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F041 When the invisible becomes visible: the cognitive composite score may predict HD severity in prodromal populations at 10-year follow-up

Year: 2024
Type: conference-abstract
Abstract: Background We generated a Cognitive Composite Score (CCS) using the ENROLL-HD Periodic Data Set (PDS#5). CCS was obtained by a formula combining four cognitive measures (SDMT, FVT, SCN, SWR) for three groups: low, average, and high cognitive performance (LCP, ACP, HCP, respectively). It is potentially predictive of motor severity in the prodromal population. Aims Validation of the CCS in a prodromal patient cohort of Italian origin across a 10-year observation period (2014–2024). Methods Out of the 80 HD participants who phenoconverted from prodromal to manifest HD, 45 were followed up annually for at least 3 years (3.28 ± 0.77). Clinical assays were completed at the prodromal (T1) and post-phenoconversion HD stages (T2 to T4). We obtained data using the CCS, Total Motor Score (TMS) and Problem Behavioral Assessment Short Form (PBA-s) at each time point. Results Correlation analyses confirmed the significant association between lower cognitive performance as measured by CCS and poorer post-phenoconversion motor performance (T2: r = -0.41, p < 0.000; T3: r = -0.45, p < 0.000; T4: r = -0.47, p = 0.001). Prodromal CCS was associated with a significantly higher TMS score in the LCP group than in the ACP and HCP groups at T3 and T4 (ANOVA repeated measures, p < 0.05). We also observed a significant negative correlation between CCS and PBA-s apathy score at T4 (r = -0.40, p = 0.007), with LCP showing higher, time-independent, apathy scores than the ACP and HCP groups (ANOVA repeated measures, p < 0.05). Conclusions CCS may potentially be used to predict the severity of clinical manifestations in cohorts of prodromal HD participants – the phase in which HD is still invisible.
DOI: 10.1136/jnnp-2024-ehdn.159
Language: English
Open Access status: gold
OpenAlex ID: W4402374425
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