A Case of Cerebral Amyloid Angiopathy-Related Inflammation (CAA-RI) Presenting as a Generalized Tonic Clonic Seizure

Abstract

Cerebral Amyloid Angiopathy (CAA) is characterized by amyloid beta-peptide deposits within the small to medium-sized vessels of the brain and leptomeninges. CAA is an important cause of intracerebral hemorrhage in older adults. Cerebral Amyloid Angiopathy Related Inflammation (CAA-ri) is, however, a rare variant of CAA that results from an autoimmune response to the deposits and is characterized by acute or subacute encephalopathy, headache, or focal neurological deficits. We present a case of a 62-year-old female who presented with a generalized tonic-clonic seizure witnessed by a family member. The event was preceded by a worsening of her dementia in the past few months. The patient had features suggestive of CAA-ri on Magnetic Resonance Imaging of the brain and was treated with high dose IV steroids. With an improvement in her cognitive symptoms and no further seizure episodes, she was discharged on oral steroids. Although CAA is well studied and well documented, its subset CAA-ri is uncommon with its clinical course and complications mentioned infrequently in medical literature. In conclusion, CAA-ri is underdiagnosed because of its rarity and remains a potentially treatable cause of subacute cognitive decline and seizures demanding further research in this area.