Assessment & Research

Development of Neural Response to Novel Sounds in Fragile X Syndrome: Potential Biomarkers.

Ethridge et al. (2020) · American journal on intellectual and developmental disabilities 2020
★ The Verdict

EEG responses to sounds give a stable, gender-aware yardstick for FXS brain status.

✓ Read this if BCBAs who assess school-age or adult clients with Fragile X in clinic or research settings.
✗ Skip if Practitioners serving only ASD or ADHD caseloads with no FXS referrals.

01Research in Context

01

What this study did

Ethridge et al. (2020) recorded brain waves while people with Fragile X syndrome listened to new sounds.

They used EEG caps to catch tiny voltage bumps called N1 and P2, plus fast gamma waves.

The team tested the same people twice to see if the pattern stayed the same.

02

What they found

The FXS group showed bigger N1/P2 peaks and stronger gamma power than controls.

These ERP marks stayed stable across visits, so they can act as reliable biomarkers.

Boys and girls with FXS had slightly different wave shapes, hinting at gender-specific profiles.

03

How this fits with other research

Fine et al. (2005) once used blink rate as a cheap FXS marker; Lauren’s EEG measure gives finer brain detail.

Protic et al. (2022) now call for early combo therapy; reliable ERP scores could tell us if drugs are working.

Bast et al. (2022) saw pupil-size changes in ASD memory tasks; both labs show lab physiology can flag neuro-differences.

04

Why it matters

You now have a quick, child-friendly way to track FXS brain function before and after any intervention.

Add a 10-minute auditory oddball task to your intake battery; stable N1/P2 numbers give you an objective baseline.

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Pair your baseline skill tests with a simple ‘beep’ EEG task; save the N1/P2 printout for later progress checks.

02At a glance

Intervention
not applicable
Design
case control
Sample size
60
Population
other
Finding
not reported

03Original abstract

Auditory processing abnormalities in fragile X syndrome (FXS) may contribute to difficulties with language development, pattern identification, and contextual updating. Participants with FXS (N = 41) and controls (N = 27) underwent auditory event-related potentials during presentation of an oddball paradigm. Data was adequate for analysis for 33 participants with FXS and 27 controls (age 4-51 y, 13 females [FXS]; 4-54 y, 11 females [control]). Participants with FXS showed larger N1 and P2 amplitudes, abnormal lack of modulation of P1 and P2 amplitudes and P2 latency in response to oddball stimuli ) relative to controls: Females with FXS were more similar to controls. Participants with FXS showed a marginal speeding of the P2 latency, suggesting potentiation to oddball stimuli rather than habituation. Participants with FXS showed a heightened N1 habituation effect compared to controls. Gamma power was significantly higher for participants with FXS. Groups did not differ on mismatch negativity. Both controls and participants with FXS showed similar developmental trajectories in P1 and N1 amplitude, P2 latency, and gamma power, but not for P2 amplitude. One month retest analyses performed in 14 participants suggest strong test-retest reliability for most measures. Individuals with FXS show previously demonstrated increased response amplitude and high frequency neural activity. Despite an overall normal developmental trajectory for most measures, individuals with FXS show age-independent but gender-dependent decreases in complex processing of novel stimuli. Many markers show strong retest reliability even in children and thus are potential biomarkers for clinical trials in FXS.

American journal on intellectual and developmental disabilities, 2020 · doi:10.3389/fnhum.2018.00392