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Unimpaired Neuropsychological Performance and Enhanced Memory Recall in Patients with Sbma: A Large Sample
S Marcato1, J R Kleinbub2, G Querin1
1Department of Neurosciences (DNS), University of Padova Via Giustiniani, 2 - 35128, Padova, Italy.
Scientific Reports
|September 13, 2018
Summary
Spinal and bulbar muscular atrophy (SBMA) patients showed no cognitive deficits in standard tests. However, they exhibited enhanced performance in prose memory, possibly linked to testosterone or mood, warranting further investigation.
Area of Science:
- Neuroscience
- Genetics
- Neurology
Background:
- Fragmented literature describes a peculiar cognitive profile in Spinal and Bulbar Muscular Atrophy (SBMA).
- Understanding SBMA's cognitive impact requires comprehensive neuropsychological evaluation.
- Previous studies on SBMA cognition have yielded inconsistent results.
Purpose of the Study:
- To investigate the cognitive functions in a large cohort of SBMA patients.
- To identify potential correlations between cognitive performance and SBMA's genetic and hormonal factors.
- To contribute to a clearer understanding of the SBMA cognitive profile.
Main Methods:
- Retrospective analysis of 64 neuropsychological evaluations from SBMA patients (2013-2015).
- Assessment of mnesic, linguistic, and executive functions using Digit Span, Prose Memory, Verbal Fluency, and Trail Making Tests.
- Statistical comparison with healthy controls using ANCOVA and regression analyses for genetic and hormonal factors.
Main Results:
- No significant cognitive deficits were found in SBMA patients across the adopted neuropsychological tests.
- SBMA patients demonstrated significantly enhanced performance in the Prose Memory test.
- No significant associations were identified between cognitive performance and CAG-repeat expansion, androgen insensitivity, or clinical profiles.
Conclusions:
- SBMA patients do not exhibit widespread cognitive impairments on standard assessments.
- Enhanced prose memory performance in SBMA may be influenced by factors like somatic mosaicism, testosterone levels, or mood state.
- Further research with detailed test batteries is recommended to elucidate SBMA's cognitive nuances.
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