Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease: a comparison of assessment instruments
Por:
Horta-Barba, A, Martinez-Horta, S, Perez-Perez, J, Puig-Davi, A, de Lucia, N, de Michele, G, Salvatore, E, Kehrer, S, Priller, J, Migliore, S, Squitieri, F, Castaldo, A, Mariotti, C, Mananes, V, Lopez-Sendon, JL, Rodriguez, N, Martinez-Descals, A, Julio, F, Januaario, C, Delussi, M, de Tommaso, M, Noguera, S, Ruiz-Idiago, J, Sitek, EJ, Wallner, R, Nuzzi, A, Pagonabarraga, J, Kulisevsky, J
Publicada:
18 jul 2023
Ahead of Print:
1 jul 2023
Resumen:
BackgroundProgressive cognitive decline is an inevitable feature of Huntington's disease (HD) but specific criteria and instruments are still insufficiently developed to reliably classify patients into categories of cognitive severity and to monitor the progression of cognitive impairment.MethodsWe 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.ResultsThe 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.ConclusionThe 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.
Filiaciones:
Horta-Barba, A:
Autonomous Univ Barcelona UAB, Dept Med, Bellaterra, Spain
Hosp Santa Creu i St Pau, Neurol Dept, Movement Disorders Unit, Barcelona, Spain
St Pau Biomed Res Inst IIB St Pau, Barcelona, Spain
Ctr Invest Red Enfermedades Neurodegenerat CIBERNE, Madrid, Spain
European Huntingtons Dis Network EHDN, Ulm, Germany
Martinez-Horta, S:
Autonomous Univ Barcelona UAB, Dept Med, Bellaterra, Spain
Hosp Santa Creu i St Pau, Neurol Dept, Movement Disorders Unit, Barcelona, Spain
St Pau Biomed Res Inst IIB St Pau, Barcelona, Spain
Ctr Invest Red Enfermedades Neurodegenerat CIBERNE, Madrid, Spain
European Huntingtons Dis Network EHDN, Ulm, Germany
Perez-Perez, J:
Autonomous Univ Barcelona UAB, Dept Med, Bellaterra, Spain
Hosp Santa Creu i St Pau, Neurol Dept, Movement Disorders Unit, Barcelona, Spain
St Pau Biomed Res Inst IIB St Pau, Barcelona, Spain
Ctr Invest Red Enfermedades Neurodegenerat CIBERNE, Madrid, Spain
European Huntingtons Dis Network EHDN, Ulm, Germany
Puig-Davi, A:
Hosp Santa Creu i St Pau, Neurol Dept, Movement Disorders Unit, Barcelona, Spain
St Pau Biomed Res Inst IIB St Pau, Barcelona, Spain
Ctr Invest Red Enfermedades Neurodegenerat CIBERNE, Madrid, Spain
European Huntingtons Dis Network EHDN, Ulm, Germany
de Lucia, N:
European Huntingtons Dis Network EHDN, Ulm, Germany
Univ Naples Federico II, Naples, Italy
de Michele, G:
European Huntingtons Dis Network EHDN, Ulm, Germany
Univ Naples Federico II, Naples, Italy
Salvatore, E:
European Huntingtons Dis Network EHDN, Ulm, Germany
Univ Naples Federico II, Naples, Italy
Kehrer, S:
European Huntingtons Dis Network EHDN, Ulm, Germany
Charite, Dept Neuropsychiat, Berlin, Germany
Priller, J:
European Huntingtons Dis Network EHDN, Ulm, Germany
Charite, Dept Neuropsychiat, Berlin, Germany
Migliore, S:
