Typical and atypical neural mechanisms support spoken word processing in Angelman syndrome

Abstract

Angelman syndrome (AS) is known to affect expressive and receptive communication abilities. This study examined individual differences in neural mechanisms underlying speech processing in children with AS (n = 24, M age = 10.01 years) and typical development (n = 30, M age = 10.82 years) using auditory event-related potentials during passive listening to common English words and novel pseudowords. A group of adults with AS (n = 7, M = 31.78 years) provided data about the upper developmental range. The typically developing group demonstrated the expected more negative amplitudes in response to words than pseudowords within 250-500 ms after stimulus onset at the left temporal scalp region. Children and adults with AS exhibited a similar left-lateralized pattern of word-pseudoword differentiation at temporal and parietal regions, but not the midline parietal memory response for known words observed in the typically developing group, suggesting typical-like word-pseudoword differentiation along with possible alterations in the automatic recall of word meaning. These results have important implications for understanding receptive and expressive communication processes in AS and support the use of auditory neural responses for characterizing individual differences in neurodevelopmental disorders with limited speech.

Keywords: Angelman syndrome; Auditory; ERP; Receptive; Speech; Word.

Figure 1.

Electrode layout of the 128-channel geodesic net (EGI, Inc.) and the left and right temporal and parietal clusters used in data analysis.

Figure 2.

Averaged left and right parietal and temporal ERP waveforms for word and pseudoword conditions in typically developing children and participants with AS. Rectangle marks the 250–500 ms interval used in the analyses.

Figure 3.

Averaged midline parietal ERP waveforms for word and pseudoword conditions in typically developing children and participants with AS. Rectangle marks the 250–500 ms interval used in the analyses.