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amyloid angiopathy related inflammation

Clotilde Hainline, Janet C Rucker, David Zagzag, John G Golfinos, Yvonne W Lui, Benjamin Liechty, Floyd A Warren, Laura J Balcer, Steven L Galetta
While cerebral amyloid angiopathy is a common cause of lobar hemorrhage, rarely it may be associated with an inflammatory response, thought to be incited by amyloid deposits. We report a 73-year-old woman with an extensive cancer history who presented with tumor-like lesions and symptoms of homonymous hemianopia and prosopagnosia. Found to have cerebral amyloid angiopathy-related inflammation proven by brain biopsy, she was treated successfully with immunosuppression.
March 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
Denise W Ng, Shino Magaki, Kevin H Terashima, Adrienne M Keener, Noriko Salamon, Stellios Karnezis, Luke Macyszyn, Harry V Vinters
Amyloid-β related angiitis (ABRA) is a rare complication of cerebral amyloid angiopathy in which amyloid-β (Aβ) deposition in the leptomeningeal and cortical vessels is associated with vasculitis characterized by transmural lymphohistiocytic, often granulomatous, inflammation. Patients usually present with acute to subacute cognitive dysfunction, headaches, and focal neurologic deficits. We report two cases of ABRA with unusual clinical presentations, including one case with fatal cerebral edema leading to herniation and Duret hemorrhages, and another associated with both lobar and deep parenchymal hemorrhages with intraventricular extension as well as hypercoagulability...
February 1, 2017: Human Pathology
Akio Kimura, Masao Takemura, Kuniaki Saito, Nobuaki Yoshikura, Yuichi Hayashi, Naoko Harada, Hiroshi Nishida, Hideto Nakajima, Takashi Inuzuka
Brain magnetic resonance imaging (MRI) of patients with Alzheimer's disease (AD) sometimes reveals multiple cerebral microbleeds (CMBs) and confluent white matter hyperintensities (WMHs) similar to those observed in cerebral amyloid angiopathy-related inflammation (CAA-I). To determine whether there might be common pathophysiological mechanisms underlying the MRI findings of multiple CMBs and confluent WMHs, we investigated the cerebrospinal fluid (CSF) profiles of 38 AD, five amnestic mild cognitive impairment (MCI), and six CAA-I patients...
December 20, 2016: Journal of Neurology
Duncan J Campbell
Neprilysin has a major role in both the generation and degradation of bioactive peptides. LCZ696 (valsartan/sacubitril, Entresto), the first of the new ARNI (dual-acting angiotensin-receptor-neprilysin inhibitor) drug class, contains equimolar amounts of valsartan, an angiotensin-receptor blocker, and sacubitril, a prodrug for the neprilysin inhibitor LBQ657. LCZ696 reduced blood pressure more than valsartan alone in patients with hypertension. In the PARADIGM-HF study, LCZ696 was superior to the angiotensin-converting enzyme inhibitor enalapril for the treatment of heart failure with reduced ejection fraction, and LCZ696 was approved by the FDA for this purpose in 2015...
March 2017: Nature Reviews. Cardiology
Yasutoshi Kido, Masao Takahashi, Nobuaki Fukuma, Takayuki Kawata, Atsushi Tanaka, Akimasa Hayashi, Junji Shibahara, Masao Daimon, Hiroyuki Morita, Hiroshi Akazawa, Issei Komuro
The main clinical manifestations of wild-type transthyretin (TTR)-related amyloidosis are progressive heart failure and neuropathy. There have been some reports on cerebral hemorrhage due to cerebral amyloid angiopathy in patients with TTR-related amyloidosis, but little is known about the vascular involvement in other organs. A 77-year-old woman experienced heart failure and was admitted for deteriorating heart failure status. Echocardiography showed diffuse hypokinesis of the left ventricle with biventricular wall thickness...
August 13, 2016: Cardiology
Benjamin Tolchin, Tadeau Fantaneanu, Michael Miller, Jeffrey Helgager, Jong Woo Lee
This report discusses a case of nonconvulsive status epilepticus, caused by cerebral amyloid angiopathy-related inflammation. Brain biopsy demonstrated cerebral amyloid angiopathy, with clinical and radiographic features indicative of a fluctuating inflammatory process. Immunomodulatory treatment with pulse steroids resulted in rapid and dramatic clinical and radiographic improvement. Cerebral amyloid angiopathy-related inflammation should be considered in the differential diagnosis of new-onset seizures after the age of 40, when associated with fluctuating multifocal T2 hyperintensities and petechial hemorrhages on gradient echo (GRE) or susceptibility-weighted (SWI) MRI sequences...
2016: Epilepsy & Behavior Case Reports
Carlo Salvarani, Jonathan M Morris, Caterina Giannini, Robert D Brown, Teresa Christianson, Gene G Hunder
Vascular inflammation is present in a subset of patients with cerebral amyloid angiopathy (CAA) and has a major influence in determining the disease manifestations. Radiological characterization of this subset is particularly important to achieve early recognition and treatment. We conducted this study to investigate the role of imaging in differentiating CAA with and without inflammation. We reviewed neuroimaging findings for 54 patients seen at Mayo Clinic over 25 years with pathological evidence of CAA and with available neuroimaging at the time of diagnosis...