Fdn IRCCS Casa Sollievo Sofferenza Res Hosp, Huntington & Rare Dis Unit, San Giovanni Rotondo, Italy
Squitieri, F:
Fdn IRCCS Casa Sollievo Sofferenza Res Hosp, Huntington & Rare Dis Unit, San Giovanni Rotondo, Italy
Castaldo, A:
European Huntingtons Dis Network EHDN, Ulm, Germany
Fdn IRCCS Ist Neurol Carlo Besta, Milan, Italy
Mariotti, C:
European Huntingtons Dis Network EHDN, Ulm, Germany
Fdn IRCCS Ist Neurol Carlo Besta, Milan, Italy
Mananes, V:
European Huntingtons Dis Network EHDN, Ulm, Germany
Hosp Univ Ramon y Cajal, Dept Neurol, Madrid, Spain
Lopez-Sendon, JL:
European Huntingtons Dis Network EHDN, Ulm, Germany
Hosp Univ Ramon y Cajal, Dept Neurol, Madrid, Spain
Rodriguez, N:
European Huntingtons Dis Network EHDN, Ulm, Germany
Fdn Jimenez Diaz, Dept Neurol, Madrid, Spain
Martinez-Descals, A:
European Huntingtons Dis Network EHDN, Ulm, Germany
Fdn Jimenez Diaz, Dept Neurol, Madrid, Spain
Julio, F:
European Huntingtons Dis Network EHDN, Ulm, Germany
Univ Coimbra, Coimbra Inst Biomed Imaging & Translat Res CIBIT, Coimbra, Portugal
Coimbra Univ Hosp, Neurol Dept, Coimbra, Portugal
Januaario, C:
European Huntingtons Dis Network EHDN, Ulm, Germany
Univ Coimbra, Coimbra Inst Biomed Imaging & Translat Res CIBIT, Coimbra, Portugal
Coimbra Univ Hosp, Neurol Dept, Coimbra, Portugal
Delussi, M:
European Huntingtons Dis Network EHDN, Ulm, Germany
Aldo Moro Univ, Apulian Ctr Huntingtons Dis SMBNOS Dept, Appl Neurophysiol & Pain Unit, Bari, Italy
de Tommaso, M:
European Huntingtons Dis Network EHDN, Ulm, Germany
Aldo Moro Univ, Apulian Ctr Huntingtons Dis SMBNOS Dept, Appl Neurophysiol & Pain Unit, Bari, Italy
Noguera, S:
European Huntingtons Dis Network EHDN, Ulm, Germany
Hosp Mare Deu Merce, Barcelona, Spain
Ruiz-Idiago, J:
European Huntingtons Dis Network EHDN, Ulm, Germany
Hosp Mare Deu Merce, Barcelona, Spain
Sitek, EJ:
European Huntingtons Dis Network EHDN, Ulm, Germany
Univ Gdansk, Fac Hlth Sci Med, Dept Neurol & Psychiat Nursing, Gdansk, Poland
St Adalbert Hosp, Dept Neurol, Gdansk, Poland
Wallner, R:
Med Univ Wroclaw, Dept Psychiat, Wroclaw, Poland
Nuzzi, A:
European Huntingtons Dis Network EHDN, Ulm, Germany
Pagonabarraga, J:
Autonomous Univ Barcelona UAB, Dept Med, Bellaterra, Spain
Hosp Santa Creu i St Pau, Neurol Dept, Movement Disorders Unit, Barcelona, Spain
St Pau Biomed Res Inst IIB St Pau, Barcelona, Spain
Ctr Invest Red Enfermedades Neurodegenerat CIBERNE, Madrid, Spain
European Huntingtons Dis Network EHDN, Ulm, Germany
Kulisevsky, J:
Autonomous Univ Barcelona UAB, Dept Med, Bellaterra, Spain
Hosp Santa Creu i St Pau, Neurol Dept, Movement Disorders Unit, Barcelona, Spain
St Pau Biomed Res Inst IIB St Pau, Barcelona, Spain
Ctr Invest Red Enfermedades Neurodegenerat CIBERNE, Madrid, Spain
European Huntingtons Dis Network EHDN, Ulm, Germany
hybrid, All Open Access, Hybrid Gold
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