Neuropsychological Aspects of Sporadic Cerebral Amyloid Angiopathy: A Case Series and Narrative Review.

Abstract

Introduction: Cerebral Amyloid Angiopathy (CAA) is a common form of cerebral small vessel disease (CSVD), characterized by the accumulation of amyloid-β (Aβ) protein in the walls of cortical and leptomeningeal arteries and arterioles. The sporadic form primarily affects the elderly and is closely associated with Alzheimer's disease (AD). Despite previous studies on cognition, the specific neuropsychological profile of CAA remains unclear. This study aims to describe the cognitive profile of CAA patients and characterize their neuropsychological aspects in the absence of a clinical diagnosis of AD.

Methods: We present a case series of six patients with probable CAA, without clinical evidence of AD, who underwent extensive neuropsychological assessment. Additionally, a narrative review was conducted to synthesize current knowledge of the cognitive and neuropsychological aspects of sporadic CAA.

Results: The narrative review indicates that CAA predominantly affects executive functioning, processing speed, episodic memory, global cognition, and visuospatial functions. In our case series, all patients exhibited impairments in these domains, except for global cognition. Notably, a specific dissociation was observed in the Rey Auditory Verbal Learning Test (RAVLT), with impaired delayed recall but preserved recognition.

Discussion: Sporadic CAA in patients without AD contributes to cognitive impairment, particularly affecting executive functioning, processing speed, visuospatial functions, and episodic memory. In our sample, memory impairment in CAA follows a dysexecutive pattern, characterized by retrieval deficits with preserved storage. This contrasts with the amnestic profile seen in AD and amnestic mild cognitive impairment (aMCI), where both retrieval and storage are compromised.

Conclusion: This distinct memory profile may represent a useful neuropsychological marker for differentiating CAA-related cognitive impairment from that associated with AD and its prodromal forms. This differentiation has potential implications for diagnosis, prognosis, and the development of tailored therapeutic strategies.