a placebo controlled double blind challenge protocol . The results of a crossover trial on these 19 children showed a significant effect for the provoking foods to worsen ratings of behaviour and to impair psychological test performance . This study shows that observations of change in behaviour associated with diet made by parents and other people with a role in the child 's care can be reproduced using double blind methodology and objective assessment s. Clinicians should give weight to the accounts of parents and consider this treatment in selected children with a suggestive medical history Rationale Fragile X syndrome ( FXS ) is the most common inherited form of developmental disability and most common single gene cause of autism . Persons with FXS frequently exhibit irritable behavior marked by aggression , self-injury , and severe tantrums . Despite frequent clinical use of atypical antipsychotic drugs to target this behavioral cluster , no systematic trials to date have assessed the efficacy and safety of these drugs in persons with FXS . Methods We conducted a prospect i ve open-label 12-week trial of aripiprazole in 12 persons aged 6–25 years ( mean age , 14.3 years ) with FXS who were free of concomitant psychoactive drugs .  Results Aripiprazole use ( mean dose , 9.8 mg/day ) was associated with treatment response ( defined by a Clinical Global Impressions-Improvement scale score of much improved or very much improved and a ≥25 % improvement on the Aberrant Behavior Checklist-Irritability subscale ) in 10 of 12 ( 87 % ) persons . Two individuals ( 13 % ) discontinued aripiprazole prior to study completion due to adverse events . One discontinuation was due to akathisia , mild drooling , and mild tiredness and the other due to moderate tiredness and moderate drooling . No significant changes in vital signs including weight or laboratory measures occurred during treatment with aripiprazole . Conclusions Aripiprazole was generally safe and well tolerated and was associated with significant improvement in irritable behavior . Given these findings , a double-blind , placebo-controlled study of aripiprazole in FXS is warranted Background Two-thirds of people living with dementia live at home in the UK and many experience distressing behavioural and psychological symptoms . This systematic review evaluates the effectiveness of non-pharmacological interventions for behavioural and psychological symptoms among community-dwelling people living with dementia . Methods This two-stage review undertook an initial mapping of the literature followed by a systematic review of relevant r and omised controlled trials . We search ed electronic data bases for pertinent studies reporting outcomes from interventions from January 2000 to March 2015 and up date d search es in October 2016 . We included studies that considered behavioural and psychological symptom management for older people living with dementia who live at home and excluded studies conducted in long-term care setting s. This paper presents findings from a narrative synthesis of 48 r and omised controlled trials evaluating interventions for people living with dementia alone , family carers alone and patient-carer dyads . Results We retrieved 17,871 de-duplicated records and screened them for potential inclusion . Evidence from 48 r and omised controlled trials suggests that family carer training and educational programmes that target problem behaviours and potential triggers can improve outcomes . Nurses and occupational therapists appear to help people with dementia with behavioural and psychological symptoms , but professional comparisons are lacking and there is no shared language about or underst and ing of behavioural and psychological symptoms amongst professionals , or between professionals and family carers . Conclusions Future research should focus on the effectiveness of components of multi-faceted programmes and their cost effectiveness and include qualitative data to better target interventions for behavioural and psychological symptoms . It is important to consider family carer readiness to use non-pharmacological strategies and to develop a shared language about the inherent needs and communications of behavioural and psychological symptoms BACKGROUND The clinical and neuropathological features associated with dementia in Down 's syndrome ( DS ) are not well established . Aims To examine clinico-pathological correlations and the incidence of cognitive decline in a cohort of adults with DS . METHOD A total of 92 hospitalized persons with DS were followed up from 1985 to December 2000 . At outset , 87 participants were dementia-free , with a median age of 38 years . Assessment s included the  Prudhoe Cognitive Function Test ( PCFT )  and the  Adaptive Behavior Scale ( ABS )  , to measure cognitive and behavioural deterioration . Dementia was diagnosed from case records and caregivers ' reports . RESULTS Eighteen ( 21 % ) patients developed dementia during follow-up , with a median age of onset 55.5 years ( range 45 - 74 ) . The PCFT demonstrated cognitive decline among those with a less severe intellectual disability ( mild and moderate ) but not among the profoundly disabled people ( severe and profound ) . Clinical dementia was associated with neuropathological features of Alzheimer 's disease , and correlated with neocortical neurofibrillary tangle densities . At the age of 60 years and above , a little more than 50 % of patients still alive had clinical evidence of dementia . CONCLUSIONS Clinical dementia associated with measurable cognitive and functional decline is frequent in people with DS after middle age , and can be readily diagnosed among less severely intellectually disabled persons using measures of cognitive function such as the  PCFT  and behavioural scales such as the ABS . In the more profoundly disabled people , the diagnosis of dementia is facilitated by the use of behavioural and neurological criteria . In this study , the largest prospect i ve DS series including neuropathology on deceased patients , the density of neurofibrillary tangles related more closely to the dementia of DS than senile plaques . In people with DS surviving to middle and old age , the development of dementia of Alzheimer type is frequent but not inevitable , and some people with DS reach old age without clinical features of dementia This study presents the outcomes of mediator analyses as part of a r and omized controlled trial of Cognitive-Behavioural Therapy ( CBT ) for young people who engage in deliberate self-harm ( DSH ) . The study involved 90 people , aged 15 - 35 years , who were r and omly assigned to CBT in addition to treatment as usual or to treatment as usual only .  The findings showed that changes in DSH were partially mediated by changes in emotion-regulation difficulties , particularly difficulties with impulse control and goal -directed behaviours . In addition , the potential mediating role of symptoms of depression , anxiety and suicidal cognitions was examined . Although the CBT intervention significantly reduced depression , anxiety and suicidal cognitions , these measures of symptom severity did not play a mediating role . These findings suggest that interventions for DSH should not primarily focus on mental disorders associated with DSH , but should be DSH-specific and should target specific emotion-regulation difficulties
Regarding the primary outcome of psychological and learning capabilities , both studies assessed the effect of interventions on  children 's verbal and non-verbal intellectual functioning using the Wechsler Intelligence Scale for Children - Revised .
AUTHORS ' CONCLUSIONS Low- quality evidence from two small trials showed that when compared to placebo , LAC may not improve intellectual functioning or hyperactive behaviour in children with FXS
BACKGROUND People with fragile X syndrome ( FXS ) have an intellectual dysfunction that can range from very mild to severe .
Symptoms can include speech and language delays and behavioural difficulties such as aggression or self injurious behaviours , emotional lability , and anxiety-related problems ( for example obsessive-compulsive symptoms and perseverative behaviours ) .
In some cases , affected people may have an additional diagnosis of attention deficit hyperactivity disorder or an autism spectrum disorder .
OBJECTIVES To review the efficacy and safety of L-acetylcarnitine in improving the psychological , intellectual , and social performance of people with FXS .