Yu-Ming Chen and Kuan-Fei Chen
Cerebral amyloid angiopathy (CAA) is a well-known pathology of Alzheimer’s disease among elderly individuals. CAA-related inflammation (CAAri) has been increasingly viewed as a rare presentation of CAA. We report the case a 65-year-old man who presented with subacute dementia and reversible delirium secondary to probable CAAri. His laboratory findings were negative except for elevated cerebrospinal fluid protein levels. Brain magnetic resonance imaging (MRI) revealed disseminated microbleeds in both the cerebral hemispheres on T2 weighted gradient images and prominent confluent T2-hyperintense white matter lesions in the bilateral temporo-occipital subcortical areas. Suspecting CAAri, we prescribed steroid pulse therapy with oral steroid tapering. The acute delirium and agitation improved after medication. However, the CAAri-induced damage to the brain function persisted, as evidenced in the follow-up clinical performance and serial Mini-Mental State Examination (MMSE).
The identification and accurate diagnosis of CAAri are important because the disease may respond to immunosuppressive therapy. In clinical settings, the detection of CAA with diffuse microbleeds in the brain is essential for making a tentative diagnosis of CAAri. Immunosuppressive therapy is the cornerstone of treatment. However, the optimal treatment duration is currently uncertain and warrants further investigation. In conclusion, the possible diagnosis of CAAri should always be considered when a patient presents with sub-acute cognitive decline and compatible MRI findings (T2-hyperintense lesions with diffuse microbleeds).
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