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Search: WFRF:(Perlman Dana)

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  • Ahmadi, Asghar, et al. (author)
  • A Classification System for Teachers’ Motivational Behaviors Recommended in Self-Determination Theory Interventions
  • 2023
  • In: Journal of Educational Psychology. - Washington, DC : American Psychological Association (APA). - 0022-0663 .- 1939-2176. ; 115:8, s. 1158-1176
  • Journal article (peer-reviewed)abstract
    • Teachers’ behavior is a key factor that influences students’ motivation. Many theoretical models have tried to explain this influence, with one of the most thoroughly researched being self-determination theory (SDT). We used a Delphi method to create a classification of teacher behaviors consistent with SDT. This is useful because SDT-based interventions have been widely used to improve educational outcomes. However, these interventions contain many components. Reliably classifying and labeling those components is essential for implementation, reproducibility, and evidence synthesis.We used an international expert panel (N = 34) to develop this classification system. We started by identifying behaviors from existing literature, then refined labels, descriptions, and examples using the Delphi panel’s input. Next, the panel of experts iteratively rated the relevance of each behavior to SDT, the psychological need that each behavior influenced, and its likely effect on motivation. To create a mutually exclusive and collectively exhaustive list of behaviors, experts nominated overlapping behaviors that were redundant, and suggested new ones missing from the classification. After three rounds, the expert panel agreed upon 57 teacher motivational behaviors (TMBs) that were consistent with SDT. For most behaviors (77%), experts reached consensus on both the most relevant psychological need and influence on motivation. Our classification system provides a comprehensive list of TMBs and consistent terminology in how those behaviors are labeled. Researchers and practitioners designing interventions could use these behaviors to design interventions, to reproduce interventions, to assess whether these behaviors moderate intervention effects, and could focus new research on areas where experts disagreed. © 2023 American Psychological Association
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2.
  • Ebrahimi-Fakhari, Darius, et al. (author)
  • Defining the clinical, molecular and imaging spectrum of adaptor protein complex 4-associated hereditary spastic paraplegia
  • 2020
  • In: Brain. - OXFORD ENGLAND : Oxford University Press (OUP). - 0006-8950 .- 1460-2156. ; 143:10, s. 2929-2944
  • Journal article (peer-reviewed)abstract
    • Bi-allelic loss-of-function variants in genes that encode subunits of the adaptor protein complex 4 (AP-4) lead to prototypical yet poorly understood forms of childhood-onset and complex hereditary spastic paraplegia: SPG47 (AP4B1), SPG50 (AP4M1), SPG51 (AP4E1) and SPG52 (AP4S1). Here, we report a detailed cross-sectional analysis of clinical, imaging and molecular data of 156 patients from 101 families. Enrolled patients were of diverse ethnic backgrounds and covered a wide age range (1.0-49.3 years). While the mean age at symptom onset was 0.8 +/- 0.6 years [standard deviation (SD), range 0.2-5.0], the mean age at diagnosis was 10.2 +/- 8.5 years (SD, range 0.1-46.3). We define a set of core features: early-onset developmental delay with delayed motor milestones and significant speech delay (50% non-verbal); intellectual disability in the moderate to severe range; mild hypotonia in infancy followed by spastic diplegia (mean age: 8.4 +/- 5.1 years, SD) and later tetraplegia (mean age: 16.1 +/- 9.8 years, SD); postnatal microcephaly (83%); foot deformities (69%); and epilepsy (66%) that is intractable in a subset. At last follow-up, 36% ambulated with assistance (mean age: 8.9 +/- 6.4 years, SD) and 54% were wheelchair-dependent (mean age: 13.4 +/- 9.8 years, SD). Episodes of stereotypic laughing, possibly consistent with a pseudobulbar affect, were found in 56% of patients. Key features on neuroimaging include a thin corpus callosum (90%), ventriculomegaly (65%) often with colpocephaly, and periventricular white-matter signal abnormalities (68%). Iron deposition and polymicrogyria were found in a subset of patients. AP4B1-associated SPG47 and AP4M1-associated SPG50 accounted for the majority of cases. About two-thirds of patients were born to consanguineous parents, and 82% carried homozygous variants. Over 70 unique variants were present, the majority of which are frameshift or nonsense mutations. To track disease progression across the age spectrum, we defined the relationship between disease severity as measured by several rating scales and disease duration. We found that the presence of epilepsy, which manifested before the age of 3 years in the majority of patients, was associated with worse motor outcomes. Exploring genotype-phenotype correlations, we found that disease severity and major phenotypes were equally distributed among the four subtypes, establishing that SPG47, SPG50, SPG51 and SPG52 share a common phenotype, an 'AP-4 deficiency syndrome'. By delineating the core clinical, imaging, and molecular features of AP-4-associated hereditary spastic paraplegia across the age spectrum our results will facilitate early diagnosis, enable counselling and anticipatory guidance of affected families and help define endpoints for future interventional trials.
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