Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease: a comparison of assessment instruments.
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Horta-Barba Andrea, Martinez-Horta Saul, Pérez-Pérez Jesús, Puig-Davi Arnau, de Lucia Natascia, de Michele Giuseppe, Salvatore Elena, Kehrer Stefanie, Priller Josef, Migliore Simone, Squitieri Ferdinando, Castaldo Anna, Mariotti Caterina, Mañanes Veronica, Lopez-Sendon Jose Luis, Rodriguez Noelia, Martinez-Descals Asunción, Júlio Filipa, Januário Cristina, Delussi Marianna, de Tommaso Marina, Noguera Sandra, Ruiz-Idiago Jesús, Sitek Emilia J, Wallner Renata, Nuzzi Angela, Pagonabarraga Javier, Kulisevsky Jaime, Cognitive Phenotype Working Group of the European Huntington’s Disease Network
Аннотация
METHODS: We collected data from a cohort of 180 positive gene-carriers: 33 with premanifest HD and 147 with manifest HD. Using a specifically developed gold-standard for cognitive status we classified participants into those with normal cognition, those with mild cognitive impairment, and those with dementia. We administered the Parkinson's Disease-Cognitive Rating Scale (PD-CRS), the MMSE and the UHDRS cogscore at baseline, and at 6-month and 12-month follow-up visits. Cutoff scores discriminating between the three cognitive categories were calculated for each instrument. For each cognitive group and instrument we addressed cognitive progression, sensitivity to change, and the minimally clinical important difference corresponding to conversion from one category to another.
RESULTS: The PD-CRS cutoff scores for MCI and dementia showed excellent sensitivity and specificity ratios that were not achieved with the other instruments. Throughout follow-up, in all cognitive groups, PD-CRS captured the rate of conversion from one cognitive category to another and also the different patterns in terms of cognitive trajectories.
CONCLUSION: The PD-CRS is a valid and reliable instrument to capture MCI and dementia syndromes in HD. It captures the different trajectories of cognitive progression as a function of cognitive status and shows sensitivity to change in MCI and dementia.