Adapting the Mullen Scales of Early Learning for a Standardized Measure of Development in Children With Rett Syndrome

2017 ◽  
Vol 55 (6) ◽  
pp. 419-431 ◽  
Author(s):  
Tessa Clarkson ◽  
Jocelyn LeBlanc ◽  
Geneva DeGregorio ◽  
Vanessa Vogel-Farley ◽  
Katherine Barnes ◽  
...  

Abstract Rett Syndrome (RTT) is characterized by severe impairment in fine motor (FM) and expressive language (EL) function, making accurate evaluations of development difficult with standardized assessm ents. In this study, the administration and scoring of the Mullen Scales of Early Learning (MSEL) were adapted to eliminate the confounding effects of FM and EL impairments in assessing development. Forty-seven girls with RTT were assessed with the Adapted-MSEL (MSEL-A), a subset (n = 30) was also assessed using the Vineland Adaptive Behavior Scales-Second Edition (Vineland-II) and a further subset (n = 17) was assessed using an eye-tracking version of the MSEL (MSEL-ET). Participants performed better on the visual reception (VR) and receptive language (RL) domains compared to the FM and EL domains on the MSEL-A. Individual performance on each domain was independent of other domains. Corresponding MSEL-A and Vineland-II domains were significantly correlated. The MSEL-ET was as accurate as the MSEL-A in assessing VR and RL, yet took a 44% less time. Results suggested that the MSEL-A and the MSEL-ET could be viable measures for accurately assessing developmental domains in children with RTT.

Author(s):  
Smita Nimkar ◽  
Suvarna Joshi ◽  
Aarti Kinikar ◽  
Chhaya Valvi ◽  
D Bella Devaleenal ◽  
...  

Abstract Introduction Tuberculous meningitis (TBM) results in significant morbidity and mortality among children worldwide. Associated neurocognitive complications are common but not well characterized. The Mullen Scales of Early Learning (MSEL), a well-established measure for assessment of neurodevelopment, has not yet been adapted for use in India. This study’s goal was to adapt the MSEL for local language and culture to assess neurocognition among children in India, and apply the adapted measure for assessment of children with TBM. Methods Administration of MSEL domains was culturally adapted. Robust translation procedures for instructions took place for three local languages: Marathi, Hindi and Tamil. Multilingual staff compared instructions against the original version for accuracy. The MSEL stimuli and instructions were reviewed by psychologists and pediatricians in India to identify items concerning for cultural bias. Results MSEL stimuli unfamiliar to children in this setting were identified and modified within Visual Reception, Fine-Motor, Receptive Language and Expressive Language Scales. Item category was maintained for adaptations of items visually or linguistically different from those observed in daily life. Adjusted items were administered to six typically developing children to determine modification utility. Two children diagnosed with confirmed TBM (ages 11 and 29 months) were evaluated with the adapted MSEL before receiving study medications. Skills were below age-expectation across visual reception, fine motor and expressive language domains. Conclusions This is the first study to assess children with TBM using the MSEL adapted for use in India. Future studies in larger groups of Indian children are warranted to validate the adapted measure.


2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Maureen Black ◽  
Doris Yimgang ◽  
Kristen Hurley ◽  
Kimberly Harding ◽  
Silvia Fernandez-Rao ◽  
...  

