jneuroJournal of Neurology and NeuropsychiatrycoVolume 2 Issue 2 (July-Dec)MultidisciplinaryEnglish6 Months NNY20251212Alzheimer’s DiseaseCase Report: Inflammatory Cerebral Amyloid Angiopathy - Recognizing a Treatable Form of Cerebral VasculitisEnglishY1621GarrettHarris1EnglishNChimaobiOchulo2EnglishYand ArjunAthreya1EnglishYhttps://doi.org/10.71117/JNNP.2025.2201Objective: To highlight the diagnostic and therapeutic importance of recognizing inflammatory cerebral amyloid angiopathy (iCAA) presenting with atypical cognitive decline and evolving neuroimaging findings.Background: Cerebral amyloid angiopathy (CAA) is an age-associated disease characterized by gradual deposition of beta-amyloid within the walls of cortical and leptomeningeal vessels. The inflammatory variant (iCAA) can cause subacute encephalopathy, seizures, and headaches with asymmetric white matter edema that can be steroid responsive. Diagnostic delays can occur due to the slow evolution of disease and overlap with more prevalent pathologies.Results: An 84-year-old woman initially presented with progressive aphasia. Brain MRI revealed lobar hemorrhage, T2 FLAIR white matter changes, and widespread cortical microbleeds. She was diagnosed with primary intracerebral hemorrhage but was lost to follow-up. Several years later, she returned with acute encephalopathy, worsening cognitive function, and radiographic changes that were inconsistent with previous explanations. The extensive workup for infectious and neoplastic conditions was unremarkable. Repeat imaging revealed persistent cortical microbleeds alongside new vasogenic edema—now fulfilling Boston Criteria 2.0 for probable CAA, with rising concern for an inflammatory variant. A brain biopsy was considered but deferred. High-dose IV methylprednisolone was initiated empirically, followed by an oral prednisone taper. Within weeks, she showed rapid clinical recovery, and follow-up MRI confirmed resolution of vasogenic edema with no new hemorrhages. Two months later, she regained baseline cognitive function and was independently performing daily tasks.Conclusions: This case illustrates how evolving radiologic patterns, when paired with unexplained clinical decline, can unmask a treatable diagnosis. iCAA mimics alternate pathologies but responds dramatically to immunosuppression. Delay in recognition can cost precious recovery time. Early identification, grounded in evolving imaging and clinical suspicion, can lead to cognitive improvement. Clinicians must remain vigilant to evolving MRI patterns and unexplained cognitive changes, particularly when prior diagnoses fail to account for clinical reality.EnglishInflammatory Cerebral Amyloid Angiopathy, Cerebral Vasculitis, Boston Criteria 2.0, Neuroimmunology, Cognitive Decline, Immunosuppressive Therapyhttps://jneuro.clsconf.com/admin/abstract?id=29References1619Charidimou A, Boulouis G, Frosch MP, et al. The Boston criteria version 2.0 for cerebral amyloid angiopathy: a multicentre, retrospective, MRIand;ndash;neuropathology diagnostic accuracy study.and;nbsp;Lancet Neurology. 2022;21(8):714and;ndash;725.DOI: https://doi.org/10.1016/S1474-4422(22)00208-3Kozberg MG, Perosa V, Gurol ME, van Veluw SJ. A practical approach to the management of cerebral amyloid angiopathy.and;nbsp;International Journal of Stroke. 2021;16(4):356and;ndash;369.DOI: https://doi.org/10.1177/1747493020974464Banerjee G, Carare R, Cordonnier C, et al. The increasing impact of cerebral amyloid angiopathy: essential new insights for clinical practice.and;nbsp;Journal of Neurology, Neurosurgery and;amp; Psychiatry. 2017;88(11):982and;ndash;994.DOI: https://doi.org/10.1136/jnnp-2016-314697Charidimou A, Boulouis G, Gurol ME, et al. Emerging concepts in sporadic cerebral amyloid angiopathy.and;nbsp;Brain. 2017;140(7):1829and;ndash;1850.DOI: https://doi.org/10.1093/brain/awx047Greenberg SM, Charidimou A. Diagnosis of cerebral amyloid angiopathy: evolution of the Boston Criteria.and;nbsp;Stroke. 2018;49(2):491and;ndash;497.DOI: https://doi.org/10.1161/STROKEAHA.117.016990Vinters HV. Cerebral amyloid angiopathy: a critical review.and;nbsp;Stroke. 1987;18(2):311and;ndash;324.DOI: https://doi.org/10.1161/01.str.18.2.311Chung Kong Khi, Anderson NE, Hutchinson D, et al. Cerebral amyloid angiopathyand;ndash;related inflammation: three case reports and a review.and;nbsp;Journal of Neurology, Neurosurgery and;amp; Psychiatry. 2011;82(1):20and;ndash;26.Salvarani C, Hunder GG, Morris JM, Brown RD Jr, Christianson T, Giannini C. Aand;beta;-related angiitis: comparison with cerebral amyloid angiopathy without inflammation and primary CNS vasculitis.and;nbsp;Neurology. 2013;81:1596and;ndash;1603.DOI: https://doi.org/10.1212/WNL.0b013e3182a9f545Jack CR Jr, Andrews JS, Beach TG, et al. Revised criteria for diagnosis and staging of Alzheimerand;rsquo;s disease: Alzheimerand;rsquo;s Association Workgroup.and;nbsp;Alzheimerand;rsquo;s and;amp; Dementia. 2024;20(8):5143and;ndash;5169.DOI: https://doi.org/10.1002/alz.13859de Souza A, Tasker K. Inflammatory cerebral amyloid angiopathy: a broad clinical spectrum.and;nbsp;Journal of Clinical Neurology. 2023;19(3):230and;ndash;241.DOI: https://doi.org/10.3988/jcn.2022.0493Theodorou A, Palaiodimou L, Malhotra K, et al. Clinical, neuroimaging, and genetic markers in cerebral amyloid angiopathyand;ndash;related inflammation: a systematic review and meta-analysis.and;nbsp;Stroke. 2023;54(1):178and;ndash;188.DOI: https://doi.org/10.1161/STROKEAHA.122.040671Todd R, McKenna E, McCluskey G, et al. Epidemiologic, clinical, and radiologic study of cerebral amyloid angiopathyand;ndash;related inflammation in Northern Ireland.and;nbsp;Neurology. 2025;105(5):e214005.DOI: https://doi.org/10.1212/WNL.0000000000214005Downes MH, Kalagara R, Rossitto CP, et al. Validation of the Boston Criteria Version 2.0 for cerebral amyloid angiopathy in patients presenting with intracerebral hemorrhage.and;nbsp;Neurology. 2025;104(7):e213460.DOI: https://doi.org/10.1212/WNL.0000000000213460