What is it about?

Cerebral amyloid angiopathy (CAA) is a common problem in older people. It can cause bleeding in the brain and problems with memory and thinking. Some people have a type of CAA that causes fast declines in memory and other brain functions. The main cause of this disease is the buildup of a protein called amyloid in the blood vessel walls of the brain. This makes the blood vessels weak and can lead to bleeding in the brain and problems with memory. Sometimes, people with CAA have episodes called "Amyloid Spells," where they have temporary problems like weakness or trouble speaking. These episodes can be mistaken for seizures or mini-strokes. Tests like CT scans or MRI scans can help diagnose CAA by showing signs of bleeding or other changes in the brain.

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Why is it important?

Understanding cerebral amyloid angiopathy (CAA) and its inflammatory subtypes is crucial. Patients with CAA may experience recurring episodes known as amyloid spells, which can be mistaken for seizures or mini-strokes. For instance, we encountered a 65-year-old woman initially diagnosed with seizures, but further evaluation revealed she was experiencing amyloid spells. MRI scans showed evidence of small brain bleeds and cortical hemorrhages typical of CAA, along with brain swelling indicating CAA-related inflammation. This highlights the importance of accurate diagnosis, as some of these patients have underlying risk factors for stroke and require careful assessment of benefits of primary or secondary stroke prevention strategies, with some benefiting from treatment with immunosuppressants. It's also important to understand that patients with CAA-related inflammation (CAARI) are different from those with vasculitis. In vasculitis, patients typically show a quick decline in mental status. However, with amyloid spells, which occur in CAARI, the symptoms come back again and again over time and develop more slowly

Perspectives

From my perspective, I strongly believe that patients with cerebral amyloid angiopathy (CAA) have a multifaceted etiology. It's crucial to accurately diagnose CAA because a misdiagnosis can expose these patients to risks of other complications. Particularly for those with inflammatory subtypes, accurate diagnosis is essential as they may require immunosuppressive medications for effective management. Additionally, patients presenting with cortical hemorrhages should receive critical care attention until they stabilize, ensuring optimal management and outcomes.

Baljinder Singh MD
New York University

Read the Original

This page is a summary of: Updated Outlook of Cerebral Amyloid Angiopathy and Inflammatory Subtypes: Pathophysiology, Clinical Manifestations, Diagnosis and Management, Journal of Alzheimer s Disease Reports, October 2022, IOS Press,
DOI: 10.3233/adr-220055.
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