Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease: a comparison of assessment instruments

被引：3

作者：

Horta-Barba, Andrea ^{[1
,2
,3
,4
,5
]}

Martinez-Horta, Saul ^{[1
,2
,3
,4
,5
]}

Perez-Perez, Jesus ^{[1
,2
,3
,4
,5
]}

Puig-Davi, Arnau ^{[2
,3
,4
,5
]}

de Lucia, Natascia ^{[5
,6
]}

de Michele, Giuseppe ^{[5
,6
]}

Salvatore, Elena ^{[5
,6
]}

Kehrer, Stefanie ^{[5
,7
]}

Priller, Josef ^{[5
,7
]}

Migliore, Simone ^{[8
]}

Squitieri, Ferdinando ^{[8
]}

Castaldo, Anna ^{[5
,9
]}

Mariotti, Caterina ^{[5
,9
]}

Mananes, Veronica ^{[5
,10
]}

Lopez-Sendon, Jose Luis ^{[5
,10
]}

Rodriguez, Noelia ^{[5
,11
]}

Martinez-Descals, Asuncion ^{[5
,11
]}

Julio, Filipa ^{[5
,12
,13
]}

Januario, Cristina ^{[5
,12
,13
]}

Delussi, Marianna ^{[5
,14
]}

de Tommaso, Marina ^{[5
,14
]}

Noguera, Sandra ^{[5
,15
]}

Ruiz-Idiago, Jesus J. ^{[5
,15
]}

Sitek, Emilia ^{[5
,16
,17
]}

Wallner, Renata ^{[18
]}

Nuzzi, Angela ^{[5
]}

Pagonabarraga, Javier ^{[1
,2
,3
,4
,5
]}

Kulisevsky, Jaime ^{[1
,2
,3
,4
,5
]}

机构：

[1] Autonomous Univ Barcelona UAB, Dept Med, Bellaterra, Spain

[2] Hosp Santa Creu I St Pau, Neurol Dept, Movement Disorders Unit, Barcelona, Spain

[3] St Pau Biomed Res Inst IIB St Pau, Barcelona, Spain

[4] Ctr Invest Red Enfermedades Neurodegenerat CIBERNE, Madrid, Spain

[5] European Huntingtons Dis Network EHDN, Ulm, Germany

[6] Univ Naples Federico II, Naples, Italy

[7] Charite, Dept Neuropsychiat, Berlin, Germany

[8] Fdn IRCCS Casa Sollievo Sofferenza Res Hosp, Huntington & Rare Dis Unit, San Giovanni Rotondo, Italy

[9] Fdn IRCCS Ist Neurol Carlo Besta, Milan, Italy

[10] Hosp Univ Ramon Y Cajal, Dept Neurol, Madrid, Spain

[11] Fdn Jimenez Diaz, Dept Neurol, Madrid, Spain

[12] Univ Coimbra, Coimbra Inst Biomed Imaging & Translat Res CIBIT, Coimbra, Portugal

[13] Coimbra Univ Hosp, Neurol Dept, Coimbra, Portugal

[14] Aldo Moro Univ, Apulian Ctr Huntingtons Dis SMBNOS Dept, Appl Neurophysiol & Pain Unit, Bari, Italy

[15] Hosp Mare Deu Merce, Barcelona, Spain

[16] Univ Gdansk, Fac Hlth Sci Med, Dept Neurol & Psychiat Nursing, Gdansk, Poland

[17] St Adalbert Hosp, Dept Neurol, Gdansk, Poland

[18] Med Univ Wroclaw, Dept Psychiat, Wroclaw, Poland

来源：

JOURNAL OF NEUROLOGY | 2023年 / 270卷 / 11期

关键词：

Huntington's disease; Cognition; Neuropsychology; Disease progression; Mild cognitive impairment; Dementia; RATING-SCALE; PREMANIFEST; PROGRESSION; NEUROPSYCHOLOGY; MANIFEST;

D O I：

10.1007/s00415-023-11804-0

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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.

引用

页码：5408 / 5417

页数：10

共 43 条

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