Title : Cerebral amyloid angiopathy related inflammation (CAARI) diagnosis and response to intravenous methylprednisolone in a district general hospital.
Abstract:
Introduction: Cerebral amyloid angiopathy related inflammation (CAARI) is a rare but now increasingly recognised presentation of cerebral amyloid angiopathy (CAA). The clinical presentation is most commonly an acute or subacute onset of cognitive decline, rapidly progressive dementia, personality changes, headache, seizures, focal neurological deficits and confusion.
Case presentation: A 78-year-old man, with a history of prostate adenocarcinoma and coronary artery bypass graft, presented to hospital with a fall. He experienced 6 months of rapidly progressive impaired working, visuospatial and semantic memory. He suffered with significant lapses in concentration with a remarkably low attention span and significant hallucinations. On the ward, he was markedly confused, agitated and aggressive. Inflammatory markers were normal, as were his renal function, liver function, bone profile and full blood count. A lumbar puncture showed slightly raised proteins. MRI head showed pronounced subcortical, central white matter oedema with multiple micro-haemorrhages. With all this combined, using the Chung criteria, we diagnosed the patient with CAARI. The facility of performing a brain biopsy was not available to confirm the diagnosis. He was then treated with methylprednisolone 1000mg IV for 3 days followed by prednisolone 60mg reducing the dose every week by 10mg until discontinue. Not only did his MRI oedema decrease, but his mental state showed significant improvement as well. His hallucinations resolved, and his attentiveness and concentration improved.
Conclusion: This will help to highlight ways in which to diagnose CAARI to those who do not have the facility to perform a brain biopsy or in patients in which is it inapplicable. We focus specifically on the history, mental state examination and radiology. Steroid therapy led to significant improvements clinically and radiologically.
What will the audience learn from your presentation?
• They will learn about a rare complication of cerebral amyloid angiopathy in the form of cerebral amyloid angiopathy-related inflammation (CAARI)
• How CAARI can present and what investigations are needed to rule out other causes
• How CAARI can be diagnosed in a hospital where some investigations are not available
• How CAARI can be treated and the response to this treatment
