摘要
HomeStrokeVol. 46, No. 9Inflammatory Cerebral Amyloid Angiopathy, Amyloid-β–Related Angiitis, and Primary Angiitis of the Central Nervous System Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticlePDF/EPUBInflammatory Cerebral Amyloid Angiopathy, Amyloid-β–Related Angiitis, and Primary Angiitis of the Central Nervous SystemSimilarities and Differences Aimen Moussaddy, MD, Ariel Levy, MD, FRCP, Daniel Strbian, MD, PhD, Sophia Sundararajan, MD, PhD, France Berthelet, MD, FRCP and Sylvain Lanthier, MD, OD, CSPQ Aimen MoussaddyAimen Moussaddy From the Neurovascular Program, Research Center (A.M., A.L., S.L.), and Department of Anatomy and Pathology (F.B.), Centre hospitalier de l’Université de Montréal, Montreal, Quebec, Canada; Department of Neurological Sciences (A.M., A.L., S.L.) and Department of Pathology and Cell Biology (F.B.), Faculty of Medicine, University of Montreal, Montreal, Quebec, Canada; Department of Neurology and Stroke Unit, University Hospitals of Cleveland/Case Medical Center, OH (S.S.); and Department of Neurology, Helsinki University Central Hospital, Helsinki, Finland (D.S.). Search for more papers by this author , Ariel LevyAriel Levy From the Neurovascular Program, Research Center (A.M., A.L., S.L.), and Department of Anatomy and Pathology (F.B.), Centre hospitalier de l’Université de Montréal, Montreal, Quebec, Canada; Department of Neurological Sciences (A.M., A.L., S.L.) and Department of Pathology and Cell Biology (F.B.), Faculty of Medicine, University of Montreal, Montreal, Quebec, Canada; Department of Neurology and Stroke Unit, University Hospitals of Cleveland/Case Medical Center, OH (S.S.); and Department of Neurology, Helsinki University Central Hospital, Helsinki, Finland (D.S.). Search for more papers by this author , Daniel StrbianDaniel Strbian From the Neurovascular Program, Research Center (A.M., A.L., S.L.), and Department of Anatomy and Pathology (F.B.), Centre hospitalier de l’Université de Montréal, Montreal, Quebec, Canada; Department of Neurological Sciences (A.M., A.L., S.L.) and Department of Pathology and Cell Biology (F.B.), Faculty of Medicine, University of Montreal, Montreal, Quebec, Canada; Department of Neurology and Stroke Unit, University Hospitals of Cleveland/Case Medical Center, OH (S.S.); and Department of Neurology, Helsinki University Central Hospital, Helsinki, Finland (D.S.). Search for more papers by this author , Sophia SundararajanSophia Sundararajan From the Neurovascular Program, Research Center (A.M., A.L., S.L.), and Department of Anatomy and Pathology (F.B.), Centre hospitalier de l’Université de Montréal, Montreal, Quebec, Canada; Department of Neurological Sciences (A.M., A.L., S.L.) and Department of Pathology and Cell Biology (F.B.), Faculty of Medicine, University of Montreal, Montreal, Quebec, Canada; Department of Neurology and Stroke Unit, University Hospitals of Cleveland/Case Medical Center, OH (S.S.); and Department of Neurology, Helsinki University Central Hospital, Helsinki, Finland (D.S.). Search for more papers by this author , France BertheletFrance Berthelet From the Neurovascular Program, Research Center (A.M., A.L., S.L.), and Department of Anatomy and Pathology (F.B.), Centre hospitalier de l’Université de Montréal, Montreal, Quebec, Canada; Department of Neurological Sciences (A.M., A.L., S.L.) and Department of Pathology and Cell Biology (F.B.), Faculty of Medicine, University of Montreal, Montreal, Quebec, Canada; Department of Neurology and Stroke Unit, University Hospitals of Cleveland/Case Medical Center, OH (S.S.); and Department of Neurology, Helsinki University Central Hospital, Helsinki, Finland (D.S.). Search for more papers by this author and Sylvain LanthierSylvain Lanthier From the Neurovascular