Developmental profile and diagnoses in children presenting with motor stereotypies

Introduction: Motor stereotypies represent a typical example of the difficulty in distinguishing non-clinical behaviors (physiological and transient) from symptoms or among different disorders (“primary stereotypies”, associated with Autistic Spectrum Disorder, Intellectual Disabilities, genetic syndromes, sensory impairment). Aim of this study was to get an accurate analysis on the relationship between stereotypies and neurodevelopmental disorders. Methods: We studied 23 children (3 girls) aged 36 to 95 months, who requested a consultation due to the persistence or the increase severity of motor stereotypies. None of patients had a previous diagnosis of ASD. The assessment included the Motor Severity Stereotypy Scale (MSSS), the Repetitive Behavior Scale-Revised (RBS-R), the Raven’s Colored Progressive Matrices (CPM), the Child Behavior Checklist for ages 1 ½ -5 or 4-18 (CBCL), the Social Responsiveness Scale (SRS) and the Autism Diagnostic Observation Schedule- Second edition (ADOS 2). Results: All patients were showing motor stereotypies for periods of time varying from 6 to 77 months. The MSSS showed each child had a limited number of stereotypies; their frequency and intensity were mild; the interference of stereotypies was variable; the impairment in the daily life was mild. The RBS-R scores resulted positive for the subscale of “Stereotypic behaviors” in all children; moreover, several children presented other repetitive behaviors, mainly “Ritualistic behavior” and “Sameness behavior”. All patients showed a normal cognitive level. The CBCL evidenced behavioral problems in 22% of the children: Internalizing problems, Attention and Withdrawn were the main complaints. On the SRS, all but one of the tested patients obtained clinical scores in the clinical range at least in one area. On the ADOS 2, four patients obtained scores indicating a moderate level of ASD symptoms, four had a mild level and fifteen showed no or minimal signs of ASD. Discussion: Motor stereotypies in children with normal cognitive level represent a challenging diagnostic issue for which a finely tailored assessment is mandatory in order to define a precise developmental profile. Notably, a careful and cautious use of standardized tests is warranted to avoid misdiagnosis. Furthermore, it is hard to consider motor stereotypies, even the primary ones, exclusively as a movement disorder

Explaining metaphors in high-functioning Autism Spectrum Disorder children: A brief report

This study investigated metaphor comprehension in a group of 24 Italian high-functioning ASD children (mean age: 8.5 y.). Children were administered a test that was composed of 'sensorial metaphors', which are understood by normally developing preschoolers, that the children had to verbally explain. Two normally developing control groups, which were composed of 5- and 6-year olds, were recruited as well, as the highest age for this test is 6 years. A one-way ANOVA applied to the four scores from the test showed a partial delay in the ASD group with respect to the 6-year-old control group, but not with respect to the 5-year-old control group. The ASD group showed some capabilities in metaphor processing; however, there were also some atypical behaviors that suggest that ASD children process metaphorical stimuli in a particular way. (c) 2011 Elsevier Ltd. All rights reserved

The Imaginary companion and the Paracosms in typical and atypical development: A case study [Le compagnon imaginaire et les paracosmes dans le développement typique et atypique à partir d'un cas clinique]

The authors describe the case of a child who was examined when he was 9 years 2 months, after having received a diagnosis of Pervasive developmental disorder not otherwise specified (PDD-NOS) when he was 4 years 10 months. The authors focus on the child's solitary and imagination activities, particularly his imaginary companion and his paracosms, within a general profile of his cognitive, linguistic and social cognition abilities. The child's profile was established partly by using standardized tests assessing intellectual, linguistic and social cognition abilities, and partly by using a semi-structured interview focused on the child's representations of his friend relationships. The results at the tests were analyzed in relation with the age norms for typical development, whereas the child's representations of friend relationships were analyzed in qualitative terms. The child showed brilliant general cognitive capabilities, adult-like language abilities and sophisticated intellectual concerns. However, some social cognition subtests and the semi-structured interview highlighted relevant difficulties in reading mental states. His solitary behaviors were overdeveloped and his imagination activities showed atypical characteristics. The present case study analyzes the distinctive features of these activities in quantitative, qualitative and functional terms, stressing the metacognitive and the emotional dimension inherent to the child's imaginative representations

Esplorare la comprensione dell'ironia in bambini con Disturbo dello Spettro Autistico ad alto funzionamento (ASDHF): i cartoni animati possono aiutare!

La letteratura sulla comprensione dell’ironia mostra una compromissione nei bambini con ASD della capacità di decodificare il linguaggio non letterale, soprattutto l’ironia verbale (Adachi et al., 2004; Martin, McDonald, 2004). Ricerche recenti hanno ottenuto risultati più confortanti, migliorando gli aspetti metodologici della valutazione (Samson, 2013; Weiss et al., 2013). Infatti se le espressioni sono semplici, i contesti ricchi di informazioni, i compiti non esclusivamente verbali, anche i bambini con ASDHF mostrano qualche accesso ai significati dell’ironia. Obiettivo del presente contributo è di analizzare qualitativamente come una prova, costituita da brevi clip di cartoni animati, con finali ironici e letterali, possa valutare la comprensione dell’ironia nei bambini ASDHF. La prova è stata somministrata a un campione di bambini con sviluppo tipico tra i 7 e i 9 anni e, in ambito clinico, a due bambini con ASDHF, di 8 e 9 anni (Melogno et al., 2013). Il materiale comprende segmenti di cartoni animati, con finali di critica ironica, critica letterale e complimento letterale (Dewes et al.,1996). Ogni clip, introdotta da informazioni contestuali, è seguita da una fase in cui adulto e bambino ricostruiscono lo scenario. Segue una domanda con alternative di risposta che verifica se il bambino ha compreso l’espressione-stimolo. Quindi continua l’esplorazione con domande aperte. La prova consente di formulare ipotesi sulla capacità dei bambini di andare oltre il piano letterale per comprendere la discrepanza tra ciò che il parlante dice e ciò che intende dire e sulla percezione delle funzioni sociali del proferimento ironico. Consente, inoltre, di fare ipotesi sul tipo di elaborazione delle informazioni, sulla capacità di integrare informazioni verbali e non verbali tenendo conto di indizi socio-percettivi. Il lavoro, ancora in corso, prevede la somministrazione della prova ad un campione con ASDHF da confrontare con quello a sviluppo tipico e il possibile sviluppo di un’App

