Abstract Details

Title Relapsing Remitting Rapidly Progressive Dementia Due to Cerebral Amyloid Angiopathy- Related Inflammation

Topic Aging, Dementia, and Behavioral Neurology

Presentation(s) P11 - Poster Session 11 (8:00 AM-9:00 AM)

Poster/Presentation
Number 3-012

Objective

This report highlights a case of relapsing remitting rapidly progressive dementia due to cerebral amyloid angiopathy-related inflammation (CAA-ri), focusing on the diagnostic challenges and the dramatic response to corticosteroid treatment.

Background CAA-ri (Cerebral Amyloid Angiopathy-related inflammation) is a rare yet reversible cause of rapidly progressive dementia, frequently overlooked due to nonspecific initial symptoms. While conditions like Creutzfeldt-Jakob disease or autoimmune encephalitis may come to mind first, CAA-ri offers a rare opportunity for cognitive recovery if diagnosed early. This case underscores the importance of recognizing CAA-ri’s distinct imaging features and the need for prompt therapeutic intervention.

Design/Methods N/A

Results A 76-year-old male presented with rapidly progressive cognitive impairment, generalized weakness, and a seizure episode. Seven years prior, he had suffered from a similar episode of cognitive decline. MRI then was unremarkable but was performed without a blood-sensitive sequence. After extensive testing including CSF analysis, he was diagnosed with probable autoimmune encephalitis and ultimately admitted to hospice only to later improve and return to his normal life. MRI during the index presentation revealed asymmetric vasogenic edema on FLAIR and numerous cerebral microbleeds on GRE primarily affecting the right cerebral hemisphere. A brain biopsy confirmed the presence of CAA-ri. The patient responded well to corticosteroid therapy, with marked cognitive improvement. Follow-up MRI demonstrated complete resolution of the vasogenic edema, and the patient returned to baseline cognitive function.

Conclusions

This case emphasizes the need for heightened clinical suspicion of CAA-ri in patients presenting with rapidly progressive dementia, particularly when initial diagnostic workups are inconclusive, or when the disease follows a relapsing-remitting pattern. The use of blood-sensitive sequences is essential for detecting characteristic microbleeds associated with CAA-ri. Timely corticosteroid therapy can result in dramatic clinical recovery, reinforcing the treatable nature of this condition.

Authors/Disclosures
Mittal S. Prajapati, MD PRESENTER	Dr. Prajapati has nothing to disclose.
Richard Cheung (Creighton University School of Medicine)	Mr. Cheung has nothing to disclose.
Saif S. Bawaneh, MD (Mayo Clinic)	Dr. Bawaneh has nothing to disclose.
Himanshu Verma, MBBS (Creighton University School of Medicine)	Dr. Verma has nothing to disclose.
Ali M. Al-Salahat, MBBS (Creighton University - Neurology Program)	Dr. Al-Salahat has nothing to disclose.
Adeolu O. Morawo, MD	Dr. Morawo has nothing to disclose.

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