Program, Research Center (A.M., A.L., S.L.), and Department of Anatomy and Pathology (F.B.), Centre hospitalier de l’Université de Montréal, Montreal, Quebec, Canada; Department of Neurological Sciences (A.M., A.L., S.L.) and Department of Pathology and Cell Biology (F.B.), Faculty of Medicine, University of Montreal, Montreal, Quebec, Canada; Department of Neurology and Stroke Unit, University Hospitals of Cleveland/Case Medical Center, OH (S.S.); and Department of Neurology, Helsinki University Central Hospital, Helsinki, Finland (D.S.). Search for more papers by this author Originally published16 Jul 2015https://doi.org/10.1161/STROKEAHA.115.010024Stroke. 2015;46:e210–e213Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: January 1, 2015: Previous Version 1 Cerebral amyloid angiopathy (CAA) results from deposition of amyloid-β fibrils in the wall of the small and medium-sized blood vessels, mostly arteries of the leptomeninges and cerebral cortex. Architectural disruption of amyloid-β laden vessels, occasionally with fibrinoid necrosis, leads to perivascular leakage. Vascular rupture causes lobar microbleeds or hematomas and high-convexity subarachnoid hemorrhages. The accumulation of amyloid-β causes vessel lumen obliteration, thereby leading to ischemic leukoencephalopathy and cerebral infarction. Varying amounts of perivascular inflammation may be present, involving multinucleated giant cells in the most severe cases.1 However, frank vasculitic destruction of the vessel wall such as is found in amyloid-β–related angiitis (ABRA)2 and primary angiitis of the central nervous system (PACNS)3 is absent in the inflammatory form of CAA (I-CAA). There is substantial clinical overlap in the phenotypes of I-CAA, ABRA, and PACNS. A proper diagnosis of these 3 conditions is necessary because their individual treatment differ. Through 3 cases, we summarize similarities and differences between I-CAA, ABRA, and PACNS.Case 1A 52-year-old woman complained about limb paresthesias lasting 3 days. She reported cognitive decline and fatigue lasting 3 months, a relentless headache that had lasted for a year, in addition to episodic migraine and tension-type headaches. Past medical history was unremarkable, except for active cigarette smoking. Physical and neurological examination was normal. Brain magnetic resonance imaging (MRI) showed nonspecific lobar, white matter, nonenhancing hyperintensities on T2-weighted sequences. No treatment was initiated in the absence of a specific diagnosis. Blood tests, including inflammatory and prothrombotic work-up, cardiac ultrasound, Holter monitoring, digital subtraction cerebral angiography, and cerebrospinal fluid (CSF) analysis were normal. She remained stable clinically. Three months later, repeat brain MRI revealed new lobar T2-hyperintensities (Figure 1A) and several lobar microbleeds on gradient-echo sequences. CNS biopsy revealed extensive amyloid-β deposits in the wall of small leptomeningeal and cortical arteries, occlusion of some small vessels, and perivascular cortical microhemorrhages but no vascular or perivascular leukocytic infiltration (Figure 1A). Genetic testing revealed apoE ε4/ε4 genotype and I-CAA was diagnosed. Positron emission tomography using 11C-Pittsburg compound B confirmed diffuse amyloid-β deposition (Figure 2). The patient responded clinically and radiologically to a 12-week course of corticosteroids. In 7 years of follow-up, she experienced 3 episodes of recurrent headaches associated with new lobar T2-hyperintensities in different locations, which responded to the same treatment. Asymptomatic T2-hyperintensies shown on MRI repeated between recurrent clinical episodes were not treated and regressed spontaneously.Download figureDownload PowerPointFigure 