Behaviour State Analysis in Rett Syndrome: Continuous data reliability measurement

Woodyatt, Gail; Marinac, Julie V.; Darnell, Ross; Sigafoos, Jeff; Halle, James W.

doi:10.1080/1034912042000295035

Cited by 17 publications

(7 citation statements)

References 3 publications

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“…Quality of life measures such as the Child Health Questionnaire-P50 have also been used [ 86 ]. Others have attempted to use direct observation to recognize the emotional state in patients with RTT [ 87 , 88 ]; however, the outcome of the emotional and behavioural state by observers is poorly defined with no consensus on agreement by observers and underscores the need for an individualised approach.…”

Section: Emotional Behavioural and Autonomic Dysregulation In Rttmentioning

confidence: 99%

Key issues in Rett syndrome: emotional, behavioural and autonomic dysregulation (EBAD) - a target for clinical trials

Singh

Santosh

2018

Orphanet J Rare Dis

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Complex neurodevelopmental disorders need multi-disciplinary treatment approaches for optimal care. The clinical effectiveness of treatments is limited in patients with rare genetic syndromes with multisystem morbidity. Emotional and behavioural dysregulation is common across many neurodevelopmental disorders. It can manifest in children across multiple diagnostic groups, including those on the autism spectrum and in rare genetic syndromes such as Rett Syndrome (RTT). There is, however a remarkable scarcity in the literature on the impact of the autonomic component on emotional and behavioural regulation in these disorders, and on the longer-term outcomes on disorder burden.RTT is a debilitating and often life-threatening disorder involving multiple overlapping physiological systems. Autonomic dysregulation otherwise known as dysautonomia is a cardinal feature of RTT characterised by an imbalance between the sympathetic and parasympathetic arms of the autonomic nervous system. Unlocking the autonomic component of emotional and behavioural dysregulation would be central in reducing the impairment seen in patients with RTT. In this vein, Emotional, Behavioural and Autonomic Dysregulation (EBAD) would be a useful construct to target for treatment which could mitigate burden and improve the quality of life of patients.RTT can be considered as a congenital dysautonomia and because EBAD can give rise to impairments occurring in multiple overlapping physiological systems, understanding these physiological responses arising out of EBAD would be a critical part to consider when planning treatment strategies and improving clinical outcomes in these patients. Biometric guided pharmacological and bio-feedback therapy for the behavioural and emotional aspects of the disorder offers an attracting perspective to manage EBAD in these patients. This can also allow for the stratification of patients into clinical trials and could ultimately help streamline the patient care pathway for optimal outcomes.The objectives of this review are to emphasise the key issues relating to the management of EBAD in patients with RTT, appraise clinical trials done in RTT from the perspective of autonomic physiology and to discuss the potential of EBAD as a target for clinical trials.

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Section: Emotional Behavioural and Autonomic Dysregulation In Rttmentioning

confidence: 99%

Key issues in Rett syndrome: emotional, behavioural and autonomic dysregulation (EBAD) - a target for clinical trials

Singh

Santosh

2018

Orphanet J Rare Dis

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“…Self-injurious behaviour has been added as a fourth alertness level in some studies (e.g. Green et al 1994;Mudford et al 1997;Guess et al 1999); however, other researchers have excluded this behaviour from the observation scales, because they expect self-injurious behaviour to occur at all alertness levels (Woodyatt et al 2004) or because they describe it as a form of communication instead of an alertness level (Siegel-Causey & Bashinski 1997). In professional practice, then, DSPs are faced with the complex task of linking particular expressions of alertness in individuals in the target population to one of these levels.…”

Section: Introductionmentioning

confidence: 99%

Initiation of activities and alertness in individuals with profound intellectual and multiple disabilities

Munde

Vlaskamp

2014

J intellect Disabil Res

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“…An observation that was described repeatedly was that an individual girl's performance could vary greatly from day to day. It has been observed that girls with RS may not be always sufficiently alert to engage in optimal communicative interactions (Woodyatt, Marinac, Darnell, Sigafoos, & Halle, 2004). Teachers are encouraged to allow time for their students with RS to achieve a sufficient state of alertness to participate fully in communicative interactions (Ryan et al, 2004).…”

Section: Discussionmentioning

confidence: 99%

Communication Skills in Girls With Rett Syndrome

Bartolotta

Zipp

Simpkins

et al. 2010

Focus Autism Other Dev Disabl

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Rett Syndrome (RS) is an X-linked, neurodevelopmental disorder that occurs primarily in females and causes significant impairment in cognition, motor control, and communication. Teachers and speech-language pathologists (SLPs) encounter girls with RS with increasing frequency as awareness of the disorder increases, yet the literature on clinical interventions with this population is limited. Parents, teachers, and SLPs were surveyed regarding the communication abilities of these girls. Respondents reported that girls with RS use multiple modalities to communicate, with eye gaze the most frequently used modality, followed by picture/symbol systems and body movements. This study provides clinical information for professionals working with girls with RS, and supports the need for additional research on strategies to improve communication in RS.

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Behaviour State Analysis in Rett Syndrome: Continuous data reliability measurement

Cited by 17 publications

References 3 publications

Key issues in Rett syndrome: emotional, behavioural and autonomic dysregulation (EBAD) - a target for clinical trials

Key issues in Rett syndrome: emotional, behavioural and autonomic dysregulation (EBAD) - a target for clinical trials

Initiation of activities and alertness in individuals with profound intellectual and multiple disabilities

Communication Skills in Girls With Rett Syndrome

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