Expanding the clinical spectrum of Fowler syndrome: Three siblings with survival into adulthood and systematic review of the literature.

Proliferative vasculopathy and hydranencephaly-hydrocephaly syndrome (PVHH, OMIM 225790), also known as Fowler syndrome, is a rare autosomal recessive disorder of brain angiogenesis. PVHH has long been considered to be prenatally lethal. We evaluated the phenotypes of the first three siblings with survival into adulthood, performed a systematic review of the Fowler syndrome literature and delineated genotype-phenotype correlations using a scoring system to rate the severity of the disease. Thirty articles were included, describing 69 individual patients. To date, including our clinical reports, 72 patients have been described with Fowler syndrome. Only 6/72 (8%) survived beyond birth. Although our three patients carry the same mutations (c.327T>A-p.Asn109Lys and c.887C>T-p.Ser296Leu) in FLVCR2, only two of them presented with the same cerebral features, ventriculomegaly and cerebral calcifications, as affected fetuses. The third sibling has a surprisingly milder clinical and radiological phenotype, suggesting intrafamilial variability. Although no clear phenotype-genotype correlation exists, some variants appear to be associated with a less severe phenotype compatible with life. As such, it is important to consider Fowler syndrome in patients with gross ventriculomegaly, cortical malformations and/or cerebral calcifications on brain imaging.

Maternal exposure to violence and offspring neurodevelopment: A systematic review.

BACKGROUND: Stress during pregnancy is known to affect fetal neurodevelopment. It seems likely therefore that intimate partner violence (IPV) and domestic violence (DV) as extreme stressors will have a similarly adverse effect. OBJECTIVES: A systematic review was conducted to assess the association between prenatal exposure to violence for mothers and developmental difficulties in their children. DATA SOURCES: PubMed, PsycInfo, CINAHL, ERIC, Science Direct, SCOPUS, PsyARTICLES, Networked Digital Library of Theses and Dissertations, Women's Studies International and Gender Studies Database were all searched using the agreed search terms. STUDY SELECTION AND DATA EXTRACTION: We include studies of women who have experienced any violence, fear of violence or aggression while pregnant, including emotional, psychological, physical or sexual violence in the context of IPV or DV. Studies were excluded if the neurodevelopmental outcomes of the offspring were not assessed. Studies from all countries were included, in English or translated to English, and search dates were not restricted. We included all years from inception of the database until the search date. SYNTHESIS: Study design and biases, assessment tools, management of confounding, results and overall quality were assessed. RESULTS: We identified 11 papers reporting on observational studies. Almost three quarters of the studies found a relationship between prenatal exposure to violence and developmental difficulties in the offspring. Differing assessment tools were used with a range of data collected and not all adjusted their findings for the same confounders. CONCLUSIONS: Current evidence on the relationship between prenatal violence exposure, as IPV or DV, and consequent child developmental disorders remains limited. Future research using comprehensive study designs, larger samples and longitudinal follow-up of the offspring could clarify this association. While maternal trauma resulting from exposure to violence may play an important role in childhood development disorders, additional intervening factors on the pathway need further explored.

Factors associated with public knowledge of and attitudes to dementia: A cross-sectional study.

INTRODUCTION: Dementia is a major public health concern but one that continues to be stigmatised. We examine lay knowledge of dementia and attitudes to people with dementia as potential precursors of public anxiety, focusing on the social characteristics associated with (a) the formation of these attitudes, and (b) the perception of the need for restriction and control for people with dementia. METHODS: Analysis of the 2014 Northern Ireland Life and Times survey, which included questions on knowledge of, attitudes to and personal experience with dementia. We used (a) latent class analysis and (b) logistic regression to examine factors associated with respondent attitudes towards dementia. RESULTS: Respondents (n = 1211) had relatively good general knowledge of dementia, but limited knowledge of specific risk factors. Negative perceptions of dementia were mitigated somewhat by personal contact. A high proportion of respondents felt that high levels of control were appropriate for people diagnosed with dementia, even at early stages of the disease. CONCLUSION: Personal antipathy to dementia was highly prevalent despite ongoing public campaigns to increase public awareness of developments in its prevention, treatment and consequent care pathways and hampering efforts to widen social inclusion. Fresh thinking and more resources may be needed to challenge persisting common misapprehension of the condition and the formation of entrenched stigma.