1. Brain magnetic resonance imaging (MRI) and histopathology in patients with inflammatory cerebral amyloid angiopathy (A), amyloid-β-related angiitis (B), and primary angiitis of the central nervous system (C). Brain MRI showing lobar T2-hyperintense lesions in all cases (left). Hematoxylin–eosin stain (middle) showing intact vessel wall in case 1 (A), vascular infiltrate by lymphocytes and granulomas, and fibrinoid necrosis in case 2 (B), and vascular infiltrate by lymphocytes, wall necrosis, and erythrocyte extravasation in case 3 (C). Immunostaining (right) showing vascular amyloid-β deposits in cases 1 (A) and 2 (B), and no amyloid-β deposits in case 3 (C).Download figureDownload PowerPointFigure 2. Positron emission tomographic scan showing diffuse uptake of 11C-Pittsburgh Compound B in inflammatory cerebral amyloid angiopathy.Case 2A 50-year-old man presented with psychomotor slowing, disinhibition, 2 weeks of persistent headaches, and subtle right hemiparesis. Brain MRI showed right temporal and insular T2-hyperintensities, and a fine leptomeningeal enhancement with gadolinium. CSF analysis documented mild lymphocytic pleocytosis, with negative cultures and stains. Herpetic encephalitis was initially suspected. Acyclovir and intravenous corticosteroids were initiated but discontinued after 4 weeks, when the results of polymerase chain reaction testing for herpes viruses came back negative. The patient had improved clinically and radiologically. He was discharged with a presumptive diagnosis of viral encephalitis. One month later, he had visual hallucinations, psychotic ideations, and relapsing headaches. Repeat MRI showed recurrent temporal hyperintensities (Figure 1B). MR angiography revealed irregular stenosis of the right middle cerebral artery. Repeat CSF analysis documented increased aseptic inflammation (leukocytes: 13×106/L; proteins: 1.24 g/L). Investigations excluded systemic malignancies, infections, and autoimmune conditions. A temporal lobe biopsy showed vessel wall damage with marked T-cell infiltration and granulomas, and some parenchymal lymphocytic infiltration (Figure 1B). Marked amyloid-β deposition was found to colocalize with the vasculitis. Infectious pathogens were not found. ABRA was therefore diagnosed. The patient was treated with corticosteroids and cyclophosphamide, which was later substituted by azathioprine to complete 3.5 years of treatment. At his last follow-up visit, 6 years after presentation, mild cognitive impairment persisted. He had no relapse.Case 3A 57-year-old wildlife conservation technician had right-sided hemiparesis and expressive aphasia. He reported fatigue and lack of concentration lasting 3 weeks. Because he was exposed to dead animals, an infectious cause was suspected, despite the absence of fever, headaches, or other suggestive signs. Brain MRI showed multifocal white matter T2-hyperintensities and vasogenic edema, without diffusion restriction (Figure 1C). Leptomeningeal and intracerebral gadolinium enhancement was present. CSF analysis showed modest inflammation (leukocytes: 2×106/L; proteins: 0.54 g/L). Extensive analysis of blood and CSF excluded an infectious process. Stereotactic brain biopsy was inconclusive. The patient followed a fluctuating clinicoradiological course, with responses to corticosteroid therapy and recurrences when discontinued. Further investigation by MR angiography identified narrowing of the left distal intracranial carotid and proximal middle cerebral artery. An open brain biopsy showed vessel wall destruction, T-cell predominant infiltration, fibrinoid necrosis, and prominent endothelial cells (Figure 1C). There were no amyloid-β deposits, granulomas, or neoplastic cells. PACNS was diagnosed. The patient was treated