Trajectory Pattern of Cognitive Decline in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy.


Journal

Neurology
ISSN: 1526-632X
Titre abrégé: Neurology
Pays: United States
ID NLM: 0401060

Informations de publication

Date de publication:
15 Jun 2022
Historique:
received: 03 01 2022
accepted: 14 04 2022
entrez: 15 6 2022
pubmed: 16 6 2022
medline: 16 6 2022
Statut: aheadofprint

Résumé

The course and pattern of cognitive decline in ischemic cerebral small vessel disease (cSVD) remains poorly characterized. We analysed the trajectory pattern of cognitive decline from age 25 to 75 years in CADASIL. We applied latent process mixed models to data obtained from CADASIL patients who were repeatedly scored during follow-up using 16 selected clinical scales or cognitive tests. The modelled evolutions of these scores obtained from 1243 observations in 265 patients recruited at the French National Referral Centre (50.1 years on average and 45.3% males) showed wide and heterogeneous variations in amplitude along the age-related progression of the disease. While the Backward Digit Span remained essentially stable, a linear deterioration of scores obtained using the Symbol Digit Numbers or Number of Errors of Trail Making Test B was detected from 25 to 75 years. In contrast, the largest score changes were observed at midlife using the Digit Cancellation Task. All other tests related to executive functions, memory performances, or global cognitive efficiency showed a rate of change accelerating especially at the advanced stage of the disease. Male gender, the presence of gait disorders or of some disability at baseline were found to predict earlier or large changes of 4 scores (Index of Sensitivity to Cueing, Delayed Total Recall, Initiation/Perseveration and Barthel Index) in a subgroup of individuals distinct form the rest of the sample. Cognitive alterations develop heterogeneously during the progression of CADASIL and vary largely according to the stage of the disease. These results suggest that not only the target population, study duration but also the stage of disease progression should be considered in preparing future clinical trials aimed at reducing cognitive decline in any such condition.

Sections du résumé

BACKGROUND AND OBJECTIVE OBJECTIVE
The course and pattern of cognitive decline in ischemic cerebral small vessel disease (cSVD) remains poorly characterized. We analysed the trajectory pattern of cognitive decline from age 25 to 75 years in CADASIL.
METHODS METHODS
We applied latent process mixed models to data obtained from CADASIL patients who were repeatedly scored during follow-up using 16 selected clinical scales or cognitive tests.
RESULTS RESULTS
The modelled evolutions of these scores obtained from 1243 observations in 265 patients recruited at the French National Referral Centre (50.1 years on average and 45.3% males) showed wide and heterogeneous variations in amplitude along the age-related progression of the disease. While the Backward Digit Span remained essentially stable, a linear deterioration of scores obtained using the Symbol Digit Numbers or Number of Errors of Trail Making Test B was detected from 25 to 75 years. In contrast, the largest score changes were observed at midlife using the Digit Cancellation Task. All other tests related to executive functions, memory performances, or global cognitive efficiency showed a rate of change accelerating especially at the advanced stage of the disease. Male gender, the presence of gait disorders or of some disability at baseline were found to predict earlier or large changes of 4 scores (Index of Sensitivity to Cueing, Delayed Total Recall, Initiation/Perseveration and Barthel Index) in a subgroup of individuals distinct form the rest of the sample.
DISCUSSION CONCLUSIONS
Cognitive alterations develop heterogeneously during the progression of CADASIL and vary largely according to the stage of the disease. These results suggest that not only the target population, study duration but also the stage of disease progression should be considered in preparing future clinical trials aimed at reducing cognitive decline in any such condition.

Identifiants

pubmed: 35705499
pii: WNL.0000000000200805
doi: 10.1212/WNL.0000000000200805
pmc: PMC9519251
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

© 2022 American Academy of Neurology.

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Auteurs

Sandrine Brice (S)

Sorbonne Université, INSERM, Unité Mixte de Recherche 1136, Institut Pierre Louis d'Épidémiologie et de Santé Publique, Paris, France.
Sorbonne Université, INSERM, Institut Pierre Louis d'Épidémiologie et de Santé Publique, AP-HP, Hôpitaux Universitaires Pitié Salpêtrière - Charles Foix, F75013 Paris, France.

Sonia Reyes (S)

Département de Neurologie et Centre Neurovasculaire Translationnel, Centre de référence CERVCO, FHU NeuroVasc, Hôpital Lariboisière, AP-HP, Université de Paris, 75010 Paris, France.

Aude Jabouley (A)

Département de Neurologie et Centre Neurovasculaire Translationnel, Centre de référence CERVCO, FHU NeuroVasc, Hôpital Lariboisière, AP-HP, Université de Paris, 75010 Paris, France.

Carla Machado (C)

Département de Neurologie et Centre Neurovasculaire Translationnel, Centre de référence CERVCO, FHU NeuroVasc, Hôpital Lariboisière, AP-HP, Université de Paris, 75010 Paris, France.

Christina Rogan (C)

Département de Neurologie et Centre Neurovasculaire Translationnel, Centre de référence CERVCO, FHU NeuroVasc, Hôpital Lariboisière, AP-HP, Université de Paris, 75010 Paris, France.

Nathalie Gastellier (N)

Département de Neurologie et Centre Neurovasculaire Translationnel, Centre de référence CERVCO, FHU NeuroVasc, Hôpital Lariboisière, AP-HP, Université de Paris, 75010 Paris, France.

Nassira Alili (N)

Département de Neurologie et Centre Neurovasculaire Translationnel, Centre de référence CERVCO, FHU NeuroVasc, Hôpital Lariboisière, AP-HP, Université de Paris, 75010 Paris, France.

Stephanie Guey (S)

Département de Neurologie et Centre Neurovasculaire Translationnel, Centre de référence CERVCO, FHU NeuroVasc, Hôpital Lariboisière, AP-HP, Université de Paris, 75010 Paris, France.
INSERM, Unité Mixte de Recherche 1161, 75010 Paris, France.

Eric Jouvent (E)

Département de Neurologie et Centre Neurovasculaire Translationnel, Centre de référence CERVCO, FHU NeuroVasc, Hôpital Lariboisière, AP-HP, Université de Paris, 75010 Paris, France.
INSERM, Unité Mixte de Recherche 1161, 75010 Paris, France.

Dominique Hervé (D)

Département de Neurologie et Centre Neurovasculaire Translationnel, Centre de référence CERVCO, FHU NeuroVasc, Hôpital Lariboisière, AP-HP, Université de Paris, 75010 Paris, France.
INSERM, Unité Mixte de Recherche 1161, 75010 Paris, France.

Sophie Tezenas du Montcel (S)

Sorbonne Université, INSERM, Unité Mixte de Recherche 1136, Institut Pierre Louis d'Épidémiologie et de Santé Publique, Paris, France.
Sorbonne Université, INSERM, Institut Pierre Louis d'Épidémiologie et de Santé Publique, AP-HP, Hôpitaux Universitaires Pitié Salpêtrière - Charles Foix, F75013 Paris, France.

Hugues Chabriat (H)

Département de Neurologie et Centre Neurovasculaire Translationnel, Centre de référence CERVCO, FHU NeuroVasc, Hôpital Lariboisière, AP-HP, Université de Paris, 75010 Paris, France hugues.chabriat@aphp.fr.
INSERM, Unité Mixte de Recherche 1161, 75010 Paris, France.

Classifications MeSH