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Cognitive decline in Dutch-type hereditary and sporadic cerebral amyloid angiopathy: a 5-year follow-up study
Journal article   Open access   Peer reviewed

Cognitive decline in Dutch-type hereditary and sporadic cerebral amyloid angiopathy: a 5-year follow-up study

Rosemarie van Dort, Vera C J van Stek-Smits, Sanne E Schriemer, Reinier G J van der Zwet, Manon R Schipper, Sabine Voigt, Ellen P Hart, Vandhana Easwaran, Hamid R Sohrabi, Kevin Taddei, …
Alzheimer's & dementia, Vol.22(7), e71629
2026
PMID: 42360287
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Published (Version of Record) Open Access CC BY V4.0

Abstract

amyloid beta (Aβ) deposition cerebral amyloid angiopathy cerebral small-vessel disease (CSVD) cognitive decline cognitive impairment Dutch-type cerebral amyloid angiopathy (D-CAA) hereditary CAA intracerebral hemorrhage (ICH) neurodegeneration sporadic cerebral amyloidangiopathy (sCAA) vascular cognitive impairment
Introduction Cerebral amyloid angiopathy (CAA) is associated with cognitive impairment, but its longitudinal course of cognitive decline remains unclear. We investigated domain-specific cognitive trajectories in Dutch-type hereditary (D-CAA) and sporadic CAA (sCAA) to compare patterns and rates of decline. Methods We included 181 participants – 93 D-CAA mutation carriers (59 without, 34 with prior intracerebral hemorrhage [ICH]) and 88 with sCAA (57 without, 31 with ICH) – who underwent annual neuropsychological assessment. Longitudinal change in global cognition, memory, processing speed, and executive function was analyzed using linear mixed models. Results Over 5 years, cognitive decline was subtle but measurable. Memory and processing speed declined in D-CAA with prior ICH, whereas D-CAA without ICH and sCAA remained relatively stable. Global cognition showed a modest but significant decline, while executive function remained stable. Discussion CAA-related cognitive decline appears domain- and stage-dependent, suggesting progressive small-vessel injury rather than acute hemorrhage may drive deterioration. Highlights ∙ Cognitive decline in CAA is subtle but measurable over time, showing domain- and stage-specific patterns rather than overall deterioration. ∙ D-CAA mutation carriers with a history of ICH showed a decline in memory and processing speed, whereas cognitive function remained relatively stable in D-CAA mutation carriers without ICH and in patients with sCAA. ∙ Executive dysfunction was present at baseline across all CAA groups and remained stable over 5 years, suggesting a stable, early-onset deficit possibly related to small-vessel pathology.

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