Uncovering the evidence for behavioral interventions with individuals with fragile X syndrome: A systematic review

Moskowitz, Lauren J.; Jones, Emily A.

doi:10.1016/j.ridd.2014.12.011

Cited by 27 publications

(15 citation statements)

References 77 publications

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“…In a recent systematic review of 31 intervention studies of individuals with FXS, overall results suggested that a behavioral approach to intervention shows promise [16]. Preliminary experience indicates that assistive technology (i.e.…”

Section: Introductionmentioning

confidence: 99%

Characterization, treatment patterns, and patient-related outcomes of patients with Fragile X syndrome in Germany: final results of the observational EXPLAIN-FXS study

et al. 2016

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BackgroundAs data on the phenotype, characteristics and management of patients with Fragile X Syndrome (FXS) are limited, we aimed to collect such data in Germany in experienced centres involved in the treatment of such patients.MethodsEXPLAIN-FXS is a prospective observational (non-interventional) study (registry) performed between April 2013 and January 2016 at 18 sites in Germany. Requirements for patient participation included confirmed diagnosis of FXS by genetic testing (>200 CGG repeats) and written informed consent. Patients were followed for up to 2 years.ResultsSeventy-five patients (84.0 % males, mean age 16.7 ± 14.5 years, ranging from 2 - 82 years) were analysed. The mean 6-item score, determined according to Giangreco (J Pediatr 129:611-614, 1996), was 6.9 ± 2.5 points. At least one neurological finding each was noted in 53 patients (69.7 %). Specifically, ataxia was noted in 5 patients (6.6 %), lack of fine motor skills in 40 patients, (52.6 %), muscle tonus disorder in 4 patients (5.3 %), and other neurological disorders in 39 patients (51.3 %). Spasticity was not noted in any patient.Seizures were reported in 6 patients (8.1 %), anxiety disorders in 22 patients (30.1 %), depression in 7 patients (9.6 %), ADHD/ADD in 36 patients (49.3 %), impairment of social behavior in 39 patients (53.4 %), and other comorbidities in 23 patients (31.5 %). The mean Aberrant Behaviour Checklist Community Edition (ABC-C) score on behavioral symptoms, obtained in 71 patients at first documentation, was 48.4 ± 27.8 (median 45.0, range 5-115). The mean visual analogue scale (VAS) score, obtained in 59 patients at first documentation, was 84.9 ± 14.6 points (median 90; range 50 – 100).ConclusionsThis report describes the largest cohort of patients with FXS in Europe. The reported observations indicate a substantial burden of disease for patients and their caregivers. Based on these observations, an early expert psychiatric diagnosis is recommended for suspected FXS patients. Further recommendations include multimodal and multi-professional management that is tailored to the individual patient’s needs.Trial registrationThe ClinTrials.gov identifier is NCT01711606. Registered on 18 October 2012.

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Section: Introductionmentioning

confidence: 99%

Characterization, treatment patterns, and patient-related outcomes of patients with Fragile X syndrome in Germany: final results of the observational EXPLAIN-FXS study

et al. 2016

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“…Despite overwhelming empirical support for the use of applied behavior analysis in assessment and treatment of problem behavior in persons with IDD, there is a paucity of research describing behavioral interventions for SIB, aggression, and other severe behavior problems exhibited by individuals with FXS in particular (e.g., Moskowitz, Carr, & Durand, 2011;Moskowitz & Jones, 2015).…”

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confidence: 99%

“…In their recent review of the literature, Moskowitz and Jones (2015) identified 31 studies that described behavioral interventions used with individuals with FXS, but reductions in problem behavior were targeted in only 14 of these studies. Furthermore, only six studies reported on behavioral treatments that targeted SIB and/or aggression in a total of nine individuals with FXS.…”

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confidence: 99%

Functional analysis and treatment of problem behavior exhibited by children with fragile X syndrome

Kurtz

Chin

Robinson

et al. 2015

Research in Developmental Disabilities

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“…A number of behavioural treatment approaches have been conducted to reduce a variety of the symptoms shown by individuals with FXS including problem behaviours (Moskowitz & Jones ). Given that the SIB displayed by participants with FXS in the current study were sensitive to social consequences in just under half of cases, it seems critical that individuals with FXS receive environmental support to reduce the likelihood that SIB is inadvertently reinforced.…”

Section: Discussionmentioning

confidence: 99%

Examining the influence of social‐environmental variables on self‐injurious behaviour in adolescent boys with fragile X syndrome

Hall

Hustyi

Barnett

2018

J intellect Disabil Res

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Uncovering the evidence for behavioral interventions with individuals with fragile X syndrome: A systematic review

Cited by 27 publications

References 77 publications

Characterization, treatment patterns, and patient-related outcomes of patients with Fragile X syndrome in Germany: final results of the observational EXPLAIN-FXS study

Characterization, treatment patterns, and patient-related outcomes of patients with Fragile X syndrome in Germany: final results of the observational EXPLAIN-FXS study

Functional analysis and treatment of problem behavior exhibited by children with fragile X syndrome

Examining the influence of social‐environmental variables on self‐injurious behaviour in adolescent boys with fragile X syndrome

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