Personality Traits Predict Meeting the WHO Recommendation of 6 Months' Breastfeeding: A Prospective General Population Cohort Study.

BACKGROUND: Although personality as well as anxiety and depression are recognized as predictors for breastfeeding initiation, evidence of an association of these factors with 6 months' exclusive breastfeeding as recommended by the World Health Organization (WHO) is sparse. PURPOSE: The purpose of this study was to investigate the associations of personality and symptoms of anxiety and depression during and after pregnancy with meeting the WHO recommendation of 6 months' exclusive breastfeeding. METHODS: In their first trimester of pregnancy, 5784 pregnant women were enrolled in Dutch primary obstetric care centers and hospitals, of which 2927 completed the breastfeeding assessments 6 months postpartum. We performed logistic regression analyses to test the associations of "big five" personality traits (NEO Five Factor Inventory), anxiety (State-Trait Anxiety Inventory), and depression (Edinburgh Postnatal Depression Scale) symptom levels during pregnancy and postpartum with meeting the WHO recommendation of 6 months' exclusive breastfeeding. RESULTS: Agreeableness (odds ratio [OR] = 1.18, P = .006) and openness (OR = 1.31, P < .001) were positively associated with meeting the WHO recommendation, whereas extraversion (OR = 0.83, P = .005) and neuroticism (OR = 1.18, P = .006) were negatively associated. After adjustment for both antenatal and postpartum symptom levels of anxiety and depression, the associations of the agreeableness, extraversion, and openness personality traits remained strong and statistically significant (P < .05). IMPLICATIONS FOR PRACTICE: Patient-centered care should take personality into account in an effort to tailor interventions to optimize breastfeeding behavior. IMPLICATIONS FOR RESEARCH: In contrast to earlier findings, personality traits may be of greater importance than symptoms of anxiety and depression for meeting the WHO recommendation of 6 months' exclusive breastfeeding.

Profiles of childhood trauma and psychopathology: US National Epidemiologic Survey.

PURPOSE: Childhood trauma may increase vulnerability to numerous specific psychiatric disorders, or a generalised liability to experience dimensions of internalising or externalising psychopathology. We use a nationally representative sample (N = 34,653) to examine the long-term consequences of childhood trauma and their combined effect as predictors of subsequent psychopathology. METHODS: Data from the US National Epidemiologic Survey on Alcohol and Related Conditions were used. Latent class analysis was used to identify childhood trauma profiles and multinomial logistic regression to validate and explore these profiles with a range of associated demographic and household characteristics. We used Structural Equation Modelling to substantiate initial latent class analysis findings by investigating a range of mental health diagnoses. Internalising and externalising domains of psychopathology were regressed on trauma profiles and associated demographic and household characteristics. We used Differential Item Functioning to examine associations between the trauma groups and a number of psychiatric disorders within internalising and externalising dimensions of mental health. RESULTS: We found a 3-class model of childhood trauma in which 85% of participants were allocated to a low trauma class; 6% to a multi-type victimization class (reporting exposures for all the child maltreatment criteria); and 9% to a situational trauma class (exposed to a range of traumas). Confirmatory Factor Analysis revealed an internalising-externalising spectrum was used to represent lifetime reporting patterns of mental health disorders. Both trauma groups showed specific gender and race/ethnicity differences, related family discord and increased psychopathology. Additionally, we found significant associations between the trauma groups and specific diagnoses within the internalising-externalising spectrum of mental health. CONCLUSIONS: The underlying patterns in the exposure to types of interpersonal and non-interpersonal traumas and associated mental health highlight the need to screen for particular types of childhood traumas when individuals present with symptoms of psychiatric disorders.

Pregnancy-specific anxiety and its association with background characteristics and health-related behaviors in a low-risk population.

