Session Information
Date: Monday, September 23, 2019
Session Title: Ataxia
Session Time: 1:45pm-3:15pm
Location: Les Muses, Level 3
Objective: Evaluate prospectively cognitive dysfunction in subjects with SCA2 using Mini Mental Examination (MMSE) and Montreal Cognition Assesment (MOCA), looking for correlation between cognitiion and motor disability, clinical variables and potentially modifier genes.
Background: Cognitive deficits in patients with cerebellar degeneration, including SCA2 have been shown. Studies on SCA2 did not find a clear change in cognitive scores in longitudinal observations. Although we have previously showed a potential association between mitochondrial polymorphism A1098G and cognitive dysfunction, little is known about factors that interfere with cognitive impairment in patients with SCA2.
Method: Symptomatic subjects with SCA2 were submitted to MMSE and MOCA; Beck Depression Inventory; motor scales: Scale for Assessment and Rating of Ataxia (SARA), and Neurologic Examination Score for Spinocerebellar Ataxia (NESSCA), SCA Functional-Index (SCAFI), and Composite-Cerebellar-Functional-Score (CCFS) at baseline and 12 months of follow up. Clinical variables, CAG repeat length at ATXN2 as well as at ATXN1, ATXN3, ATXN7, CACNA1A and RAI1 were explored as modifiers factors.
Results: 49 SCA2 patients were studied . 78% of patients are in the normal range of MMSE, but 75% presented reduced MOCA scores. At baseline, all motor scales showed correlation with MMSE and MOCA ( p<0.01), while only MOCA correlated with disease duration and CAG expanded repeat at ATXN2( p 0.05). After one year, SARA and NESSCA progressed on average 1.75(CI 95% 0.92-2.57) 1.45 (CI 95% 0.74-2.16) , respectively. In contrast, MMSE and MOCA scores were apparently stable - deltas being -0.45 ( -1.71 - 0.29) and -0.42 (-1.56-0.73) points (p=0.22 and 0.45), respectively. Delta MMSE were inversely correlated with the CAGexp at ATXN2 (rho=-0.36, p< 0.05). The other risk factors (age, CAG repeat numbers at genes of interest) did not correlate with deltas of MOCA and of MMSE.
Conclusion: Executive, memory, language and visuospatial dysfunctions, detected by MOCA, are quite common in SCA2. Disease duration correlated with MOCA, and motor scores correlated with MMSE and MOCA. Cognitive scores did not change after one year. Results support the suggestion that speed of progression of motor and cognitive dysfunctions are dissociated. On exploratory analysis, carriers of larger expansions presented worse deltas on MMSE. Future clinical trials should consider these differences in their outcomes .
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To cite this abstract in AMA style:
T. Monte, E. Reckziegel, M. Augustin, O. Barsottini, JL. Pedroso, F. Vargas, ML. Saraiva-Pereira, V. Leotti, L. Jardim. Prospective study of cognition in SCA2 [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/prospective-study-of-cognition-in-sca2/. Accessed September 7, 2024.« Back to 2019 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/prospective-study-of-cognition-in-sca2/