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Use of the Behavioral Dyscontrol Scale-2 (BDS-2) to measure executive dysfunction in FXTAS
Seyedeh Ala Mokhtabad Amrei1,2, Hasan Hasan1,3, Ellery Santos1,4
1UC Davis MIND Institute, UC Davis Health, Sacramento, CA, United States.
Background/Objective:
Fragile X-associated Tremor/Ataxia Syndrome (FXTAS) is a neurodegenerative disorder caused by a premutation (55 to 200 CGG repeats) in the FMR1 gene. This expansion leads to mRNA toxicity, resulting in the deterioration of executive and cognitive abilities alongside characteristic brain changes detected via Magnetic Resonance Imaging (MRI). While the Behavioral Dyscontrol Scale-2 (BDS-2) is commonly used to evaluate executive function, its specific relationship with neuroanatomical markers in FXTAS has not been fully examined. This study investigated the association between BDS-2 and MRI scores in FXTAS.
Methods:
Correlation and linear regression analyses were conducted to evaluate how BDS-2 scores correlate with MRI total scores and other neurodegeneration variables, measured by white matter hyperintensities, relative to the MMSE and CANTAB subtests in 56 FMR1 premutation carriers with and without FXTAS.
Results:
MRI scores were negatively correlated with BDS-2 total scores (Pearson's r = -0.66, p < 0.0001). The association between BDS-2 total scores and MRI scores was still significant after accounting for CGG repeat length, sex, and age as covariates (β = -0.36, p = 0.002). In the complete-case sample analysis (n = 30), the executive functioning subtests of the CANTAB were positively correlated with the MRI total score (ρ = 0.46-0.61, p < 0.05), while the MMSE demonstrated a negative correlation (ρ = -0.40, p = 0.03).
Conclusion:
These findings indicate that BDS-2 can be used alongside other instruments for measuring executive dysfunction in FXTAS, demonstrating the potential for clinical utility to complement traditional cognitive and automated executive measures.
Insights
The Behavioral Dyscontrol Scale-2 (BDS-2) effectively measures executive dysfunction in Fragile X-associated Tremor/Ataxia Syndrome (FXTAS). This scale shows a significant association with neuroimaging markers, complementing other cognitive assessments.
Area of Science:
- Neuroscience
- Genetics
- Radiology
Background:
- Fragile X-associated Tremor/Ataxia Syndrome (FXTAS) is a neurodegenerative disorder linked to the FMR1 gene premutation.
- FXTAS is characterized by mRNA toxicity, leading to cognitive decline and distinct brain changes visible on MRI.
- The Behavioral Dyscontrol Scale-2 (BDS-2) assesses executive function, but its correlation with neuroanatomical changes in FXTAS requires further investigation.
Purpose of the Study:
- To investigate the relationship between scores on the Behavioral Dyscontrol Scale-2 (BDS-2) and Magnetic Resonance Imaging (MRI) findings in individuals with FXTAS.
- To determine if BDS-2 scores correlate with neuroanatomical markers of neurodegeneration in FXTAS patients.
Main Methods:
- Correlation and linear regression analyses were employed.
- The study included 56 FMR1 premutation carriers, with and without FXTAS.
- BDS-2 scores were compared with MRI total scores, white matter hyperintensities, MMSE, and CANTAB subtests.
Main Results:
- MRI scores showed a significant negative correlation with BDS-2 total scores (r = -0.66, p < 0.0001).
- This association remained significant after controlling for CGG repeat length, sex, and age (β = -0.36, p = 0.002).
- Executive function subtests of the CANTAB positively correlated with MRI scores, while MMSE showed a negative correlation.
Conclusions:
- The BDS-2 is a valuable tool for assessing executive dysfunction in FXTAS, demonstrating a clear link with neuroimaging markers.
- Findings support the clinical utility of BDS-2 as a complementary measure alongside traditional cognitive and automated executive function tests in FXTAS management.
- The study highlights the importance of integrating behavioral assessments with neuroimaging for a comprehensive understanding of FXTAS.
