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cerebral amyloid angiopathy 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
Konark Malhotra, Shino D Magaki, Maria Inmaculada Cobos Sillero, Harry V Vinters, Reza Jahan, Robert D Brown, David S Liebeskind
Perimesencephalic subarachnoid hemorrhage (PM-SAH) refers to intracranial hemorrhage located in the perimesencephalic cistern. The etiology remains mainly unclear, although venous leakage or rupture has been postulated. We report an interesting case of a 57-year-old healthy man who presented initially with PM-SAH with worsening of subcortical lesions on follow-up neuroimaging. Histopathological examination demonstrated cerebral amyloid angiopathy with perivascular inflammation.
December 7, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Shokei Kim-Mitsuyama
There is accumulating evidence that RAS inhibitors not only reduce blood pressure, but also exert pleiotropic effects, including a renoprotective effect, amelioration of insulin resistance, reduction in onset of diabetes, and suppression of cardiovascular remodelling,. However, the definite benefit of RAS inhibition in treatment of hypertension with CKD or DM is not conclusive. We previously performed the OlmeSartan and Calcium Antagonists Randomized (OSCAR) study comparing the preventive effect of high-dose ARB therapy versus ARB plus CCB combination therapy on cardiovascular morbidity and mortality in 1164 Japanese elderly hypertensive patients with baseline type 2 diabetes and/or CVD (Am J Med (2012))...
September 2016: Journal of Hypertension
Shokei Kim-Mitsuyama
There is accumulating evidence that RAS inhibitors not only reduce blood pressure, but also exert pleiotropic effects, including a renoprotective effect, amelioration of insulin resistance, reduction in onset of diabetes, and suppression of cardiovascular remodelling,. However, the definite benefit of RAS inhibition in treatment of hypertension with CKD or DM is not conclusive. We previously performed the OlmeSartan and Calcium Antagonists Randomized (OSCAR) study comparing the preventive effect of high-dose ARB therapy versus ARB plus CCB combination therapy on cardiovascular morbidity and mortality in 1164 Japanese elderly hypertensive patients with baseline type 2 diabetes and/or CVD (Am J Med (2012))...
September 2016: Journal of Hypertension
F Schöberl, O E Eren, F A Wollenweber, T Kraus, L Kellert
Sporadic cerebral amyloid angiopathy (CAA) is a cerebral small vessel disease in the elderly. Neuropathologically, it is characterized by deposition of amyloid-ß (Aß) in the wall of small to medium-sized arteries, capillaries and venules of the cerebral cortex and leptomeninges. Over the last years it was recognized as an important cause of spontaneous intracerebral hemorrhage and cognitive deficits in the elderly. The clinical and radiological manifestations are diverse ranging from acute onset focal neurological deficits due to intracerebral lobar hemorrhage to subacute progressive cognitive impairment due to Aß-mediated inflammation confluent subcortical edema...
September 2016: Fortschritte der Neurologie-Psychiatrie
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
Yun Zhai, Toru Yamashita, Yumiko Nakano, Zhuoran Sun, Jingwei Shang, Tian Feng, Ryuta Morihara, Yusuke Fukui, Yasuyuki Ohta, Nozomi Hishikawa, Koji Abe
Recently, aging societies have been showing an increasingly strong relationship between Alzheimer's disease (AD) and chronic cerebral hypoperfusion (HP). In the present study, we created a new mouse model for AD with HP, and investigated its clinical and pathological characteristics. Alzheimer's disease transgenic mice (APP23) were subjected to bilateral common carotid arteries stenosis with ameroid constrictors for slowly progressive cerebral HP. In contrast to simple APP23 mice, cerebral HP exacerbated motor and cognitive dysfunctions with white matter lesions and meningo-parenchymal amyloid-β (Aβ) burdens...
June 13, 2016: Journal of Alzheimer's Disease: JAD
Philipp Eisele, Angelika Alonso, Martin Griebe, Kristina Szabo, Michael G Hennerici, Achim Gass
OBJECTIVE: In multiple sclerosis (MS) lesions blood-brain-barrier (BBB) breakdown is a common phenomenon delineating the phase of focal inflammation in developing MS lesions. In other pathologies like cerebral amyloid angiopathy or arteriosclerotic cerebral small vessel disease permanent cerebral microbleeds (CMB) have been shown to be sensitive markers indicating BBB dysfunction. We were interested in the potential role of T(2)*-weighted MRI and CMBs as BBB integrity markers in MS. METHODS: A large cohort of 189 MS patients (179 relapsing remitting MS and 10 secondary progressive MS) was investigated on a 3T MRI system with conventional and T(2)*-weighted gradient echo MRI (T(2)*w) sequences...
May 2016: Multiple Sclerosis and related Disorders
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
Patrizia Giannoni, Margarita Arango-Lievano, Ines Das Neves, Marie-Claude Rousset, Kévin Baranger, Santiago Rivera, Freddy Jeanneteau, Sylvie Claeysen, Nicola Marchi
Clinical and experimental evidence point to a possible role of cerebrovascular dysfunction in Alzheimer's disease (AD). The 5xFAD mouse model of AD expresses human amyloid precursor protein and presenilin genes with mutations found in AD patients. It remains unknown whether amyloid deposition driven by these mutations is associated with cerebrovascular changes. 5xFAD and wild type mice (2 to 12months old; M2 to M12) were used. Thinned skull in vivo 2-photon microscopy was used to determine Aβ accumulation on leptomeningeal or superficial cortical vessels over time...
April 2016: Neurobiology of Disease
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
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