BACKGROUND: Pregnancy-specific anxiety is an important risk factor for adverse pregnancy outcomes. It is therefore needed to gain insight in which women are at risk for elevated levels (> 85th percentile) of pregnancy-specific anxiety. Additionally, given that unhealthy behaviour has been suggested as a possible pathway linking pregnancy-specific anxiety to adverse pregnancy outcomes, it is important to examine whether higher levels of pregnancy-specific anxiety are associated with negative health-related behaviours (smoking, alcohol use and too much weight gain). METHODS: Using a study sample of 4541 low-risk pregnant women who filled in the Pregnancy Related Anxiety Questionnaire-Revised (PRAQ-R), we first examined which socio-demographic, pregnancy-related and psychological background characteristics were significantly associated with a PRAQ-R score above the 85th percentile. Secondly, we examined the association between pregnancy-specific anxiety and self-reported health-related behaviours (smoking, alcohol use and too much weight gain) while controlling for significant background characteristics. For both research questions, backward regression analysis was applied. RESULTS: Results showed that nulliparity (OR=2.33, 95% confidence interval (CI)=1.97-2.77), anxious or depressed mood (OR=3.29, 95% CI=2.74-3.94) and non-Dutch ethnicity, especially Turkish (OR=3.47, 95% CI=2.16-5.59) or Moroccan (OR=2.97, 95% CI=1.84-4.81), were most strongly associated with elevated pregnancy-specific anxiety levels. Women with higher pregnancy-specific anxiety levels were more likely to gain too much weight during pregnancy (odds ratio (OR) linear term=1.49, 95% CI=1.21-1.83), while both very low and high levels of pregnancy-specific anxiety were associated with smoking (OR linear term=0.13, 95% CI=0.04-0.45, OR quadratic term=1.81, 95% CI=1.32-2.47). No association with alcohol use was found. CONCLUSIONS: In conclusion, our results show nulliparity, anxious or depressed mood and non-Dutch ethnicity as three major vulnerability factors for elevated levels of pregnancy-specific anxiety. Furthermore, our results show an association between pregnancy-specific anxiety and negative health-related behaviours, which is worth examining in future studies.

The relationship of dystonia and choreoathetosis with activity, participation and quality of life in children and youth with dyskinetic cerebral palsy.

AIM: To relate dystonia and choreoathetosis with activity, participation and quality of life (QOL) in children and youth with dyskinetic Cerebral Palsy (CP). METHODS: Fifty-four participants with dyskinetic CP (mean age 14y6m, SD 4y2m, range 6-22y) were included. The Dyskinesia Impairment Scale (DIS) was used to evaluate dystonia and choreoathetosis. Activity, participation and quality of life (QOL) were assessed with the Gross Motor Function Measure (GMFM), the Functional Mobility Scale (FMS), the Jebsen-Taylor Hand Function Test (JTT), the ABILHAND-Kids Questionnaire (ABIL-K), the Life Habits Kids (LIFE-H) and the Quality of Life Questionnaire for children with CP (CP-QOL). Spearman's rank correlation coefficient (rs) was used to assess the relationship between the movement disorders and activity, participation and QOL measures. RESULTS: Significant negative correlations were found between dystonia and the activity scales with Spearman's rank correlation coefficient (rs) varying between -0.65 (95% CI = -0.78 to -0.46) and -0.71 (95% CI = -0,82 to -0.55). Correlations were also found with the LIFE-H (rs = -0.43; 95%CI = -0.64 to -0.17) and the CP-QOL (rs = -0.32; 95%CI = -0.56 to -0.03). As far as choreoathetosis is concerned, no or only weak relationships were found with the activity, participation and quality of life scales. INTERPRETATION: This cross-sectional study is the first to examine the relationship of dystonia and choreoathetosis in dyskinetic CP with the level of activity, participation and QOL. The results revealed dystonia has a higher impact on activity, participation and quality of life than choreoathetosis. These findings seem to suggest it is necessary to first focus on dystonia reducing intervention strategies and secondly on choreoathetosis.

Macrostructural and Microstructural Brain Lesions Relate to Gait Pathology in Children With Cerebral Palsy.

