Objective: We aimed to assess the ability of Brain Network Activation (BNA) EEG analysis to differentiate between patients with fragile X-associated tremor/ataxia syndrome (FXTAS), Parkinson’s disease (PD) patients and healthy individuals and to study association of these BNA scores with the degree of cognitive impairment, motor dysfunction and FMR1 CGG repeat size.

Background: FXTAS is a late-onset neurodegenerative disorder affecting carriers of a premutation in the FMR1 gene. BNA EEG analysis algorithm is a non-invasive technology developed by ElMindA, which is a new imaging approach that can detect changes in brain activity and functional connectivity. BNA combines EEG-technology with big-data analytics and machine learning, utilizing state-of-the-art algorithms, such as digital-signal processing, graph theory, clustering analysis, and pattern recognition. Such brain patterns offer the important promise of identifying neuromarkers that may serve as useful objective diagnostic and monitoring tools for brain disorders.

Methods: Up to now 6 patients were recruited (5 men, age: 75.0± 5.4 years, symptom duration: 10.4 ± 6.0 years) and passed the Neurotrax Computerized Cognitive Battery and were rated according to the FXTAS scale. The patients performed the cognitive visual Go/NoGo task with EEG recording and BNA results were obtained and compared to those of 32 PD patients (12 early PD) and 10 healthy age-matched controls (HC).

Results: The BNA data analyzed represented 2 different cognitive-motor processes- motor response and response inhibition, which were proven as relevant to PD in previous analyses. FXTAS subjects showed substantially different brain activation from HC in both response inhibition and motor potential, as opposed to PD patients who are markedly different from HC only in response inhibition. Positive correlations were found between NoGo BNA score and executive index Go and NoGo BNA score and age. No other correlations were found.

Conclusions: These preliminary results suggest that BNA EEG analysis may assist in differentiating between FXTAS, PD and HC, as FXTAS involves more extensive network disruption related to the diffuse neuropathology. Further research should follow with longitudinal BNA EEG analyses in asymptomatic and symptomatic FMR1 premutation carriers in order to improve our understanding of the basic BNA disruption and to study the potential of BNA as a neurophysiological biomarker for FXTAS.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/changes-in-brain-network-activation-bna-in-patients-with-fragile-x-associated-tremor-ataxia-syndrome-fxtas/