Abstract Objectives In low and middle-income countries, early child development (ECD) is associated with stunting, but the association with length-for-age z-scores (LAZ) is understudied. The objective is to examine whether the association with ECD among infants extends beyond stunting to LAZ and whether it is altered by nurturance or inflammation. Methods Sample: 513 infants (mean age 8.6 months, SD 2.2), 20% stunted (LAZ < -2) participated in a randomized controlled trial of micronutrient powder (MNP) in rural India. Following baseline, infants were re-evaluated at 6- and 12-months. LAZ was calculated from measured length, inflammation (C-reactive protein, CRP) from blood draw; nurturance from home observation (HOME Inventory), and ECD from Mullens Early Learning Scale (visual reception, fine/gross motor and receptive/expressive language). Linear mixed effects models were conducted, accounting for repeated measures and clustering, adjusted for child age, anemia, maternal education, household assets, and intervention. LAZ interactions with CRP and HOME scores were tested. Results LAZ was significantly positively associated with all ECD domains over time. HOME was positively associated with visual reception and expressive language (Table 1). HOME interactions were marginal for fine motor (P = 0.058) and significant for receptive language (P = 0.015). For HOME scores < -1 SD, LAZ was positively related to fine motor and receptive language, for HOME scores >1 SD, LAZ was not related to ECD (Figure 1). CRP was not related to ECD and CRP interaction was not significant. Conclusions The positive association between LAZ and ECD illustrates vulnerability prior to the threshold of stunting. Maternal nurturance is positively associated with multiple domains of infants’ ECD and attenuates relations between LAZ and receptive language and fine motor. Inflammation (measured by CRP) is not associated with ECD. Linear growth within normal and nurturant caregiving are needed to promote ECD. Funding Sources Mathile Institute for the Advancement of Human Nutrition, Nutrition International, Sackler Institute for Nutrition Science of the New York Academy of Sciences.


Author(s):  
Juan Bornman ◽  
MaryAnn Romski ◽  
Kerstin Tonsing ◽  
Rose Sevcik ◽  
Robyn White ◽  
...  

Background: South African speech-language therapists have identified the need for culturally valid and sensitive assessment tools that can accommodate multiple languages and cover a reasonable age range. The Mullen Scales of Early Learning (MSEL) extend from birth to 68 months, contain five separate subscales including receptive language, expressive language, gross motor, fine motor and visual reception scale, are straightforward to administer and have been successfully used in other African countries, such as Uganda. It also identifies a child’s strengths and weaknesses and provides a solid foundation for intervention planning.Objectives: This research aimed to demonstrate the appropriateness and usefulness of the translated and culturally and linguistically adapted MSEL across four South African languages (Afrikaans, isiZulu, Setswana and South African English) through two sub-aims: (1) to describe differences, if any, in MSEL performance across language groups and (2) to describe differences, if any, in MSEL performance between age groups.Method: A total of 198 typically developing children between the ages of 21 and 68 months spread across the four language groups were individually assessed with the culturally and linguistically adapted and translated MSEL.Results: A one-way analysis of variance (ANOVA) showed no statistically significant differences between the four language groups for total MSEL scores. A Welch’s one-way ANOVA showed that the total MSEL scores were significantly different between age groups.Conclusion: The translation and adaptation of the MSEL was successful and did not advantage or disadvantage children based on their home language, implying that linguistic equivalence was achieved. The MSEL results differed between age groups, suggesting that the measure was also successful in differentiating the performance of children at different developmental levels.


Neurology ◽  
2017 ◽  
Vol 89 (16) ◽  
pp. 1684-1690 ◽  
Author(s):  
Constance L. Smith-Hicks ◽  
Siddharth Gupta ◽  
Joshua B. Ewen ◽  
Manisha Hong ◽  
Lisa Kratz ◽  
...  