Background Even though lower-limb motor disorders are core features of spastic cerebral palsy (sCP), the relationship with brain lesions remains unclear. Unraveling the relation between gait pathology, lower-limb function, and brain lesions in sCP is complex for several reasons; wide heterogeneity in brain lesions, ongoing brain maturation, and gait depends on a number of primary motor functions/deficits (eg, muscle strength, spasticity). Objective To use a comprehensive approach combining conventional MRI and diffusion tensor imaging (DTI) in children with sCP above 3 years old to relate quantitative parameters of brain lesions in multiple brain areas to gait performance. Methods A total of 50 children with sCP (25 bilateral, 25 unilateral involvement) were enrolled. The investigated neuroradiological parameters included the following: (1) volumetric measures of the corpus callosum (CC) and lateral ventricles (LVs), and (2) DTI parameters of the corticospinal tract (CST). Gait pathology and primary motor deficits, including muscle strength and spasticity, were evaluated by 3D gait analysis and clinical examination. Results In bilateral sCP (n = 25), volume of the LV and the subparts of the CC connecting frontal, (pre)motor, and sensory areas were most related to lower-limb functioning and gait pathology. DTI measures of the CST revealed additional relations with the primary motor deficits (n = 13). In contrast, in unilateral sCP, volumetric (n = 25) and diffusion measures (n = 14) were only correlated to lower-limb strength. Conclusions These results indicate that the combined influence of multiple brain lesions and their impact on the primary motor deficits might explain a large part of the gait pathology in sCP.

Children with Spastic Cerebral Palsy Experience Difficulties Adjusting Their Gait Pattern to Weight Added to the Waist, While Typically Developing Children Do Not.

The prevalence of childhood overweight and obesity is increasing in the last decades, also in children with Cerebral Palsy (CP). Even though it has been established that an increase in weight can have important negative effects on gait in healthy adults and children, it has not been investigated what the effect is of an increase in body weight on the characteristics of gait in children with CP. In CP, pre and post three-dimensional gait analyses are performed to assess the effectiveness of an intervention. As a considerable amount of time can elapse between these measurements, and the effect of an alteration in the body weight is not taken into consideration, this effect of increased body weight is of specific importance. Thirty children with the predominantly spastic type of CP and 15 typically developing (TD) children were enrolled (age 3-15 years). All children underwent three-dimensional gait analysis with weight-free (baseline) and weighted (10% of the body weight added around their waist) trials. Numerous gait parameters showed a different response to the added weight for TD and CP children. TD children increased walking velocity, step- and stride length, and decreased double support duration with a slightly earlier timing of foot-off, while the opposite was found in CP. Similarly, increased ranges of motion at the pelvis (coronal plane) and hip (all planes), higher joint angular velocities at the hip and ankle, as well as increased moments and powers at the hip, knee and ankle were observed for TD children, while CP children did not change or even showed decreases in the respective measures in response to walking with added weight. Further, while TD children increased their gastrocnemius EMG amplitude during weighted walking, CP children slightly decreased their gastrocnemius EMG amplitude. As such, an increase in weight has a significant effect on the gait pattern in CP children. Clinical gait analysts should therefore take into account the negative effects of increased weight during pre-post measurements to avoid misinterpretation of treatment results. Overweight and obesity in CP should be counteracted or prevented as the increased weight has detrimental effects on the gait pattern.

Clinical patterns of dystonia and choreoathetosis in participants with dyskinetic cerebral palsy.

AIM: The aim of the study was to map clinical patterns of dystonia and choreoathetosis and to assess the relation between functional classifications and basal ganglia and thalamus lesions in participants with dyskinetic cerebral palsy (CP). METHODS: In this cross-sectional study, 55 participants with dyskinetic CP (mean age 14y 6mo, SD 4y 1mo; range 6-22y) were assessed with the Dyskinesia Impairment Scale and classified with the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), and Communication Function Classification System (CFCS). RESULTS: Dystonia and choreoathetosis are simultaneously present. Median levels of dystonia (70.2%) were significantly higher than levels of choreoathetosis (26.7%) and both were significantly higher during activity than at rest (both p<0.01). High correlations were found between dystonia levels and GMFCS level (Spearman's rank correlation coefficient, rS =0.70; 95% confidence interval [CI] 0.53-0.81; p<0.01) and MACS (rS =0.65; 95% CI 0.47-0.81; p<0.01), and fair correlation with CFCS (rs =0.36; 95% CI=0.11-0.57; p<0.05). No significant correlation was found between choreoathetosis levels and motor classifications. Finally, higher choreoathetosis levels were found in participants with pure thalamus and basal ganglia lesions (p=0.03) than mixed lesions, but not for dystonia (p=0.41). INTERPRETATION: Dystonia and choreoathetosis increase during activity. However, dystonia predominates and seems to have a larger impact on functional abilities. Our findings further suggest that choreoathetosis seems to be more linked to pure thalamus and basal ganglia lesions than dystonia.