for 2 years with corticosteroids combined initially with cyclophosphamide followed by azathioprine. He responded clinically and radiologically, without relapses. Four years after presentation, residual cognitive decline and mild expressive aphasia persisted.DiscussionPartial overlap exists between the clinical features and investigation results of I-CAA,1 ABRA,2 and PACNS.3 Most I-CAA and ABRA patients are aged ≥50 years, whereas one-half of PACNS patients present before this age. Presenting symptoms indistinctively consist of different combinations of new-onset headaches, neuropsychiatric manifestations, focal neurological deficits, and epileptic seizures. Hallucinations may be more common in ABRA.2 Because spinal vessels are spared from amyloid-β deposition, symptoms of myelopathy are unexpected in I-CAA and ABRA and, therefore, suggest PACNS. Under these 3 conditions, variable degrees of CSF inflammation may be present. Autoantibodies and other blood markers of inflammation are generally absent. Brain MRI commonly shows white matter T2-hyperintensities (Figure 1) and leptomeningeal gadolinium enhancement may be present. However, multiple lobar microbleeds present on gradient-echo sequences are hallmarks of I-CAA. Their association with ABRA remains understudied. Acute infarcts, pseudotumor lesions, and intracerebral gadolinium–enhancing lesions are consistent with ABRA and PACNS. Cerebral angiography is unremarkable in I-CAA, but may show nonspecific vasculitic changes in ABRA and PACNS if medium-sized arteries are involved. The use of high-resolution contrast-enhanced vessel wall MRI may distinguish these changes from nonvasculitic causes.4 CNS biopsy may reveal distinctive histopathologic changes (Figure 3). The amount of amyloid-β deposits and the location and severity of the inflammatory reaction differ between I-CAA, ABRA, and PACNS. By definition, CAA is characterized by vessel wall amyloid deposits. An intense perivascular inflammation with multinucleated giant cells is found in a minority of CAA patients, possibly those with an exaggerated inflammatory response to vascular leakages that occur from amyloid-β laden arteries. ApoE ε4/ε4 genotype or other cofactors not yet identified may potentiate the intense inflammatory response in I-CAA. By contrast, frank vessel wall inflammation is found in ABRA and PACNS, including architectural destruction and infiltration by leukocytes. Multinucleated giant cells forming granulomas, fibrinoid necrosis, or both are variably present. In ABRA, amyloid-β colocalizes with inflammatory changes and can be shown within phagocytes in the vessel wall. This suggests that amyloid-β deposits comprise the main antigen triggering the vasculitic reaction. Conversely, amyloid-β deposits are absent or do not colocalize with the inflammatory changes in PACNS, suggesting that they are incidental findings or innocent bystanders under this condition. Antigens triggering the vasculitic response in PACNS are presumably independent from amyloid-β deposits. I-CAA generally responds clinically and radiologically to short courses of immunosuppressant treatment; however, long-term recurrences are frequent. Superiority of prolonged immunosuppressant treatment, which is recommended in ABRA and PACNS, remains unproven in I-CAA. One way of increasing our knowledge on these conditions is recruiting patients into ongoing trials, such as The INTERnational Study on Primary Angiitis of the CEntral nervous system (INTERSPACE).5Download figureDownload PowerPointFigure 3. Distinctive histopathologic changes of cerebral amyloid angiopathy (CAA), inflammatory CAA (I-CAA), amyloid-β-related angiitis (ABRA), and primary angiitis of the central nervous system (PACNS).TAKE-HOME POINTSInflammatory form of