May 2016: Medicine (Baltimore)
Dimitri Renard, Anne Wacongne, Anne Le Floch, Eric Thouvenot
No abstract text is available yet for this article.
2016: European Neurology
Yazan S Batarseh, Quoc-Viet Duong, Youssef M Mousa, Sweilem B Al Rihani, Khaled Elfakhri, Amal Kaddoumi
Amyloid-β (Aβ) pathology is known to promote chronic inflammatory responses in the brain. It was thought previously that Aβ is only associated with Alzheimer's disease and Down syndrome. However, studies have shown its involvement in many other neurological disorders. The role of astrocytes in handling the excess levels of Aβ has been highlighted in the literature. Astrocytes have a distinctive function in both neuronal support and protection, thus its involvement in Aβ pathological process may tip the balance toward chronic inflammation and neuronal death...
March 4, 2016: International Journal of Molecular Sciences
Shuguang Chu, Feijia Xu, Ya Su, Hong Chen, Xin Cheng
Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a relatively rare syndrome of reversible encephalopathy and could be divided into two subtypes of inflammatory CAA (ICAA) and amyloid-β-related angiitis (ABRA) according to histopathology. We present a case of pathologically proved ABRA with partial seizures and headache, and a focal lesion in the right temporal lobes on magnetic resonance imaging. Summarized from previous 139 ABRA and ICAA cases, ABRA is preferred when the lesion is enhanced on MRI and requires combination drug therapy, while ICAA is highly suspected with ApoE genotype of ɛ4/ɛ4...
2016: Journal of Alzheimer's Disease: JAD
Solène Ronsin, Gianluca Deiana, Ana Filipa Geraldo, Françoise Durand-Dubief, Laure Thomas-Maisonneuve, Maïté Formaglio, Virginie Desestret, David Meyronet, Norbert Nighoghossian, Yves Berthezène, Jérôme Honnorat, François Ducray
OBJECTIVE: To identify the clinical and radiologic features that should raise suspicion for the pseudotumoral presentation of cerebral amyloid angiopathy-related inflammation (CAA-I). METHODS: We retrospectively reviewed the characteristics of 5 newly diagnosed and 23 previously reported patients in whom the CAA-I imaging findings were initially interpreted as CNS neoplasms. RESULTS: Most cases (85%) occurred in patients >60 years old. The clinical characteristics at presentation included subacute cognitive decline (50%), confusion (32%), focal deficits (32%), seizures (25%), and headaches (21%)...
March 8, 2016: Neurology
Dimitri Renard, Anne Wacongne, Xavier Ayrignac, Mahmoud Charif, Genevieve Fourcade, Souhayla Azakri, Anne Le Floch, Stephane Bouly, Cecilia Marelli, Caroline Arquizan, Christophe Hirtz, Audrey Gabelle, Eric Thouvenot, Sylvain Lehmann
BACKGROUND: Decreased cerebrospinal fluid (CSF) amyloid-β 1-40 (Aβ40) and amyloid-β 1-42 (Aβ42) and increased total and phosphorylated tau (t-tau, p-tau) concentrations have been described in cerebral amyloid angiopathy (CAA). OBJECTIVE: Our aim was to analyze these biomarkers in patients with CAA-related inflammation (CAA-I). METHODS: We prospectively recruited nine patients with acute phase CAA-I fulfilling Chung criteria. CSF was analyzed for t-tau, p-tau, Aβ42, and Aβ40...
2016: Journal of Alzheimer's Disease: JAD
Andreas Charidimou
Cerebral amyloid angiopathy- related inflammation (CAA-ri) is an aggressive disease subtype of CAA with characteristic clinical and radiological findings. CAA-ri is an important diagnosis to reach in clinical practice, as patients typically respond to prompt immunosuppressive treatment. A definitive diagnosis of CAA-ri still requires a brain biopsy, and hence developing non-invasive diagnostic criteria and biomarkers for this syndrome are key priorities in the field. CAA-ri has gained additional interest for its notable similarities to amyloid-related imaging abnormalities, a complication of immunotherapy treatments in Alzheimer's disease patients...
2016: Journal of Alzheimer's Disease: JAD
Eitan Auriel, Andreas Charidimou, M Edip Gurol, Jun Ni, Ellis S Van Etten, Sergi Martinez-Ramirez, Gregoire Boulouis, Fabrizio Piazza, Jacopo C DiFrancesco, Matthew P Frosch, Oct Vio M Pontes-Neto, Ashkan Shoamanesh, Yael Reijmer, Anastasia Vashkevich, Alison M Ayres, Kristin M Schwab, Anand Viswanathan, Steven M Greenberg
IMPORTANCE: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an important diagnosis to reach in clinical practice because many patients with the disease respond to immunosuppressive therapy. Reliable noninvasive diagnostic criteria for CAA-ri would allow some patients to avoid the risk of brain biopsy. OBJECTIVE: To test the sensitivity and specificity of clinical and neuroimaging-based criteria for CAA-ri. DESIGN, SETTING, AND PARTICIPANTS: We modified the previously proposed clinicoradiological criteria and retrospectively analyzed clinical medical records and magnetic resonance imaging fluid-attenuated inversion recovery and gradient-echo scans obtained from individuals with CAA-ri and noninflammatory CAA...