Factorial invariance of pregnancy-specific anxiety dimensions across nulliparous and parous pregnant women.

The 10-item version of the Pregnancy-Related Anxiety Questionnaire-Revised was developed based on a sample of nulliparous women. Whether this measure is also a valuable tool for future research on pregnancy-specific anxiety is unclear. Our study tested for invariance of this measure across nulliparous women and parous women by using a dataset of 6004 women pregnant up to 35 weeks. Results showed that whereas the factor structure of the 10-item version of the Pregnancy-Related Anxiety Questionnaire-Revised was noninvariant, removing item 8 from the measure created a measure with invariant factor loadings that can be used for all pregnant women.

Altered functional connectivity of the language network in ASD: role of classical language areas and cerebellum.

The development of language, social interaction and communicative skills is remarkably different in the child with autism spectrum disorder (ASD). Atypical brain connectivity has frequently been reported in this patient population. However, the neural correlates underlying their disrupted language development and functioning are still poorly understood. Using resting state fMRI, we investigated the functional connectivity properties of the language network in a group of ASD patients with clear comorbid language impairment (ASD-LI; N = 19) and compared them to the language related connectivity properties of 23 age-matched typically developing children. A verb generation task was used to determine language components commonly active in both groups. Eight joint language components were identified and subsequently used as seeds in a resting state analysis. Interestingly, both the interregional and the seed-based whole brain connectivity analysis showed preserved connectivity between the classical intrahemispheric language centers, Wernicke's and Broca's areas. In contrast however, a marked loss of functional connectivity was found between the right cerebellar region and the supratentorial regulatory language areas. Also, the connectivity between the interhemispheric Broca regions and modulatory control dorsolateral prefrontal region was found to be decreased. This disruption of normal modulatory control and automation function by the cerebellum may underlie the abnormal language function in children with ASD-LI.

Randomized trial of modified constraint-induced movement therapy with and without an intensive therapy program in children with unilateral cerebral palsy.

BACKGROUND: Constraint-induced movement therapy (CIMT) has gained emerging evidence and popularity in children with unilateral cerebral palsy (CP). However, many issues remain unanswered regarding the best approach. OBJECTIVE: This study investigated the additional effects of an intensive therapy program to promote hand function combined with home-based modified CIMT (m-CIMT). METHODS: . Fifty-one children (mean age 8 years 9 months) were randomized to m-CIMT alone or m-CIMT with intensive therapy (IT). All children had to wear a constraint on the unaffected hand for 1 hour, 5 days/week for 10 weeks. Children in the m-CIMT + IT group also received 3 sessions of 45 minutes weekly of intensive therapy for distal muscle strengthening and hand function, using unimanual and bimanual activities. The Assisting Hand Assessment (AHA) was the primary outcome measure. Secondary outcome measures were muscle tone, strength, Melbourne Assessment, Jebsen-Taylor test, and ABILHAND-Kids questionnaire. Assessments were administered at baseline, after intervention, and at 10-week follow-up. RESULTS: Significant between-group differences in AHA were in favor of the m-CIMT + IT group (P = .04). Both groups demonstrated comparable improvements in muscle tone (P = .002), strength (P < .0001), grip strength (P = .02), and unimanual capacity (Melbourne Assessment and Jebsen-Taylor, P < .0001). Younger children and children with poorer hand function benefited from both interventions, whereas older children and children with better hand function only benefited from the combined approach. CONCLUSIONS: The combination of m-CIMT with an intensive therapy program on distal hand function and strength enhances the effects of m-CIMT alone for improving bimanual performance.

Does somatosensory discrimination activate different brain areas in children with unilateral cerebral palsy compared to typically developing children? An fMRI study.