Objective:To determine safety and perform a preliminary assessment of dose-dependent efficacy of dextromethorphan in normalizing electrographic spikes, clinical seizures, and behavioral and cognitive functions in girls with Rett syndrome.Methods:We used a prospective randomized, open-label trial in fast metabolizers of dextromethorphan to examine the effect of dextromethorphan on core clinical features of Rett syndrome. Interictal spike activity and clinical seizures were determined using EEG and parent reporting. Cognitive data were obtained using the Mullen Scales of Early Learning and Vineland Adaptive Behavior Scales, while behavioral data were obtained from parent-completed checklists, the Aberrant Behavior Checklist–Community Version, and the Screen for Social Interaction. Anthropometric data were obtained according to the National Health and Nutrition Examination Survey. The Rett Syndrome Severity Scale provided a clinical global impression of the effect of dextromethorphan on clinical severity.Results:Dextromethorphan is safe for use in 3- to 15-year-old girls with Rett syndrome. Thirty-five girls were treated with 1 of 3 doses of dextromethorphan over a period of 6 months. Statistically significant dose-dependent improvements were seen in clinical seizures, receptive language, and behavioral hyperactivity. There was no significant improvement in global clinical severity as measured by the Rett Syndrome Severity Scale.Conclusions:Dextromethorphan is a potent noncompetitive antagonist of the NMDA receptor channel that is safe for use in young girls with Rett syndrome. Preliminary evidence suggests that dextromethorphan may improve some core features of Rett syndrome.Classification of evidence:This study provides Class IV evidence that dextromethorphan at various doses does not change EEG spike counts over 6 months, though precision was limited to exclude an important effect.


2020 ◽  
Vol 11 ◽  
Author(s):  
Jenny Luu ◽  
Rachel Jellett ◽  
Maya Yaari ◽  
Melissa Gilbert ◽  
Josephine Barbaro

Introduction: Previous research suggests children diagnosed with autism spectrum disorder (ASD or “autism”) born extremely and very preterm face substantially delayed development than their peers born full-term. Further, children born preterm are proposed to show a unique behavioral phenotype, which may overlap with characteristics of autism, making it difficult to disentangle their clinical presentation. To clarify the presentation of autism in children born preterm, this study examined differences in key indicators of child development (expressive language, receptive language, fine motor, and visual reception) and characteristics of autism (social affect and repetitive, restricted behaviors).Materials and Methods: One fifty-eight children (136 full-term, twenty-two preterm) diagnosed with autism, aged 22–34 months, were identified prospectively using the Social Attention and Communication Surveillance tools during community-based, developmental surveillance checks in the second year of life. Those identified at “high likelihood” of an autism diagnosis were administered the Mullen Scales of Early Learning and the Autism Diagnostic Observation Schedule.Results: The children born preterm and full-term did not differ significantly in their fine motor, visual reception, expressive language, or receptive language skills. No significant differences in social affect and repetitive and restrictive behavior traits were found.Discussion: The findings of this study differs from previous research where children diagnosed with autism born very or extremely preterm were developmentally delayed and had greater autistic traits than their term-born peers. These null findings may relate to the large proportion of children born moderate to late preterm in this sample. This study was unique in its use of a community-based, prospectively identified sample of children diagnosed with autism at an early age. It may be that children in these groups differ from clinic- and hospital-based samples, that potential differences emerge later in development, or that within the autism spectrum, children born preterm and full-term develop similarly. It was concluded that within the current sample, at 2 years of age, children diagnosed with autism born preterm are similar to their peers born full-term. Thus, when clinicians identify characteristics of autism in children born preterm, it is important to refer the child for a diagnostic assessment for autism.


2018 ◽  
Vol 3 (5) ◽  
pp. e000747 ◽  
Author(s):  
Gillian A Lancaster ◽  
Gareth McCray ◽  
Patricia Kariger ◽  
Tarun Dua ◽  
Andrew Titman ◽  
...  

