scholarly journals The Role of Amyloid PET in Diagnosing Possible Transmissible Cerebral Amyloid Angiopathy in Young Adults with a History of Neurosurgery: A Case Series

2021 ◽  
pp. 1-5
Author(s):  
Laura Michiels ◽  
Donatienne Van Weehaeghe ◽  
Rik Vandenberghe ◽  
Jelle Demeestere ◽  
Koen Van Laere ◽  
...  
Keyword(s):  
Young Adults ◽  
Cerebral Amyloid Angiopathy ◽  
Case Series ◽  
Amyloid Angiopathy ◽  
Amyloid Pet ◽  
Childhood All ◽  
History Of Neurosurgery ◽  
History Of ◽  
Cerebral Amyloid

Background: Cerebral amyloid angiopathy (CAA) is a common cause of cerebrovascular disease in the elderly. There is accumulating evidence suggestive of transmissibility of β-amyloid resulting in amyloid pathology at younger age. According to the Boston criteria, defining CAA in patients <55 years requires histological evidence which may hamper diagnosis. We explored the role of amyloid PET in the diagnosis of possible transmissible CAA in young adults. Cases: We report 4 young adults (<55 years) presenting with clinical and neuroimaging features suggestive of CAA but without genetic evidence of hereditary CAA explaining the young onset. A common factor in all cases was a medical history of neurosurgery during childhood. All patients underwent amyloid PET to support the diagnosis of an amyloid-related pathology and the result was positive in all 4. Conclusion: Combining the clinical presentation and imaging findings of the 4 cases, we postulate transmissible CAA as the possible diagnosis. Further epidemiological studies are required to gain more insight in the prevalence of this novel entity. Amyloid PET may be a useful, non-invasive tool in these analyses especially since pathological evidence will be lacking in most of these studies.

Cerebral amyloid angiopathy–associated intracerebral hemorrhage: pathology and management

2012 ◽  
Vol 32 (4) ◽  
pp. E7 ◽  
Author(s):  
Prachi Mehndiratta ◽  
Sunil Manjila ◽  
Thomas Ostergard ◽  
Sylvia Eisele ◽  
Mark L. Cohen ◽  
...  
Keyword(s):  
Intracerebral Hemorrhage ◽  
Cerebral Amyloid Angiopathy ◽  
Management Strategies ◽  
Case Series ◽  
The Elderly ◽  
Postoperative Hemorrhage ◽  
Amyloid Angiopathy ◽  
Increased Risk ◽  
Cerebral Amyloid

Amyloid angiopathy–associated intracerebral hemorrhage (ICH) comprises 12%–15% of lobar ICH in the elderly. This growing population has an increasing incidence of thrombolysis-related hemorrhages, causing the management of hemorrhages associated with cerebral amyloid angiopathy (CAA) to take center stage. A concise reference assimilating the pathology and management of this clinical entity does not exist. Amyloid angiopathy–associated hemorrhages are most often solitary, but the natural history often progresses to include multifocal and recurrent hemorrhages. Compared with other causes of ICH, patients with CAA-associated hemorrhages have a lower mortality rate but an increased risk of recurrence. Unlike hypertensive arteriolar hemorrhages that occur in penetrating subcortical vessels, CAA-associated hemorrhages are superficial in location due to preferential involvement of vessels in the cerebral cortex and meninges. This feature makes CAA-associated hemorrhages easier to access surgically. In this paper, the authors discuss 3 postulates regarding the pathogenesis of amyloid hemorrhages, as well as the established clinicopathological classification of amyloid angiopathy and CAA-associated ICH. Common inheritance patterns of familial CAA with hemorrhagic strokes are discussed along with the role of genetic screening in relatives of patients with CAA. The radiological characteristics of CAA are described with specific attention to CAA-associated microhemorrhages. The detection of these microhemorrhages may have important clinical implications on the administration of anticoagulation and antiplatelet therapy in patients with probable CAA. Poor patient outcome in CAA-associated ICH is associated with dementia, increasing age, hematoma volume and location, initial Glasgow Coma Scale score, and intraventricular extension. The surgical management strategies for amyloid hemorrhages are discussed with a review of published surgical case series and their outcomes with a special attention to postoperative hemorrhage.