Aside from motor impairment, many children with unilateral cerebral palsy (CP) experience altered tactile, proprioceptive, and kinesthetic awareness. Sensory deficits are addressed in rehabilitation programs, which include somatosensory discrimination exercises. In contrast to adult stroke patients, data on brain activation, occurring during somatosensory discrimination exercises, are lacking in CP children. Therefore, this study investigated brain activation with functional magnetic resonance imaging (fMRI) during passively guided somatosensory discrimination exercises in 18 typically developing children (TD) (age, M=14 ± 1.92 years; 11 girls) and 16 CP children (age, M=15 ± 2.54 years; 8 girls). The demographic variables between both groups were not statistically different. An fMRI compatible robot guided the right index finger and performed pairs of unfamiliar geometric shapes in the air, which were judged on their equality. The control condition comprised discrimination of music fragments. Both groups exhibited significant activation (FDR, p<.05) in frontoparietal, temporal, cerebellar areas, and insula, similar to studies in adults. The frontal areas encompassed ventral premotor areas, left postcentral gyrus, and precentral gyrus; additional supplementary motor area (SMA proper) activation in TD; as well as dorsal premotor, and parietal operculum recruitment in CP. On uncorrected level, p<.001, TD children revealed more left frontal lobe, and right cerebellum activation, compared to CP children. Conversely, CP children activated the left dorsal cingulate gyrus to a greater extent than TD children. These data provide incentives to investigate the effect of somatosensory discrimination during rehabilitation in CP, on clinical outcome and brain plasticity.

The role of attention in the affective life of people with severe or profound intellectual disabilities.

Although it is shown that attention plays an important role both in the onset and in the regulation of emotions in people without disabilities there is no information about how attention is related to emotions in people with severe or profound intellectual disability (ID). Therefore, in our study, we investigated the role of attention in the onset and regulation of the emotions of persons with severe or profound ID. We presented 27 participants with 4 staff-selected negative and 4 staff-selected positive stimuli. The situations were videotaped and their heart rate and attention was measured. Contrary to the expected higher attention to negative stimuli during the onset of negative emotions, we did not find differences in attention in the fourth to sixth second of stimulus presentation. However, in support of the emotion regulation theory of Gross (2008) we did find less attention to the negative stimuli than to the positive stimuli after these first 6s of stimuli presentation. As expected from research in people without disabilities, there was also a negative relationship between the heart rate and the probability of being attentive. Our results suggest that people with severe and profound ID use attentional deployment to regulate their emotions and that, as in people without disabilities, a low heart rate is associated with attention.

How does brain activation differ in children with unilateral cerebral palsy compared to typically developing children, during active and passive movements, and tactile stimulation? An fMRI study.

The aim of the functional magnetic resonance imaging (fMRI) study was to investigate brain activation associated with active and passive movements, and tactile stimulation in 17 children with right-sided unilateral cerebral palsy (CP), compared to 19 typically developing children (TD). The active movements consisted of repetitive opening and closing of the hand. For passive movements, an MRI-compatible robot moved the finger up and down. Tactile stimulation was provided by manually stroking the dorsal surface of the hand with a sponge cotton cloth. In both groups, contralateral primary sensorimotor cortex activation (SM1) was seen for all tasks, as well as additional contralateral primary somatosensory cortex (S1) activation for passive movements. Ipsilateral cerebellar activity was observed in TD children during all tasks, but only during active movements in CP children. Of interest was additional ipsilateral SM1 recruitment in CP during active movements as well as ipsilateral S1 activation during passive movements and tactile stimulation. Another interesting new finding was the contralateral cerebellum activation in both groups during different tasks, also in cerebellar areas not primarily linked to the sensorimotor network. Active movements elicited significantly more brain activation in CP compared to TD children. In both groups, active movements displayed significantly more brain activation compared to passive movements and tactile stimulation.

Can the Dyskinesia Impairment Scale be used by inexperienced raters? A reliability study.

