Measures of attention in Rett syndrome: Internal consistency reliability

Area of Science:

• Neuroscience
• Developmental Psychology
• Clinical Psychology

Background:

• Rett syndrome (RTT) is an X-linked neurodevelopmental disorder caused by MECP2 gene mutations.
• RTT is characterized by severe impairments in expressive language and purposeful hand use.
• Gaze-based tasks offer a method to assess cognitive functions bypassing motor limitations in RTT.

Purpose of the Study:

• To estimate the internal consistency reliability of three key attention aspects in children with RTT.
• To evaluate the suitability of gaze-based tasks for assessing attention in RTT for clinical trials.

Main Methods:

• Sixty-six children with RTT (ages 2-19) participated.
• Assessed Sustained Attention (butterfly task), Disengaging/Shifting Attention (gap/overlap task), and Selective Attention (search task) using gaze-tracking.
• Analyzed reliability using split-half coefficients and Cronbach alphas.

Main Results:

• Acceptable to excellent reliability (0.70-0.93) was found for Sustained and Disengaging/Shifting Attention measures.
• Reliability improved with aggregated trials, with acceptable levels often achieved using 6-9 trials.
• Selective Attention measures demonstrated limited reliability.

Conclusions:

• Gaze-based tasks reliably assess critical aspects of attention in RTT.
• This represents a significant advancement for developing cognitive measures in RTT clinical and translational research.