Communication assessment for individuals with Rett syndrome: A systematic review

Sigafoos, Jeff; Kagohara, Debora M.; Meer, Larah van der; Green, Vanessa A.; O’Reilly, Mark F.; Lancioni, Giulio E.; Lang, Russell; Rispoli, Mandy; Zisimopoulos, Dimitrios

doi:10.1016/j.rasd.2010.10.006

Cited by 44 publications

(38 citation statements)

References 19 publications

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“…The authors reported that, although caregivers and teachers interpreted the behaviors as communicative, it was unclear from the available information whether the behaviors were intentional communicative bids, or simply expressions of physiological arousal in response to environmental change (autonomic instability is a comorbid characteristic of the syndrome). Sigafoos et al (2011) subsequently reviewed the eight published studies that have explicitly examined communicative behaviors with individuals with RTT (combined N 5 41). Evidence of communicative behaviors was found for some participants in each of the studies, but the variability in methods made it impossible to draw firm conclusions regarding the range of communicative behaviors and abilities in this population.…”

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confidence: 99%

“…Collectively, the literature reviews conducted by Sigafoos and colleagues (2009;2011) reveal two important gaps in the RTT communication literature: (a) studies confirming the communicative functions of potentially communicative behaviors, and (b) studies employing recognized research designs and reliable measurement of dependent variables to generate experimental evidence of positive responses to communication interventions. The purpose of the current preliminary study was to begin addressing both of these gaps by assessing the capacity of a small clinical sample of individuals with RTT to learn and produce novel, functionally communicative behaviors evaluated using well-established FA-FCT procedures and methodologically rigorous, singlesubject experimental designs.…”

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confidence: 99%

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Functional Communication Training in Rett Syndrome: A Preliminary Study

Byiers¹,

Dimian

Symons³

2014

American Journal on Intellectual and Developmental Disabilities

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Rett syndrome (RTT) is associated with a range of serious neurodevelopmental consequences including severe communicative impairments. Currently, no evidence-based communication interventions exist for the population ( Sigafoos et al., 2009 ). The purpose of the current study was to examine the effectiveness of functional assessment (FA) and functional communication training (FCT) methods for teaching 3 individuals (ages 15-47 years) with classic RTT novel communicative behaviors. Using single-case experimental designs, functional reinforcers were identified (FA) and each participant quickly learned to activate a voice-output switch to obtain a reinforcer (FCT). These results suggest that individuals with classic RTT can learn novel communicative responses, which has important implications for future intervention research.

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confidence: 99%

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confidence: 99%

Functional Communication Training in Rett Syndrome: A Preliminary Study

Byiers¹,

Dimian

Symons³

2014

American Journal on Intellectual and Developmental Disabilities

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“…2,9 In this late stage, various prelinguistic behavioural patterns such as specific body movements, reaching, and facial expressions have been reported to fulfil a communicative function, eye gazing being the most common form. [10][11][12][13][14] The developmental pathway (especially the first 2y of life) of such idiosyncratic communicative behavioural patterns resembles a black box, which requires further study. The early verbal development of females with PSV might prove crucial for the further understanding of speech-language acquisition in the context of the whole RTT complex, since early vocalizations in infancy constitute precursors to speech.…”

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confidence: 99%

Early speech–language development in females with Rett syndrome: focusing on the preserved speech variant