BackgroundRenewed global commitment to the improvement of early child development outcomes, as evidenced by the focus of the United Nations Sustainable Development Goal 4, highlights an increased need for reliable and valid measures to evaluate preventive and interventional efforts designed to affect change. Our objective was to create a new tool, applicable across multicultures, to measure development from 0 to 3 years through metadata synthesis.MethodsFourteen cross-sectional data sets were contributed on 21 083 children from 10 low/middle-income countries (LMIC), assessed using seven different tools (caregiver reported or directly assessed). Item groups, measuring similar developmental skills, were identified by item mapping across tools. Logistic regression curves displayed developmental trajectories for item groups across countries and age. Following expert consensus to identify well-performing items across developmental domains, a second mapping exercise was conducted to fill any gaps across the age range. The first version of the tool was constructed. Item response analysis validated our approach by putting all data sets onto a common scale.Results789 individual items were identified across tools in the first mapping and 129 item groups selected for analysis. 70 item groups were then selected through consensus, based on statistical performance and perceived importance, with a further 50 items identified at second mapping. A tool comprising 120 items (23 fine motor, 23 gross motor, 20 receptive language, 24 expressive language, 30 socioemotional) was created. The linked data sets on a common scale showed a curvilinear trajectory of child development, highlighting the validity of our approach through excellent coverage by age and consistency of measurement across contributed tools, a novel finding in itself.ConclusionsWe have created the first version of a prototype tool for measuring children in the early years, developed using novel easy to apply methodology; now it needs to be feasibility tested and piloted across several LMICs.


2012 ◽  
Vol 22 (5) ◽  
pp. 514-519 ◽  
Author(s):  
Annette Majnemer ◽  
Catherine Limperopoulos ◽  
Michael Shevell ◽  
Charles Rohlicek ◽  
Bernard Rosenblatt ◽  
...  

AbstractObjectiveThis study compares the developmental and functional outcomes at school entry between boys and girls born with a congenital cardiac defect who required early surgical correction.Study designA prospective cohort of 94 children, including 49 percent boys, were followed up to 5 years of age and assessed for developmental progress. Developmental measures included Wechsler Preschool and Primary Scale of Intelligence – cognitive; Peabody Picture Vocabulary Test – receptive language; Peabody Developmental Motor Scale – motor; and Child Behaviour Checklist – behaviour. Measures of function included the Vineland Adaptive Behavior Scale and Functional Independence Measure for Children (WeeFIM).ResultsThe mean scores of the boys on the WeeFIM subscales, such as self-care, mobility, cognition, were significantly lower than that of the girls. There was a trend for a greater proportion of boys to have abnormalities on neurological examination (boys 37.5 percent abnormal, girls 19.5 percent abnormal). Verbal, performance, and full scale Intellectual Quotients were 5–7 points lower in boys but did not reach significance (full scale Intellectual Quotient: boys 87.7 plus or minus 22.2; girls 93.9 plus or minus 19.3). Boys were more likely to have fine motor delays (50 percent, 82.7 plus or minus 16.5) compared with girls (28.2 percent, 87.0 plus or minus 15.8). There were no gender differences in receptive language or behavioural difficulties.ConclusionsBoys born with congenital heart disease requiring early surgical repair appear to be at enhanced risk for neuromotor impairments and activity limitations. Findings support gender differences in the pathogenesis of early brain injury following hypoxic–ischaemic insults. This has implications for neuroprotective strategies to prevent brain injury.


2020 ◽  
Vol 11 (2) ◽  
pp. 351-357
Author(s):  
Renu Rathi ◽  
Bharat Rathi ◽  
Rakesh Khatana ◽  
Suraj Sankh

Background: Rett syndrome-RS comes under Autism spectrum disorder-ASD which is a neurodevelopmental syndrome. It is diagnosed by the main differentiating features of lack of interpersonal and communication skills, poor eye contact, delayed speech with pervasive abnormal body movements. Aim and Objectives: This case report is aimed at dissemination of comprehensive role of Ayurveda in management of ASD, Rett syndrome. Material and Methods: RS is the severe form of ASD. This case study of 2.3 year’s girl presented with RS and global delay, being treated with wholistic approach. It comprises Ayurveda chikitsa and other therapies like Yoga, hydrotherapy, occupational, music, physiotherapy and many more. Observation and Result: Patient has shown promising results in all developmental milestones such as gross motor, fine motor and personal social in 6 months duration except language. Different varieties of massage therapy, diet and Basti, Nasya (Panchkarma) procedures, Omkar mantra chanting, passive Yogasana were done. Conclusion: In this case report, mainly Ayurveda interventions were implemented with wholistic approach as an adjuvant, received good result in gross motor development which is very difficult in RS, hence it is a unique case. It also opened the door of wholistic approach with the hope to deliver the good result in similar disorders.