Developmental profile and diagnoses in children presenting with motor stereotypies

Introduction: Motor stereotypies represent a typical example of the difficulty in distinguishing non-clinical behaviors (physiological and transient) from symptoms or among different disorders (primary stereotypies, associated with Autistic Spectrum Disorder (ASD), Intellectual Disabilities, genetic syndromes, sensory impairment). The aim of this study was to obtain an accurate assessment on the relationship between stereotypies and neurodevelopmental disorders.Methods: We studied 23 children (3 girls) aged 36 to 95 months, who requested a consultation due to the persistence or increased severity of motor stereotypies. None of patients had a previous diagnosis of ASD. The assessment included the Motor Severity Stereotypy Scale (MSSS), the Repetitive Behavior Scale-Revised (RBS-R), the Raven’s Colored Progressive Matrices (CPM), the Child Behavior Checklist for ages 1 ½ -5 or 4-18 (CBCL), the Social Responsiveness Scale (SRS) and the Autism Diagnostic Observation Schedule- Second edition (ADOS 2). Results: All patients were showing motor stereotypies for periods of time varying from 6 to 77 months. The MSSS showed each child had a limited number of stereotypies; their frequency and intensity were mild. The interference of stereotypies was variable; the impairment in daily life was mild. The RBS-R scores were positive for the subscale of Stereotypic behaviors in all children. Moreover, several children presented other repetitive behaviors, mainly Ritualistic behavior and Sameness behavior. All patients showed a normal cognitive level. The CBCL evidenced behavioral problems in 22% of the children: Internalizing problems, Attention and Withdrawn were the main complaints. On the SRS, all but one of the tested patients obtained clinical scores in the clinical range for at least one area. On the ADOS 2, four patients obtained scores indicating a moderate level of ASD symptoms, four had a mild level and fifteen showed no or minimal signs of ASD.Discussion: Motor stereotypies in children with normal cognitive level represent a challenging diagnostic issue for which a finely tailored assessment is mandatory in order to define a precise developmental profile. Thus, careful and cautious use of standardized tests is warranted to avoid misdiagnosis. Furthermore, it is hard to consider motor stereotypies, even the primary ones, exclusively as a movement disorder

Aspetti individuali, interpersonali e sociali del bullismo etnico: studio su un campione nazionale di studenti della scuola secondaria di primo grado

La presente ricerca ha esplorato alcuni dei fattori potenzialmente coinvolti nel bullismo etnico (rendimento scolastico, relazioni amicali e integrazione sociale percepita tra studenti italiani e stranieri) coinvolgendo studenti italiani e stranieri provenienti da diversi istituti scolastici secondari di primo grado. Nel primo studio, le informazioni raccolte su un campione di studenti italiani (N = 1638) hanno evidenziato la presenza di una relazione negativa tra bullismo e rendimento scolastico e tra bullismo e integrazione sociale percepita con i compagni di scuola stranieri, mentre nessun dato rilevante è emerso in merito alle relazioni amicali. Lo studio 2 ha analizzato invece la vittimizzazione nel gruppo degli studenti stranieri (N = 425) ed ha rilevato relazioni significative e negative solo con le relazioni amicali. Questi esiti richiedono un approfondimento sul tema del bullismo etnico e suggeriscono di prestare maggiore attenzione alle relazioni sociali tra pari e alla qualità dell’integrazione scolastica tra studenti stranieri e nativi nei programmi di intervento antibullismo e, più in generale, nei percorsi educativi

CPX-351 treatment in secondary acute myeloblastic leukemia is effective and improves the feasibility of allogeneic stem cell transplantation: results of the Italian compassionate use program

Secondary acute myeloid leukemia (sAML) poorly responds to conventional treatments and allogeneic stem cell transplantation (HSCT). We evaluated toxicity and efficacy of CPX-351 in 71 elderly patients (median age 66 years) with sAML enrolled in the Italian Named (Compassionate) Use Program. Sixty days treatment-related mortality was 7% (5/71). The response rate at the end of treatment was: CR/CRi in 50/71 patients (70.4%), PR in 6/71 (8.5%), and NR in 10/71 (19.7%). After a median follow-up of 11 months relapse was observed in 10/50 patients (20%) and 12 months cumulative incidence of relapse (CIR) was 23.6%. Median duration of response was not reached. In competing risk analysis, CIR was reduced when HSCT was performed in first CR (12 months CIR of 5% and 37.4%, respectively, for patients receiving (=20) or not (=30) HSCT, p\u2009=\u20090.012). Twelve-months OS was 68.6% (median not reached). In landmark analysis, HSCT in CR1 was the only significant predictor of longer survival (12 months OS of 100 and 70.5%, for patients undergoing or not HSCT in CR1, respectively, p\u2009=\u20090.011). In conclusion, we extend to a real-life setting, the notion that CPX is an effective regimen for high risk AML patients and may improve the results of HSCT