cerebral amyloid angiopathy, amyloid-β–related angiitis, and primary angiitis of the central nervous system (CNS) share several clinical features and investigation results.The presence of multiple lobar microbleeds on gradient-echo brain magnetic resonance imaging and apoE ε4/ε4 genotype help distinguish inflammatory form of cerebral amyloid angiopathy.Amyloid-β–related angiitis and primary angiitis of the central nervous system are clinically similar. Presenting age <50 years and myelopathy symptoms suggest primary angiitis of the central nervous system. CNS biopsy may reveal distinctive histopathologic changes.Whether amyloid-β–related angiitis and primary angiitis of the central nervous system can be differentiated by gradient-echo magnetic resonance imaging (multiple versus few lobar microbleeds) and positron emission tomography using 11C-Pittsburg compound B (diffuse versus minimal uptake) remains to be investigated.DisclosuresDr Lanthier received an unrestricted research grant for the INTERnational Study on Primary Angiitis of the CEntral nervous system from La Fondation des Gouverneurs de l’Espoir. The other authors report no conflicts.FootnotesCorrespondence to Sylvain Lanthier, MD, OD, CSPQ, 1560 Sherbrooke St E, Suite GR-1159, H2L4M1, Montreal, Quebec, Canada. E-mail [email protected]References1. Eng JA, Frosch MP, Choi K, Rebeck GW, Greenberg SM.Clinical manifestations of cerebral amyloid angiopathy-related inflammation.Ann Neurol. 2004; 55:250–256. doi: 10.1002/ana.10810.CrossrefMedlineGoogle Scholar2. Scolding NJ, Joseph F, Kirby PA, Mazanti I, Gray F, Mikol J, et al. Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy.Brain. 2005; 128(pt 3):500–515. doi: 10.1093/brain/awh379.CrossrefMedlineGoogle Scholar3. Hajj-Ali RA, Singhal AB, Benseler S, Molloy E, Calabrese LH.Primary angiitis of the CNS.Lancet Neurol. 2011; 10:561–572. doi: 10.1016/S1474-4422(11)70081-3.CrossrefMedlineGoogle Scholar4. Swartz RH, Bhuta SS, Farb RI, Agid R, Willinsky RA, Terbrugge KG, et al. Intracranial arterial wall imaging using high-resolution 3-tesla contrast-enhanced MRI.Neurology. 2009; 72:627–634. doi: 10.1212/01.wnl.0000342470.69739.b3.CrossrefMedlineGoogle Scholar5. Lanthier S, Calabrese LH, Ferro JM, Putaala J, Strbian D, Chagnon M, et al; INTERSPACE Investigators. The INTERnational Study on Primary Angiitis of the CEntral nervous system–a call to the world.Int J Stroke. 2014; 9:E23. doi: 10.1111/ijs.12284.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited ByTheodorou A, Palaiodimou L, Malhotra K, Zompola C, Katsanos A, Shoamanesh A, Boviatsis E, Dardiotis E, Spilioti M, Sacco S, Werring D, Cordonnier C, Alexandrov A, Paraskevas G and Tsivgoulis G (2022) Clinical, Neuroimaging, and Genetic Markers in Cerebral Amyloid Angiopathy-Related Inflammation: A Systematic Review and Meta-Analysis, Stroke, 54:1, (178-188), Online publication date: 1-Jan-2023. Tanner J, Richie M, Cadwell C, Eliaz A, Kim S, Haq Z, Rasool N, Shah M and Guterman E (2022) Amyloid-β related angiitis presenting as eosinophilic meningitis: a case report, BMC Neurology, 10.1186/s12883-022-02638-w, 22:1, Online publication date: 1-Dec-2022. Lázaro Romero A, Moreno Loscertales C and Marta Moreno E (2022) Unilateral cerebral amyloid angiopathy after neurointervention, Neurología (English Edition), 10.1016/j.nrleng.2021.05.009, 37:4, (310-312), Online publication date: 1-May-2022. Lázaro Romero A, Moreno Loscertales C and Marta Moreno E (2022) Angiopatía amiloide cerebral unilateral tras una neurointervención, Neurología, 10.1016/j.nrl.2021.05.006, 37:4, (310-312), Online publication date: 1-May-2022. Wang M, Tian J, Zhang J, Zhao S, Song M and Wang Z (2022) Human Galectin-7 Gene LGALS7 Promoter Sequence Polymorphisms and Risk of Spontaneous