2019 ◽  
Vol 6 (Supplement_2) ◽  
pp. S990-S991
Author(s):  
Daniel Olson ◽  
Molly Lamb ◽  
Amy Connery ◽  
Kathryn Colborn ◽  
Muktha S Natrajan ◽  
...  

Abstract Background The impact of early post-natal Zika virus (ZIKV) infection on neurodevelopment (ND) is unknown. A prospective study of post-natal ZIKV infection in rural Guatemala (ZIKV study) enrolled a cohort of children ages 1–5 years, including children previously enrolled in a dengue virus (DENV) study during the 2015–2016 ZIKV epidemic. We evaluated ND outcomes by age and ZIKV infection status. Methods Subjects enrolled in the ZIKV study June 2017–April 2018 underwent ND testing using the Mullen Scales of Early Learning (MSEL) at baseline and 12 months later. ZIKV/DENV-1/2 FRNT50 was performed on enrollment and on banked serum samples from the 2015 to 2016 subset. ZIKV serostatus and MSEL scores were correlated using multiple linear mixed models, adjusted for age and gender when appropriate, to evaluate their association. Geolocation was used to explore clustering of ZIKV serostatus and MSEL score. Results We enrolled 183 children (43% female, mean age 3.2 years). Of these, 38 (21%) were classified as ZIKV-positive (+), 111 (61%) ZIKV-negative (-), 31 (17%) ZIKV-possible, and 3 (2%) ZIKV-indeterminate. ZIKV(+) cases and higher composite MSEL scores clustered in more densely populated areas (Figure 1). ZIKV(+) serostatus was associated with higher MSEL composite (increase in log score 0.09, P = 0.003) and subdomain scores: fine motor (0.13, P = 0.011), visual reception (0.15, P = 0.002), receptive language (0.09, P = 0.041), gross motor (0.14, P = 0.09), and expressive language (0.09, P = 0.058) (Figure 2). Of the 78 children (43%) with 2015–2016 samples, 46 (59%) remained ZIKV(−), 16 (21%) seroconverted from ZIKV(−) or possible/indeterminate to ZIKV(+), and 16 (21%) were indeterminate when enrolled in the ZIKV study. ZIKV seroconversion was associated with higher composite (0.13, P = 0.02) MSEL scores compared with ZIKV(−). Conclusion In this exploratory analysis, post-natal ZIKV infection was not associated with adverse ND outcomes in children age 1–5 years. Overall, ZIKV(+) status was associated with higher average ND scores than ZIKV(−), and scores decreased with age for most children, independent of ZIKV status. The correlation of ZIKV(+) status and higher MSEL scores may be confounded by geographic-related factors or other confounders. NIAID Contract HHSN272201300015I Task Order HHSN27200013 (Co-PIs: FMM & EJA). Disclosures Flor M. Munoz, M.D, Biocryst: Grant/Research Support; CDC: Research Grant; Moderna: Other Financial or Material Support, Safety Monitoring Board Member/Chair; NIH: Research Grant; Novavax: Research Grant; UP to Date: Author and Editor - Royalties, Other Financial or Material Support.


2019 ◽  
Vol 34 (7) ◽  
pp. 1246-1246
Author(s):  
A M Colbert ◽  
D Bauer ◽  
P Arroyave ◽  
S Hernández ◽  
M A Martínez ◽  
...  