February 2016: JAMA Neurology
Amanda M Carpenter, Inder P Singh, Chirag D Gandhi, Charles J Prestigiacomo
Intracerebral haemorrhage (ICH) is associated with the greatest morbidity and mortality of all stroke subtypes. Established risk factors for ICH include hypertension, alcohol use, current cigarette smoking, and use of oral anticoagulants and/or antiplatelet agents. Familial aggregation of ICH has been observed, and the heritability of ICH risk has been estimated at 44%. Few genes have been found to be associated with ICH at the population level, and much of the evidence for genetic risk factors for ICH comes from single studies conducted in relatively small and homogenous populations...
January 2016: Nature Reviews. Neurology
María Carmona-Iragui, Ana Fernández-Arcos, Daniel Alcolea, Fabrizio Piazza, Estrella Morenas-Rodriguez, Sofía Antón-Aguirre, Isabel Sala, Jordi Clarimon, Oriol Dols-Icardo, Valle Camacho, Frederic Sampedro, Josep Munuera, Fidel Nuñez-Marin, Alberto Lleó, Juan Fortea, Beatriz Gómez-Ansón, Rafael Blesa
We report a biomarker and genetic evaluation of four patients with cerebral amyloid angiopathy-related inflammation (CAA-ri) treated with corticosteroids. Patients presented with focal symptomatology and cognitive impairment. MRI revealed cortical microbleeds and asymmetrical hyperintense white matter lesions (WML). Cerebrospinal fluid (CSF) biomarker analyses showed increased anti-Aβ autoantibodies, t-Tau, and p-Tau and decreased Aβ40 and Aβ42. After treatment, focal symptomatology disappeared, and WML and anti-Aβ autoantibodies decreased...
2016: Journal of Alzheimer's Disease: JAD
Andreas Traschütz, Theophilos Tzaridis, Arndt-Hendrik Penner, Klaus Kuchelmeister, Horst Urbach, Elke Hattingen, Michael T Heneka
OBJECTIVE: To investigate whether the occurrence or clearance of microhemorrhages in cerebral amyloid angiopathy (CAA)-related vascular inflammation can be modified by immunosuppressive treatment. METHODS: Clinical and radiologic follow-up for more than 5 years of a patient with histopathologically confirmed CAA-related vascular inflammation treated with a prolonged and tapered regimen of IV cyclophosphamide and oral steroids. RESULTS: Under long-term immunosuppressive treatment, a reduced number of cortical micobleeds was observed on repeat MRIs because of both the prevention of new microbleeds and the clearance of those existing at baseline...
December 2015: Neurology® Neuroimmunology & Neuroinflammation
Prashant Raghavan, Seamus Looby, T David Bourne, Max Wintermark
BACKGROUND AND PURPOSE: Cerebral amyloid angiopathy with inflammation (CAA-I) is a less well-recognized clinically and radiologically distinct subtype of CAA. We aim to describe the imaging manifestations of this uncommon entity. MATERIALS AND METHODS: A retrospective review of the medical records and imaging database yielded 9 patients with clinical and radiological findings compatible with CAA-I. The neurological findings at presentation, MRI findings including the presence of white matter involvement, mass effect, microhemorrhages and contrast enhancement, treatment provided and outcome were evaluated...
February 2016: Journal of Neuroradiology. Journal de Neuroradiologie
Jacopo C DiFrancesco, Martina Longoni, Fabrizio Piazza
Amyloid-related imaging abnormalities (ARIA) represent the major severe side effect of amyloid-beta (Aβ) immunotherapy for Alzheimer's disease (AD). Early biomarkers of ARIA represent an important challenge to ensure safe and beneficial effects of immunotherapies, given that different promising clinical trials in prodromal and subjects at risk for AD are underway. The recent demonstration that cerebrospinal fluid (CSF) anti-Aβ autoantibodies play a key role in the development of the ARIA-like events characterizing cerebral amyloid angiopathy-related inflammation generated great interest in the field of immunotherapy...
2015: Frontiers in Neurology
Jacopo C DiFrancesco, Mehdi Touat, Massimo Caulo, Massimo Gallucci, Béatrice Garcin, Richard Levy, Antonino Uncini, Fabrizio Piazza
BACKGROUND: Cerebral amyloid angiopathy-related inflammation (CAA-ri) represents the most readily responsive form of CAA, if diagnosed and treated early. Although CAA-ri typically presents with a monophasic pattern, recurrences have been occasionally reported. OBJECTIVES: To describe the evolution of the clinical and neuroradiological features of CAA-ri recurrence. METHODS: From the 60 CAA-ri cases recruited through the iCAβ International Network, we identified those patients who experienced a CAA-ri recurrence at more than 12 months after the first inflammatory event...
2015: Journal of Alzheimer's Disease: JAD
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