Intracerebral Hemorrhage: A Prospective Study, Frontiers in Molecular Neuroscience, 10.3389/fnmol.2022.840340, 15 Kozberg M, Perosa V, Gurol M and van Veluw S (2020) A practical approach to the management of cerebral amyloid angiopathy, International Journal of Stroke, 10.1177/1747493020974464, 16:4, (356-369), Online publication date: 1-Jun-2021. Rill C, Scheerer L, Iorgu A and Bonmann E (2021)(2021) Aβ-related Angiitis (ABRA)—A Rare Paraneoplastic Cause of Cerebral Vasculitis in a Young Patient, The Neurologist, 10.1097/NRL.0000000000000316, 26:3, (103-107) Wu J, Yao M and Ni J (2021)(2021) Cerebral amyloid angiopathy-related inflammation: current status and future implications, Chinese Medical Journal, 10.1097/CM9.0000000000001427, 134:6, (646-654) Gill C, Piquet A and Cho T (2021) Central Nervous System Vasculitis Neuroimmunology, 10.1007/978-3-030-61883-4_24, (385-404), . Suthiphosuwan S, Bharatha A, Hsu C, Lin A, Maloney J, Munoz D, Palmer C and Osborn A (2020) Tumefactive Primary Central Nervous System Vasculitis: Imaging Findings of a Rare and Underrecognized Neuroinflammatory Disease, American Journal of Neuroradiology, 10.3174/ajnr.A6736, 41:11, (2075-2081), Online publication date: 1-Nov-2020. Tominaga K, Kawaguchi T, Fujimura M, Saito A, Watanabe M and Tominaga T (2020) Slowly progressive cerebral amyloid angiopathy-related inflammation: Characteristic findings of sequential magnetic resonance imaging, Clinical Neurology and Neurosurgery, 10.1016/j.clineuro.2020.106198, 197, (106198), Online publication date: 1-Oct-2020. Ichimata S, Hata Y, Yoshida K and Nishida N (2020) Autopsy of a multiple lobar hemorrhage case with amyloid‐β‐related angiitis, Neuropathology, 10.1111/neup.12637, 40:3, (280-286), Online publication date: 1-Jun-2020. Van Ommeren R, Izenberg A, Shadowitz S, Aviv R and Keith J (2020) Clinical Reasoning: An 81-year-old woman with decreased consciousness and fluctuating right facial droop, Neurology, 10.1212/WNL.0000000000009410, 94:19, (843-848), Online publication date: 12-May-2020. McGraw C, LaHue S, Ferris S, Bollen A and Richie M (2020) A 52-Year-Old Man With Seizures and Progressive Cerebrovascular Lesions, The Neurohospitalist, 10.1177/1941874419899791, 10:2, (109-114), Online publication date: 1-Apr-2020. Hao Q, Tsankova N, Shoirah H, Kellner C and Nael K (2020) Vessel Wall MRI Enhancement in Noninflammatory Cerebral Amyloid Angiopathy, American Journal of Neuroradiology, 10.3174/ajnr.A6445, 41:3, (446-448), Online publication date: 1-Mar-2020. Hatano Y, Sugai A, Yamagishi T, Nakajima A, Kakita A and Onodera O (2020) Amyloid β-related angiitis presenting extensive brain involvement without detection of hemorrhagic lesions: A case report出血性病変が検出されずに広範な脳病変に至ったアミロイドβ関連血管炎の1例, Rinsho Shinkeigaku, 10.5692/clinicalneurol.cn-001340, 60:3, (187-192), . Poli L, De Giuli V, Piazza F, Volonghi I, Bigliardi G, Vallone S, Nichelli P, Gasparotti R, Zini A, Padovani A and Pezzini A (2020) A challenging diagnosis of reversible “vascular” dementia: Cerebral amyloid angiopathy-related inflammation, Journal of Neuroimmunology, 10.1016/j.jneuroim.2019.577109, 338, (577109), Online publication date: 1-Jan-2020. Du Y, Liu C, Ma C, Xu X, Zhou X, Zhou H and Huang C (2019) Cerebral amyloid angiopathy-related inflammation: a case report presenting with a rare variant in SORL1 gene, BMC Neurology, 10.1186/s12883-019-1326-2, 19:1, Online publication date: 1-Dec-2019. Soun J, Song J, Romero J and Schaefer P (2019) Central Nervous System Vasculopathies, Radiologic Clinics of North America, 10.1016/j.rcl.2019.07.005, 57:6, (1117-1131), Online publication date: 1-Nov-2019. Ribeiro B, Muniz B and Marchiori E (2019) Cerebral amyloid angiopathy-related inflammation: findings on magnetic resonance imaging, Radiologia