Cerebral Amyloid Angiopathy-Related Inflammation (CAA-ri) is a rare subset of CAA presenting as cognitive decline with headaches and seizures. Patients may inappropriately acquire the diagnosis of dementia if imaging is unobtained. Here we discuss an unusual presentation of CAA-ri in a man with acute personality change and seizures.

A 76-year-old man was brought to the emergency department with acute personality change noticed by bystanders. He was found to be in status epilepticus. Basic labs were normal. CT angiography of head was negative for acute intracranial processes. Lumbar puncture revealed mildly elevated protein of 53 mg/dl, elevated total cell count of 30 mg/dl (7% neutrophils, 37% lymphocytes) and glucose level of 71 mg/dl. Acyclovir was started empirically.

MRI brain revealed punctate foci of susceptibility at the gray-white matter junction, suggestive of amyloid angiopathy. This was associated with confluent areas of subcortical white matter signal abnormality, predominantly in the right parietal lobe, as well as leptomeningeal enhancement (Figure 1).

IV Methylprednisolone was started. The patient’s altered mental status improved significantly after initiation of high-dose steroid therapy.

Two months later, his mental status improved, no longer exhibiting any behavioral or personality changes. Repeat MRI showed marked improvement in the previously identified confluent areas of subcortical white matter signal abnormalities (Figure 2).  

This case highlights the importance of considering neurological disease processes that may present as acute primary psychiatric disorder in the elderly population, without prior psychiatric disease.

CAA-ri is rarely described, representing a subset of CAA in patients. The diagnosis is important to consider as it can present as stroke, seizure, or cognitive decline –requiring different management. Herein, we report a case of CAA-ri that can be misdiagnosed owing to its unusual presentation of acute personality change. Early detection and management with high-dose steroids can often resolve symptoms without significant residual deficits.