Clinical variability and the role of diagnostic criteria of cerebral amyloid angiopathy-related inflammation (CAA-RI): A case series

2021 ◽  
Vol 429 ◽  
pp. 118793
Author(s):  
Fulvio Pasquin ◽  
Valentina Tommasini ◽  
Alessandro Dinoto ◽  
Francesco Biaduzzini ◽  
Laura D'Acunto ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Cerebral Amyloid Angiopathy ◽  
Case Series ◽  
Amyloid Angiopathy ◽  
Clinical Variability ◽  
Cerebral Amyloid

Amyloid-PET in cerebral amyloid angiopathy

Neurology ◽  
2017 ◽  
Vol 89 (14) ◽  
pp. 1437-1438 ◽  
Author(s):  
Nicolas Raposo ◽  
Joshua A. Sonnen
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Amyloid Pet ◽  
Cerebral Amyloid

Abstract W MP89: Validation of Clinical-Radiological Criteria for the Diagnosis of Cerebral Amyloid Angiopathy-related Inflammation

Stroke ◽  
2014 ◽  
Vol 45 (suppl_1) ◽  
Author(s):  
Eitan Auriel ◽  
Mahmut Edip Gurol ◽  
Jun Ni ◽  
Ellis Van Etten ◽  
Sergi Martinez-Remirez ◽  
...  
Keyword(s):  
Sensitivity And Specificity ◽  
Cerebral Amyloid Angiopathy ◽  
Vascular Inflammation ◽  
Brain Biopsy ◽  
Control Group ◽  
Amyloid Angiopathy ◽  
Radiological Criteria ◽  
Disease Subtype ◽  
History Of ◽  
Cerebral Amyloid

Introduction: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a disease subtype characterized by rapidly progressive cognitive decline, seizures, headaches, T2-hyperintense MRI lesions, and neuropathologic evidence of CAA-associated vascular inflammation. CAA-ri is an important diagnosis to reach in clinical practice, as many patients respond to immunosuppressive therapy. Definitive diagnosis of CAA-ri generally requires brain biopsy, however, highlighting the importance of developing noninvasive diagnostic criteria. Objectives: To test the sensitivity and specificity of modified criteria (Table 1) for possible and probable CAA-ri in groups of subjects with histologically proven CAA-ri and non-CAA-ri. Methods: After refining previously proposed clinical- radiological criteria we retrospectively analyzed clinical charts and MRI FLAIR and gradient-echo scans obtained from 17 subjects with pathologically confirmed CAA-ri and 37 control subjects with pathologic diagnosis of non-inflammatory CAA. The control group was further divided into those with past intracerebral hemorrhage (ICH) (n=21) and those with cerebral microbleeds (CMB) only and no history of ICH (n=16). Results: In the CAA-ri group 14/17 (82.4%) met criteria for both probable and possible CAA-ri. One (4.7%) of the subjects in the control CAA-ICH group (n=21) met the criteria for possible and none met criteria for probable CAA-ri. In the control CAA-no ICH group 1/16 (6.25%) and 11/16 (68.75%) met criteria for probable and possible CAA-ri respectively. This yields sensitivity and specificity of 82% and of 97.3% respectively for the probable criteria and sensitivity and specificity of 82% and 67.6% respectively for the possible criteria. Conclusions: Our data suggest that a reliable diagnosis of CAA-ri can be reached from basic clinical and radiographic information alone with good sensitivity and excellent specificity.

Familial Cerebral Cavernous Malformation Mimicking Cerebral Amyloid Angiopathy

2021 ◽  
pp. 10.1212/CPJ.0000000000001055
Author(s):  
Mohamed Ridha ◽  
Yasmin Aziz ◽  
Joseph Broderick
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Cavernous Malformation ◽  
Pathogenic Mutation ◽  
Cerebral Cavernous Malformation ◽  
Amyloid Angiopathy ◽  
History Of ◽  
Cerebral Amyloid ◽  
Cerebral Microhemorrhages ◽  
Familial Cerebral Cavernous Malformation ◽  
Work Up

A 67-year-old man was referred from ophthalmology for possible cerebral amyloid angiopathy (CAA) discovered during work-up of possible optic neuropathy. MRI (figure 1) demonstrated innumerable periventricular, brainstem, and cortical cerebral microhemorrhages (CMH). Scattered, non-specific white matter hyperintensities was seen on T2-weighted imaging without surrounding hypointense rim. He had no hypertension, and the distribution was uncharacteristic for CAA. Despite absent family history of stroke or seizure, testing for familial cerebral cavernous malformation (FCCM) identified a pathogenic mutation of KRIT1 (c.382G>T).

