{
  "abstract": "Literature Review Cerebral amyloid angiopathy related inflammation (CAARI) is an inflammatory central nervous system disorder closely related to the more common non-inflammatory variant, cerebral amyloid angiopathy (CAA). Symptoms include cognitive decline, focal neurological deficits and encephalopathy, 1 2 with the most common imaging findings being T2 hyperintense white matter lesions and cortical microbleeds on susceptibility weighted imaging.1–4 Definitive diagnosis is only confirmed with brain biopsy. Clinicoradiological criteria5 have been proposed to reduce requirement for biopsy, however rare case reports of patients with normal susceptibility weighted imaging on presentation6 7 may delay time to diagnosis. In this report we discuss such a case.Case Report The case is of a 75yo male who presented with subacute cognitive decline, mixed aphasia and visual field deficits. Initial imaging with magnetic resonance imaging (MRI) showed asymmetric T2 hyperintensity in the cortical and subcortical left occipital lobe suggestive of inflammation, however, was not associated with typical signs of underlying cerebral amyloid angiopathy such as cerebral microbleeds, superficial siderosis or lobar haemorrhage. Initial differentials included ischaemic, inflammatory and neoplastic pathologies. Due to diagnostic uncertainty, the decision was made to proceed with brain biopsy which confirmed a diagnosis of CAARI. The patient went on to receive high dose steroid therapy with clinical improvement.Conclusion The neuroradiological findings of cerebral amyloid angiopathy on susceptibility weighted imaging may not always be present in patients with cerebral amyloid-related inflammation and as such, gaining diagnostic certainty with brain biopsy remains indicated in selected cases.References Theodorou A, Palaiodimou L, Malhotra K, Zompola C, Katsanos AH, Shoamanesh A, Boviatsis E, Dardiotis E, Spilioti M, Sacco S, Werring DJ, Cordonnier C, Alexandrov AV, Paraskevas GP, Tsivgoulis G. Clinical, neuroimaging, and genetic markers in cerebral amyloid angiopathy-related inflammation: a systematic review and meta-analysis. Stroke 2023;54(1):78–188. DOI: 10.1161/STROKEAHA.122.040671Koo J, Park M, Yoo HS, Joo B, Ahn SJ , Lee JH, Ryu YH, Suh SH. Multimodal imaging findings of cerebral amyloid angiopathy related inflammation with unusual clinical manifestation: a case report. Investigative Magnetic Resonance Imaging 2023;27(1):62–66. DOI: 10.13104/imri.2022.1104Wu JJ, Yao M, Ni J. Cerebral amyloid angiopathy-related inflammation: current status and future implications. Chinese Medical Journal 2021;134(6):646–654. DOI: 10.1097/CM9.0000000000001427Salvarani C, Morris JM, Giannini C, Brown RD Jr, Christianson T, Hunder GG. Imaging findings of cerebral amyloid angiopathy, aβ-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. Medicine (Baltimore) 2016;95(20). DOI: 10.1097/MD.0000000000003613Chung KK, Anderson NE, Hutchinson D, Synek B, Barber PA. Cerebral amyloid angiopathy related inflammation: three case reports and a review. Journal of Neurology, Neurosurgery and Psychiatry 201;82(1):20–26. DOI: 10.1136/jnnp.2009.204180Dudley A, Sweeney K, Looby S, Farrell M, McGovern E. Teaching neuroimage: cerebral amyloid angiopathy-related inflammation. Neurology 2022;99:216–217. DOI:10.1212/WNL.0000000000200833Aghetti A, Sène D, Polivka M, Shor N, Lechtman S, Chabriat H, Jouvent E, Guey S. Cerebral amyloid angiopathy related inflammation with prominent meningeal involvement. A report of 2 cases. Frontiers in Neurology 2019;10. DOI:10.3389/fneur.2019.00984",
  "authors": [
    {
      "affiliations": [
        "SA Health, Magill, SA, Australia"
      ],
      "name": "Jack Rumbelow"
    }
  ],
  "title": "3507 Histopathological diagnosis of CAARI without MRI features of CAA: a case report",
  "uid": "b8d59993-ce50-5ae5-9de8-38099a8dde95"
}