The Storm Behind the Silence - Detecting Cerebral Amyloid Angiopathy-Related Inflammation in a Patient with Dementia

Sep 28, 2026

Uploaded by dabgoudw

Cerebral amyloid angiopathy–related inflammation (CAA-RI) is a rare, treatable inflammatory variant of cerebral amyloid angiopathy that often presents with acute or subacute cognitive or behavioral decline. The report describes a 76-year-old man with frontotemporal dementia admitted after weeks of worsening agitation followed by unresponsiveness. MRI showed bifrontal vasogenic edema, cortical superficial siderosis, and microhemorrhages consistent with CAA-RI. High-dose intravenous corticosteroids with a prolonged taper led to clinical improvement back to baseline before discharge.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages3

Reading Time8 min

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Article Information

Keywords

Submission, Acceptance, and Publication Dates

Case Presentation

Clinical Course and Treatment

Diagnostic Workup

Imaging and EEG

Discussion

CAA-RI Features, Classification, and Diagnostic Criteria

Images in Hospital Medicine The Storm Behind the Silence: Detecting Cerebral Amyloid AngiopathyRelated Inflammation in a Patient with Dementia Edward C Daingerfield, MD1,2, Vijairam Selvaraj, MD, MPH1,2, Kwame Dapaah-Afriyie, MD, MBA1,2 1 Division of Hospital Medicine, Department of Medicine, Miriam Hospital, 2 Department of Medicine, Brown University Journal of Brown Hospital Medicine Vol. 5, Issue 1, 2025 Article Information Keywords: dementia, cerebral amyloidangiopathy, CAA-RI, altered mental status, encephalopathy [https://doi.org/10.56305/001c.151726](https://doi.org/10.56305/001c.151726) Submitted: November 18, 2025 EST Accepted: December 01, 2025 EST Published: January 01, 2026 EST Abstract Cerebral amyloid angiopathy–related inflammation (CAA-RI) is a rare, treatable inflammatory variant of cerebral amyloid angiopathy that often presents with acute or subacute cognitive or behavioral decline. We describe a 76-year-old man with frontotemporal dementia who was admitted for an episode of unresponsiveness following several weeks of worsening agitation. MRI demonstrated bifrontal vasogenic edema, cortical superficial siderosis, and microhemorrhages, consistent with CAA-RI. The patient was treated with high-dose intravenous corticosteroids, followed by a prolonged taper, with clinical improvement to his baseline mental status prior to discharge. This case underscores the importance of considering CAA-RI in older adults with preexisting cognitive impairment who develop acute mental status changes. A 76-year-old man with a pertinent history of frontotemporal dementia presented to the hospital from his memory care unit with an episode of unresponsiveness in the setting of subacute behavioral dysregulation in the form of worsening agitation. There was no history of fevers, antecedent head trauma, or recent medication adjustments. On admission, his labs, including basic metabolic panel, complete blood count, and urine analysis, were unremarkable. TSH was low at 0.108 (ref: 0.330-4.120 uIU/ml), but Total T3 and Free T4 were normal. Treponemal IgM and IgG were unreactive. A CT brain was performed in the emergency department, which showed vasogenic edema in the bilateral frontal lobes with trace subarachnoid hemorrhage and hemorrhagic contusion in the right frontal lobe. A repeat CT performed six hours later showed no interval change. The patient was subsequently admitted, and both neurosurgery and neurology were consulted. They recommended initiating IV dexamethasone for vasogenic edema and IV levetiracetam for seizure prophylaxis. A routine EEG showed diffuse theta background slowing, suggesting an underlying mild to moderate diffuse encephalopathy without focal epileptiform discharges. An MRI of the brain was performed, which showed bifrontal vasogenic edema with both superficial siderosisand microhemorrhages, consistent with cerebral amyloidangiopathy-related inflammation (CAA-RI) (Figure 1) . The patient was initiated on treatment with high-dose IV methylprednisolone 1g/day for 5 days. His hospital course was complicated by agitation, which was treated with reorientation and as-needed anti-psychotics. The ag- itation was felt to be multifactorial secondary to his underlying acute process (CAA-RI), his underlying frontotemporal dementia, medication side effect due to high-dose steroids, and hospital-acquired delirium. His mental status improved throughout his stay, and on the day of discharge, the patient had returned to his baseline mental status. He was discharged with a prolonged steroid taper and follow-up with his primary neurologist. Patients with preexisting cognitive impairment can experience a decline in their usual mental status for multiple reasons. Common causes include systemic or central nervous system infections, inflammatory conditions that trigger cytokine release (e.g., interleukins), cerebrovascular events (both ischemic and hemorrhagic), new-onset seizures, CNS medication

FAQs

What is CAA-RI and how does it typically present?

CAA-RI is a rare, treatable inflammatory variant of cerebral amyloid angiopathy that often causes acute or subacute cognitive or behavioral decline.

How was CAA-RI diagnosed in this patient?

Brain imaging showed bifrontal vasogenic edema, cortical superficial siderosis, and microhemorrhages, which were consistent with CAA-RI, alongside supportive evaluation including EEG and other tests.

What treatment led to improvement, and what was the outcome?

The patient received high-dose intravenous corticosteroids (methylprednisolone) followed by a prolonged taper, with clinical improvement to his baseline mental status prior to discharge.

Sep 28, 2026

Uploaded by dabgoudw