Longitudinal neuropsychological findings in amyloid beta-related angiitis (AβRA): a case report

Amyloid β-related angiitis (AβRA) is a clinicopathological diagnosis of primary central nervous system angiitis theoretically triggered by vascular deposition of amyloid β peptide. Deposits of Aβ are associated with degeneration of the vasculature, thereby increasing risks of a stroke and/or cognitive impairment. Despite this, no prior studies have presented a detailed neuropsychological profile associated with AβRA. We present longitudinal neuropsychological findings for the case of a 58-year-old man with biopsy-diagnosed AβRA. Neuropsychological test results and clinical presentation demonstrated a mild to moderate dysexecutive syndrome implicating dorsolateral frontal and orbitofrontal-subcortical systems involvement. Despite prior reports of cognitive decline following a diagnosis of AβRA, cognitive functioning remained relatively stable over a 15-month period after immunosuppressive treatment. For the most part, objective measures did not demonstrate a measurable change in cognitive functioning, except for a mild decline in memory. There were subjective reports of improvement in cognitive and adaptive functioning from the patient and his spouse over this time period. The clinical significance of these results is discussed in the context of theories of executive dysfunction, and with reference to previously-published cases of AβRA.