BACKGROUND: The Dyskinesia Impairment Scale (DIS) is a new scale for measuring dystonia and choreoathetosis in dyskinetic Cerebral Palsy (CP). Previously, reliability of this scale has only been assessed for raters highly experienced in discriminating between dystonia and choreoathetosis. AIMS: The aims of this study are to examine the reliability of the DIS used by inexperienced raters, new to discriminating between dystonia and choreoathetosis and to determine the effect of clinical expertise on reliability. METHODS: Twenty-five patients (17 males; 8 females; age range 5-22 years; mean age = 13 years 6 months; SD = 5 years 4 months) with dyskinetic CP were filmed with the DIS standard video protocol. Two junior physiotherapists (PTs) and three senior PTs, all of whom were new to discriminating between dystonia and choreoathetosis, were trained in scoring the DIS. Afterward, they independently scored all patients from the video recordings using the DIS. Reliability was assessed by (1) Intraclass Correlation Coefficient (ICC), (2) Standard Error of Measurement (SEM) and Minimal Detectable Difference (MDD) and (3) Cronbach's alpha for internal consistency. RESULTS: Interrater reliability for the total DIS, and for the dystonia and choreoathetosis subscales was good for the junior PTs and moderately high to excellent for the senior PTs. SEM and MDD values for the total DIS were 6% and 15% respectively for the junior PTs and 4% and 12% respectively for the senior PTs. Cronbach's alpha ranged between 0.87 and 0.95 for the junior PTs and between 0.76 and 0.93 for the senior PTs. CONCLUSIONS: Reliability of the DIS scores for the inexperienced junior and senior PTs was sufficient in comparison with scores from the experienced raters in the previous study, indicating that the DIS can be used by inexperienced PTs new to discriminating between dystonia and choreoathetosis, and also that its reliability is not dependent on clinical expertise. However, based on the measurement errors and questionnaire data, familiarity with operational definitions of dystonia and choreoathetosis is crucial to improve scoring reliability.

The tell-tale: what do heart rate; skin temperature and skin conductance reveal about emotions of people with severe and profound intellectual disabilities?

Identifying emotions in people with severe and profound intellectual disabilities is a difficult challenge. Since self-reports are not available, behaviour is the most used source of information. Given the limitations and caveats associated with using behaviour as the sole source of information about their emotions, it is important to supplement behavioural information with information from another source. As it is accepted that emotions consist of language, behaviour and physiology, in this article we investigated if physiology could give information about the emotions of people with severe and profound intellectual disabilities. To this aim we tested hypotheses derived from the motivational model of Bradley, Codispoti, Cuthbert, and Lang (2001) about the relation between heart rate and the valence of emotions and between heart rate, skin conductance and skin temperature and behavioural expressions of emotions of people with severe and profound intellectual disability. We presented 27 participants with 4 staff-selected negative and 4 staff-selected positive stimuli. The situations were videotaped and their heart rate, skin conductance and skin temperature was measured. Each behaviour of the participant was coded using the observational method developed by Petry and Maes (2006). As hypothesized, we found a lower heart rate when participants were presented with negative stimuli than when they were presented with positive stimuli in the first 6s of stimuli presentation. Their skin temperature was higher for the expression of low intensity negative emotions compared to the expression of low intensity positive emotions. The results suggest that, as with people without disability, heart rate and skin temperature can give information about the emotions of persons with severe and profound ID.

The Dyskinesia Impairment Scale: a new instrument to measure dystonia and choreoathetosis in dyskinetic cerebral palsy.

AIM: The aim of this study was to examine the reliability and validity of the Dyskinesia Impairment Scale (DIS). The DIS consists of two subscales: dystonia and choreoathetosis. It measures both phenomena in dyskinetic cerebral palsy (CP). METHOD: Twenty-five participants with dyskinetic CP (17 males; eight females; age range 5­22y; mean age 13y 6mo; SD 5y 4mo), recruited from special schools for children with motor disorders, were included. Exclusion criteria were changes in muscle relaxant medication within the previous 3 months, orthopaedic or neurosurgical interventions within the previous year, and spinal fusion. Interrater reliability was verified by two independent raters. For interrater reliability, intraclass correlation coefficients were assessed. Standard error of measurement, the minimal detectable difference, and Cronbach's alpha for internal consistency were determined. For concurrent validity of the DIS dystonia subscale, the Barry­Albright Dystonia Scale was administered. RESULTS: The intraclass correlation coefficient for the total DIS score and the two subscales ranged between 0.91 and 0.98 for interrater reliability. The reliability of the choreoathetosis subscale was found to be higher than that of the dystonia subscale. The standard error of the measurement and minimal detectable difference values were adequate. Cronbach's alpha values ranged from 0.89 to 0.93. Pearson's correlation between the dystonia subscale and Barry­Albright Dystonia Scale was 0.84 (p<0.001). INTERPRETATION: Good to excellent reliability and validity were found for the DIS. The DIS may be promising for increasing insights into the natural history of dyskinetic CP and evaluating interventions. Future research on the responsiveness of the DIS is warranted.