Brasileira, 10.1590/0100-3984.2017.0117, 52:1, (66-67) (2018) Causes of Spontaneous Intracranial Hemorrhage Transient Ischemic Attack and Stroke, 10.1017/9781316161609.008, (113-126) Dumitrascu O, Okazaki E, Cobb S, Zarka M, De Souza S, Kumar G and O’Carroll C (2017) Amyloid-Beta-Related Angiitis with Distinctive Neuro-Ophthalmologic Features, Neuro-Ophthalmology, 10.1080/01658107.2017.1374982, 42:4, (237-241), Online publication date: 4-Jul-2018. Schaumberg J, Trauscheid M, Eckert B, Petersen D, Schulz-Schaeffer W, Röther J and Heide W (2017) Zerebrale Amyloidangiopathie assoziiert mit InflammationCerebral amyloid angiopathy associated with inflammation, Der Nervenarzt, 10.1007/s00115-017-0469-x, 89:6, (682-691), Online publication date: 1-Jun-2018. Corovic A, Kelly S and Markus H (2017) Cerebral amyloid angiopathy associated with inflammation: A systematic review of clinical and imaging features and outcome, International Journal of Stroke, 10.1177/1747493017741569, 13:3, (257-267), Online publication date: 1-Apr-2018. Jin T and Chen Y (2018) Cerebral amyloid angiopathy-related inflammation Radiopaedia.org, 10.53347/rID-58270 Geschwind M and Murray K (2018) Differential diagnosis with other rapid progressive dementias Human Prion Diseases, 10.1016/B978-0-444-63945-5.00020-9, (371-397), . Jhaveri M, Salzman K, Ross J, Moore K, Osborn A and Ho C (2018) Perivascular Space Enhancing Lesions Expertddx: Brain and Spine, 10.1016/B978-0-323-44308-1.50169-0, (470-473), . Salam S, Anandarajah M, Al-Bachari S, Pal P, Sussman J and Hamdalla H (2017) Relapsing cerebral amyloid angiopathy-related inflammation: the wax and the wane, Practical Neurology, 10.1136/practneurol-2017-001599, 17:5, (392-395), Online publication date: 1-Oct-2017. Heuer E, Jacobs J, Du R, Wang S, Keifer O, Cintron A, Dooyema J, Meng Y, Zhang X and Walker L Amyloid-Related Imaging Abnormalities in an Aged Squirrel Monkey with Cerebral Amyloid Angiopathy, Journal of Alzheimer's Disease, 10.3233/JAD-160981, 57:2, (519-530) Hainline C, Rucker J, Zagzag D, Golfinos J, Lui Y, Liechty B, Warren F, Balcer L and Galetta S (2017) Tumoral Presentation of Homonymous Hemianopia and Prosopagnosia in Cerebral Amyloid Angiopathy–Related Inflammation, Journal of Neuro-Ophthalmology, 10.1097/WNO.0000000000000474, 37:1, (48-52), Online publication date: 1-Mar-2017. Dutra L, de Souza A, Grinberg-Dias G, Barsottini O and Appenzeller S (2017) Central nervous system vasculitis in adults: An update, Autoimmunity Reviews, 10.1016/j.autrev.2016.12.001, 16:2, (123-131), Online publication date: 1-Feb-2017. Yi L (2017) Lateralized Cerebral Amyloid Angiopathy presenting with recurrent Lacunar Ischemic Stroke, Journal of Neuroscience and Neurological Disorders, 10.29328/journal.jnnd.1001005, 1:1, (029-032) Chu S, Xu F, Su Y, Chen H, Cheng X and Yu J Cerebral Amyloid Angiopathy (CAA)-Related Inflammation: Comparison of Inflammatory CAA and Amyloid-β-Related Angiitis, Journal of Alzheimer's Disease, 10.3233/JAD-151036, 51:2, (525-532) Willinsky R (2016) Evaluation of the Cerebral Vessels Diseases of the Brain, Head and Neck, Spine 2016-2019, 10.1007/978-3-319-30081-8_3, (17-22), . Gaillard F (2014) Inflammatory cerebral amyloid angiopathy Radiopaedia.org, 10.53347/rID-28025 September 2015Vol 46, Issue 9 Advertisement Article InformationMetrics © 2015 American Heart Association, Inc.https://doi.org/10.1161/STROKEAHA.115.010024PMID: 26185185 Manuscript receivedJune 4, 2015Manuscript acceptedJune 5, 2015Originally publishedJuly 16, 2015Manuscript revisedJune 4, 2015 Keywordsamyloid-beta-related angiitiscerebral amyloid angiopathycerebrospinal fluidmagnetic resonance imagingprimary angiitis of the central nervous systemPDF download Advertisement SubjectsMetabolismPathophysiology