American Journal of Medical Case Reports
ISSN (Print): 2374-2151 ISSN (Online): 2374-216X Website: Editor-in-chief: Samy, I. McFarlane
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American Journal of Medical Case Reports. 2021, 9(5), 275-277
DOI: 10.12691/ajmcr-9-5-3
Open AccessCase Report

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

Asghar Marwat1, , Assad Marwat2, Hina Bangash3, Sadaqat Liaqat1, Muzammil Khan1 and Shaza Khalid1

1Department of Internal Medicine Conemaugh Memorial Medical Center Johnstown PA

2Shifa College of Medicine Islamabad, Pakistan

3Rawalpindi Medical University, Rawalpindi, Pakistan

Pub. Date: February 22, 2021

Cite this paper:
Asghar Marwat, Assad Marwat, Hina Bangash, Sadaqat Liaqat, Muzammil Khan and Shaza Khalid. A Case of Cerebral Amyloid Angiopathy-Related Inflammation (CAA-RI) Presenting as a Generalized Tonic Clonic Seizure. American Journal of Medical Case Reports. 2021; 9(5):275-277. doi: 10.12691/ajmcr-9-5-3


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.

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