Abstract Objective The literature supports using tests developed in high-income countries to assess children in low and lower-middle income countries (LMICs) when carefully translated, adapted, and applied (Holding et al., 2018; Mitchell et al., 2017). Research has shown the Mullen Scales of Early Learning (MSEL) to have adequate validity and sensitivity when used in LMICs (Bangirana et al., 2014; Koura et al., 2013), as well as equivalency to the American normative sample in lower risk populations (Bornman et al., 2010). Here, we describe the pattern of MSEL results in rural Guatemala. Participants and Method Children (n = 842; M enrollment age = 15.9 months; range 0-5 years) enrolled in an observational study of postnatal Zika exposure in rural Guatemala were administered an adapted and translated version of the MSEL (Connery et al., in press). To date, 352 children completed one, 393 children completed two, and 97 children completed three MSELs, for a total of 1,429 administrations. Results MSEL composite scores were similar to the American normative sample in children &lt;12 months (M = 93.3, SD = 11.1), but lower for children ages 1-5 years (mean = 71.1, SD = 15.1, p &lt; 0.0001). Moreover, lower scores were observed in children ages 1-5 years for all MSEL subscales, with the largest differences observed in receptive language (&lt;12 years: mean = 47.8, SD = 7.1; 1-5 years: mean = 35.1, SD = 10.0, p &lt; 0.0001). Conclusions Results are consistent with research that demonstrates a widening gap in test performance over time between children from higher and lower risk communities (Fernald et al., 2011; Paxson et al., 2005; Schady et al., 2015). Although findings are not meant to diagnose individual children, they highlight population changes in neurodevelopmental skills and the need for a better understanding of developmental patterns in LMICs. Future analyses will evaluate the impact of developmental risk factors over time and the performance of the MSEL in this population. References Bangirana, P., Opoka, R. O., Boivin, M. J., Idro, R., Hodges, J. S., Romero, R. A., … John, C. C. (2014). Severe Malarial Anemia is Associated With Long-term Neurocognitive Impairment. Clinical Infectious Diseases, 59(3), 336–344. https://doi.org/10.1093/cid/ciu293. Bornman, J., Sevcik, R. A., Romski, M., & Pae, H. K. (2010). Successfully Translating Language and Culture when Adapting Assessment Measures, ppi_254 111.118. Retrieved from https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1741-1130.2010.00254.x. Fernald, L. C. H., Weber, A., Galasso, E., & Ratsifandrihamanana, L. (2011). Socioeconomic gradients and child development in a very low income population: Evidence from Madagascar. Developmental Science, 14(4), 832–847. https://doi.org/10.1111/j.1467-7687.2010.01032.x. Holding, P., Anum, A., van de Vijver, F. J. R., Vokhiwa, M., Bugase, N., Hossen, T., … Gomes, M. (2018). Can we measure cognitive constructs consistently within and across cultures? Evidence from a test battery in Bangladesh, Ghana, and Tanzania. Applied Neuropsychology: Child, 7(1), 1-13 https://doi.org/10.1080/21622965.2016.1206823. Koura, K. G., Boivin, M. J., Davidson, L. L., Ouédraogo, S., Zoumenou, R., Alao, M. J., … Bodeau-Livinec, F. (2013). Usefulness of child development assessments for low-resource settings in francophone Africa. Journal of Developmental and Behavioral Pediatrics : JDBP, 34(7), 486–93. https://doi.org/10.1097/DBP.0b013e31829d211c. Mitchell, J. M., Tomlinson, M., Bland, R. M., Houle, B., Stein, A., & Rochat, T. J. (2017). Confirmatory factor analysis of the Kaufman assessment battery in a sample of primary school-aged children in rural South Africa. South African Journal of Psychology, 1–19. https://doi.org/10.1177/0081246317741822. Paxson, C., Schady, N., Izquierdo, S., León, M., Lucio, R., Ponce, J., … Hall, W. (2005). Cognitive Development among Young Children in Ecuador The Roles of Wealth, Health, and Parenting. Retrieved from http://econ.worldbank.org. Schady, N., Behrman, J., Araujo, M. C., Azuero, R., Bernal, R., Bravo, D., … Vakis, R. (2015). Wealth gradients in early childhood cognitive development in five Latin American countries. The Journal of Human Resources, 50(2), 446–463. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/25983344.


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