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

Asghar Marwat^1, , 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

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

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.

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Copyright

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References

[1]	Viswanathan A, Greenberg SM. Cerebral amyloid angiopathy in elderly. Ann Neurol. 2011 Dec; 70 (6): 871-80.
[2]	Charidimou A, Gang Q, Werring DJ. Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatry. 2012 Feb; 83 (2): 124-37.
[3]	C.Kinnecom, M. H. Lev, L. Wendell et al., “Course of cerebral amyloid angiopathy-related inflammation,” Neurology, vol 68, no.17, pp. 1411-1416, 2007.
[4]	J.A. Eng, M. P. Frosch, K. Choi, G. W. Rebeck, and S. M. Greenberg, “Clinical manifestations of cerebral amyloid angiopathy-related inflammation,” Annals of Neurology, vol. 55, no 2, pp. 250-256, 2004.
[5]	F.Piazza, S. M. Greenberg, M. Savoiardo et al., “Anti-amyloid Beta autoantibodies in cerebral amyloid angiopathy-related inflammation: Implications for amyloid-modifying therapies,” Annals of Neurology, vol. 73, no. 4, pp. 449-458, 2013.
[6]	Carlo Salvarani, MD, Gene G. Hunder, MD, Jonathan M. Morris, MD, Robert D. Brown, Jr, MD, MPH, Teresa Christianson, and Caterina Giannini, MD, PhD. Aβ-related angiitis. Comparison with CAA without inflammation and primary CNS vasculitis. Neurology. 2013 Oct 29; 81(18): 1596-1603.
[7]	Scolding NJ, Joseph F, Kirby PA, et al. Aβ-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 2005; 128: 500-515.
[8]	Masahito Yamada. Cerebral Amyloid Angiopathy: Emerging Concepts. J Stroke. 2015 Jan; 17(1): 17-30.
[9]	Benjamin Tolchin, Tadeau Fantaneanu, Michael Miller, Jeffrey Helgager, and Jong Woo Lee. Status epilepticus caused by cerebral amyloid angiopathy-related inflammation. Epilepsy Behav Case Rep. 2016; 6: 19-22.
[10]	Danve A1, Grafe M2, Deodhar A3. Amyloid beta-related angiitis--a case report and comprehensive review of literature of 94 cases. Semin Arthritis Rheum. 2014 Aug; 44(1): 86-92.
[11]	Francesca Crosta, Berardino Orlandi, Federica De Santis, Gianni Passalacqua, Jacopo C. DiFrancesco, Fabrizio Piazza, Alessia Catalucci, Giovambattista Desideri, and Carmine Marini. Cerebral Amyloid Angiopathy-Related Inflammation: Report of a Case with Very Difficult Therapeutic Management. Case Rep Neurol Med. 2015; 2015.
[12]	Morishige M1, Abe T, Kamida T, Hikawa T, Fujiki M, Kobayashi H, Okazaki T, Kimura N, Kumamoto T, Yamada A, Kawano Y. Cerebral vasculitis associated with amyloid angiopathy: case report. Neurol Med Chir (Tokyo). 2010; 50(4): 336-8.
[13]	P. de la Riva, F. Moreno, N. Carrera, M. Barandiarán, M. Arruti, J.F. Martí-Massó. Response to treatment with corticoids in a case of inflammatory amyloid angiopathy without performing a biopsy. REV NEUROL 2012;55:408-412.