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A neurophysiological model of speech production deficits in fragile X syndrome.

ABSTRACT: Fragile X syndrome is the most common inherited intellectual disability and monogenic cause of autism spectrum disorder. Expressive language deficits, especially in speech production, are nearly ubiquitous among individuals with fragile X, but understanding of the neurological bases for these deficits remains limited. Speech production depends on feedforward control and the synchronization of neural oscillations between speech-related areas of frontal cortex and auditory areas of temporal cortex. Interaction in this circuitry allows the corollary discharge of intended speech generated from an efference copy of speech commands to be compared against actual speech sounds, which is critical for making adaptive adjustments to optimize future speech. We aimed to determine whether alterations in coherence between frontal and temporal cortices prior to speech production are present in individuals with fragile X and whether they relate to expressive language dysfunction. Twenty-one participants with full-mutation fragile X syndrome (aged 7-55 years, eight females) and 20 healthy controls (matched on age and sex) completed a talk/listen paradigm during high-density EEG recordings. During the talk task, participants repeated pronounced short vocalizations of 'Ah' every 1-2 s for a total of 180 s. During the listen task, participants passively listened to their recordings from the talk task. We compared pre-speech event-related potential activity, N1 suppression to speech sounds, single trial gamma power and fronto-temporal coherence between groups during these tasks and examined their relation to performance during a naturalistic language task. Prior to speech production, fragile X participants showed reduced pre-speech negativity, reduced fronto-temporal connectivity and greater frontal gamma power compared to controls. N1 suppression during self-generated speech did not differ between groups. Reduced pre-speech activity and increased frontal gamma power prior to speech production were related to less intelligible speech as well as broader social communication deficits in fragile X syndrome. Our findings indicate that coordinated pre-speech activity between frontal and temporal cortices is disrupted in individuals with fragile X in a clinically relevant way and represents a mechanism contributing to prominent speech production problems in the disorder.

SUBMITTER: Schmitt LM

PROVIDER: S-EPMC7425415 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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A neurophysiological model of speech production deficits in fragile X syndrome.

Schmitt Lauren M LM Wang Jun J Pedapati Ernest V EV Thurman Angela John AJ Abbeduto Leonard L Erickson Craig A CA Sweeney John A JA

Brain communications 20191209 1

Fragile X syndrome is the most common inherited intellectual disability and monogenic cause of autism spectrum disorder. Expressive language deficits, especially in speech production, are nearly ubiquitous among individuals with fragile X, but understanding of the neurological bases for these deficits remains limited. Speech production depends on feedforward control and the synchronization of neural oscillations between speech-related areas of frontal cortex and auditory areas of temporal cortex ...[more]

PMID: 32924010

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Project description:Deficits in fluent speech production following left hemisphere stroke are a central concern because of their impact on patients' lives and the insight they provide about the neural organization of language processing. Fluent speech production requires the rapid coordination of phonological, semantic, and syntactic processing, so this study examined how deficits in connected speech relate to these language sub-systems. Behavioural data (N = 69 participants with aphasia following left hemisphere stroke) consisted of a diverse and comprehensive set of narrative speech production measures and measures of overall severity, semantic deficits, and phonological deficits. These measures were entered into a principal component analysis with bifactor rotation-a latent structure model where each item loads on a general factor that reflects what is common among the items, and orthogonal factors that explain variance not accounted for by the general factor. Lesion data were available for 58 of the participants, and each factor score was analysed with multivariate lesion-symptom mapping. Effects of connectivity disruption were evaluated using robust regression with tract disconnection or graph theoretic measures of connectivity as predictors. The principal component analysis produced a four-factor solution that accounted for 70.6% of the variance in the data, with a general factor corresponding to the overall severity and length and complexity of speech output (complexity factor), a lexical syntax factor, and independent factors for Semantics and Phonology. Deficits in the complexity of speech output were associated with a large temporo-parietal region, similar to overall aphasia severity. The lexical syntax factor was associated with damage in a relatively small set of fronto-parietal regions which may reflect the recruitment of control systems to support retrieval and correct usage of lexical items that primarily serve a syntactic rather than semantic function. Tract-based measures of connectivity disruption were not statistically associated with the deficit scores after controlling for overall lesion volume. Language network efficiency and average clustering coefficient within the language network were significantly associated with deficit scores after controlling for overall lesion volume. These results highlight overall severity as the critical contributor to fluent speech in post-stroke aphasia, with a dissociable factor corresponding to lexical syntax.

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A neurophysiological model of speech production deficits in fragile X syndrome.

Publications

A neurophysiological model of speech production deficits in fragile X syndrome.

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