A new technique based on video-recorded behavior analysis can help assess communication ability in children with Angelman syndrome and may be used in future clinical studies of experimental therapies, according to researchers.
Their study, “Quantitative Measurement of Communication Ability in Children with Angelman Syndrome,” was published in the Journal of Applied Research in Intellectual Disabilities.
Angelman syndrome is a genetic disorder characterized by severe cognitive impairments, including speech problems. Most Angelman syndrome patients do not develop common speech tools and suffer from delays in other forms of communication skills, such as signs and gestures.
The inability to recognize communication patterns in these patients can have a negative impact on the assessment of treatment effectiveness and progression. So, finding ways to effectively evaluate communication in Angelman children is an important step toward better outcomes.
University of South Florida researchers developed a new method to improve the assessment and quantification of verbal and nonverbal communication ability in kids with Angelman syndrome.
The researchers tested their approach in nine children ages 34 months (nearly 3 years old) to 126 months (10.5 years old) with a confirmed diagnosis of Angelman syndrome, and in a single healthy 16-month-old toddler (the control child).
To quantify communication behavior, a speech-language pathologist engaged in a single 30-minute play session to elicit vocalization and nonverbal communication attempts from each study participant, using a LENA Pro recording device worn by the child in a special T-shirt.
The recorded sounds were than transcribed and categorized according to the Stark Assessment of Early Vocal Development-Revised. The childrens’ gestures were also classified according to function, behavioral regulation, or social interaction.
Researchers found that none of the Angelman children used complex forms of vocalization, and the sounds they made were similar to those of children between 0 and 6 months old. The healthy child used as a matching control used more advanced levels of vocalization.
A more detailed audio analysis revealed that in Angelman children, most vocalizations were either laughter or isolated vowels, with only three children producing combinations of consonants and vowels.
These children frequently used vowels that required little tongue movement, and they mainly kept their tongue in a central position in their mouth, which could be related to impaired control of speech-associated movement.
An evaluation of physical movements and signs used to communicate indicated that the most-used gestures were to protest or point. However, Angelman children spent almost twice the amount of time not gesturing compared to the healthy toddler.
These results demonstrate that children with Angelman syndrome tend to use more primitive forms of communication that are likely related to neurological impairment comprising “deficits in receptive and expressive language,” according to researchers.
“Angelman syndrome researchers agree the neuropsychological outcome measures that exist currently do not accurately measure the cognition, communication, or motor ability of a child with AS,” researchers wrote.
“The results of this study show that not only can the present authors capture, quantify and analyze the communication ability of patients with Angelman syndrome, but it can be done with specificity. This means the use of this assessment will detect even the smallest amount of change in communication ability or oral motor function during clinical studies of new therapeutics for this devastating disorder,” they wrote.