Cerebral amyloid angiopathy (CAA) is one of the most frequent causes of non-traumatic intracerebral hemorrhage (ICH). ICH recurrence risk is significantly higher in patients with CAA than for those without the condition, and CAA is a risk factor for the development of dementia, particularly Alzheimer’s disease. There is a growing body of research describing neuropsychological impairment observed in patients with CAA. Among patients with a history of CAA-related ICH, the most commonly identified cognitive impairments include attention, processing speed, executive functioning, and episodic memory. However, little is known about potential additive or synergistic effects of each CAA-related lesion (such as recurrent ICHs) on cognitive functioning.

We present a case of a 74-year-old female with sporadic CAA, who had recurrent ICHs involving the left occipitoparietal lobe, left frontoparietal lobe, right occipital lobe, and left frontal lobe. She experienced residual visual impairment and probable Charles Bonnet Syndrome. Her clinical presentation and cognitive functioning were tracked with an inpatient neuropsychological evaluation completed after each ICH occurrence within the past year, as well as an outpatient neuropsychological evaluation completed approximately 3-months post-discharge from her most recent hospital admission. Record review, including clinical notes, lab tests, and imaging results supplement her performance on serial inpatient and outpatient neuropsychological evaluations.

Data from three inpatient neuropsychological screenings and one lengthier outpatient evaluation are presented. With each inpatient evaluation, her profile demonstrated further cognitive decline involving visuospatial skills, semantic fluency, and episodic memory. In fact, results from her last inpatient screening raised concern for an underlying cortical degenerative process. In contrast, her follow-up outpatient evaluation, after three separate ICH events within one year, demonstrated an isolated set-shifting impairment, with intact performance across all other domains, which ruled out the prior suspicion of a cortical process.

While specific domains of cognition are more vulnerable in CAA, it is difficult to identify a specific and expected cognitive pattern given the extensive number of varied neurological insults patients typically develop throughout the disease course. This case demonstrates the wide range effects of repeated ICH, as well as the contrast between the acute effects of new lesions and the lasting effects of these lesions on cognitive ability after a period of recovery and stabilization. Given that our service was able to perform neuropsychological assessment in the acute phase of each ICH and in the subacute phase after a period of stabilization, this case adds to the literature by providing an example of the additive or synergistic effects of each CAA-related lesion over time.