Marschik

Pini

Bartl-Pokorny

et al. 2012

Develop Med Child Neuro

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ABBREVIATIONSPSV Preserved speech variant RTT Rett syndrome AIM Our aim was to contribute new findings related to the pre-regressional verbal development of females with a variant of Rett syndrome (RTT) as the loss of spoken language is one of the key clinical features of RTT, and it would be of particular interest to study the early speech-language development of females who are considered to have preserved some speech-language abilities.METHOD We analysed 461 minutes of audio-video recordings containing play situations and the daily routines of six females (aged 7 to 24 months; mean birthweight 3057g, SD 195g) with the preserved speech variant (PSV) of RTT. All videos were recorded by parents and analysed retrospectively after the diagnosis PSV was made.RESULTS From the age of 7 months onwards, we observed two types of vocalizations, appearing intermittently: (1) apparently normal sequences; and (2) atypical (i.e. inhalatory, pressed, or high-pitched crying-like) vocalizations. Some participants failed to reach the milestone of canonical babbling. We observed a limited phonological and lexical complexity and a restricted compositional variability. Volubility was reduced during the whole period under observation. Hand stereotypies with simultaneous atypical vocalizations appeared only during the second year of life. INTERPRETATIONThe intermittent character of normal versus abnormal verbal behaviours might contribute to an early identification of children with a possible genetic mutation, and provides evidence that speech-language functions are abnormal from the very beginning.Rett syndrome (RTT, MIM 312750), a profoundly disabling neurodevelopmental disorder that predominantly occurs in females, is mainly caused by mutations in the gene MECP2 for the methyl-CpG-binding protein 2 (Xq28).1 It is assumed that MeCP2, a regulator of neuronal activity-dependent synaptic maturation, plays a central role in postnatal brain development. Disruption of MeCP2 affects a wide range of neurodevelopmental functions such as cognitive processes, purposeful hand use, and communicative abilities.2 The pathogenesis of RTT is characterized by a four-stage trajectory, the second of which is the regression period, in which the clinical signs become more prominent.1 The trajectory can be observed in females with classic RTT as well as in other variants of RTT, of which the so-called preserved speech variant (PSV) or Zappella variant (Z-RTT) has a more benign overall pathogenesis, including better manual and speech-language abilities. 1,3,4 Females with PSV have the same staging and a number of the same symptoms as in classic RTT (e.g. the characteristic hand stereotypies) but usually show no general growth failure or deceleration of head growth; epilepsy and hyperventilation are rare.3-5 Furthermore, individuals with PSV show a postregressional improvement in hand use; language abilities may be regained or preserved. Lexicon size and syntactic complexity are reported to increase slowly, but are usually accompanied by features such as e...

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“…feeding and play skills). Eye gaze technology is commonly used by girls with RTT to promote communication; and research continues to investigate use of eye gaze to assess cognition, communication, and visual processing in this population [3][4][5][6]. Evidence to support the efficacy or proper dosing of these interventions individually or in combination is limited at best.…”

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confidence: 99%

Improving Upper Extremity Motor Skills in Girls with Rett Syndrome Using Virtual Reality

Mraz

Amadio

Diener

et al. 2016

J. Intellect. Disabl. Diagn. Treat.

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Introduction: Rett Syndrome is a genetic disorder that limits a girl's ability to use her upper extremities for daily activities, such as dressing and playing. One possible intervention to improve upper extremity function in this population is virtual reality, which can be used to increase activity demands during therapy sessions.Objectives: To determine the feasibility of using internet-based virtual reality intervention for Rett Syndrome (RTT-IVR), to decrease hand wringing/mouthing and increase hand and arm movements away from the midline by identifying attributes and limitations to the proposed intervention. Materials and Methods:Using FAAST Software and Microsoft Kinect sensor, RTT-IVR was trialed with 6 girls with RTT. Upper extremity movements were used to play free Internet games as means of increasing repetitions and purposeful arm movements. Data regarding attributes and limitations of the RTT-IVR intervention were collected via observation and post-session parent interviews.Results: Interviews and observation revealed successful game play when games were motivating, clearly established cause and effect, and matched level of cognitive ability of the participant. Limitations include technological glitches regarding Kinect sensor sensitivity and identifying appropriate games for each participant's interests and abilities.Conclusion: Internet based virtual reality interventions for girls with RTT should be highly individualized to increase motivation and success of intervention.

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Communication assessment for individuals with Rett syndrome: A systematic review

Cited by 44 publications

References 19 publications

Functional Communication Training in Rett Syndrome: A Preliminary Study

Functional Communication Training in Rett Syndrome: A Preliminary Study

Early speech–language development in females with Rett syndrome: focusing on the preserved speech variant

Improving Upper Extremity Motor Skills in Girls with Rett Syndrome Using Virtual Reality

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