Vessels Sing Their ARIAs: The Role of Vascular Amyloid in the Age of Aducanumab

Stroke ◽  
2021 ◽  
Author(s):  
Lukas Sveikata ◽  
Andreas Charidimou ◽  
Anand Viswanathan
Keyword(s):  
Clinical Trials ◽  
Alzheimer Disease ◽  
Cerebral Amyloid Angiopathy ◽  
Amyloid Β ◽  
High Rate ◽  
Amyloid Angiopathy ◽  
Cerebrovascular Pathology ◽  
Cerebral Amyloid

We review the implications of the recently approved aducanumab amyloid-β immunotherapy for treating Alzheimer disease with comorbid cerebral amyloid angiopathy. In clinical trials, amyloid-β immunotherapy has been associated with a high rate of amyloid-related imaging abnormalities, potentially driven by coexisting cerebral amyloid angiopathy. Therefore, immunotherapy’s efficacy in patients may be modified by coexisting cerebrovascular pathology. We discuss the contributions of cerebral amyloid angiopathy on the development of amyloid-related imaging abnormalities and propose strategies to identify cerebral amyloid angiopathy in patients considered for immunotherapy.

scholarly journals Hereditary cerebral amyloid angiopathy, Piedmont-type mutation

2020 ◽  
Vol 6 (2) ◽  
pp. e411 ◽  
Author(s):  
Mariel G. Kozberg ◽  
Susanne J. van Veluw ◽  
Matthew P. Frosch ◽  
Steven M. Greenberg
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Ex Vivo ◽  
Amyloid Plaques ◽  
Amyloid Angiopathy ◽  
Pathologic Examination ◽  
Potential Association ◽  
History Of ◽  
Intracerebral Hemorrhages ◽  
Β Amyloid ◽  
Cerebral Amyloid

ObjectiveWe present here a case report of a patient with a family history of intracerebral hemorrhages (ICHs) who presented with multiple large lobar hemorrhages in rapid succession, with cognitive sparing, who was found to have a mutation in the β-amyloid coding sequence of amyloid precursor protein (Leu705Val), termed the Piedmont-type mutation, the second ever reported case of this form of hereditary cerebral amyloid angiopathy (CAA).MethodsTargeted pathologic examination was performed aided by the use of ex vivo MRI.ResultsSevere CAA was observed mainly involving the leptomeningeal vessels and, to a far lesser extent, cortical vessels, with no amyloid plaques or neurofibrillary tangles.ConclusionsThis leptomeningeal pattern of β-amyloid deposition coupled with multiple large hemorrhages demonstrates unique pathophysiologic characteristics of CAA associated with the Piedmont-type mutation, suggesting a potential association between leptomeningeal CAA and larger ICHs.

Cerebrospinal Fluid Anti-Amyloid-β Autoantibodies and Amyloid PET in Cerebral Amyloid Angiopathy-Related Inflammation

2015 ◽  
Vol 50 (1) ◽  
pp. 1-7 ◽  
Author(s):  
María Carmona-Iragui ◽  
Ana Fernández-Arcos ◽  
Daniel Alcolea ◽  
Fabrizio Piazza ◽  
Estrella Morenas-Rodriguez ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Cerebral Amyloid Angiopathy ◽  
Amyloid Β ◽  
Amyloid Angiopathy ◽  
Amyloid Pet ◽  
Cerebral Amyloid

scholarly journals Diagnostic Utility of Amyloid PET in Cerebral Amyloid Angiopathy-Related Symptomatic Intracerebral Hemorrhage

2014 ◽  
Vol 34 (5) ◽  
pp. 753-758 ◽  
Author(s):  
Jean-Claude Baron ◽  
Karim Farid ◽  
Eamon Dolan ◽  
Guillaume Turc ◽  
Siva T Marrapu ◽  
...  
Keyword(s):  
Intracerebral Hemorrhage ◽  
Cerebral Amyloid Angiopathy ◽  
Visual Analysis ◽  
Diagnostic Value ◽  
Amyloid Angiopathy ◽  
Amyloid Pet ◽  
Healthy Elderly ◽  
Symptomatic Intracerebral Hemorrhage ◽  
Significant Difference ◽  
Cerebral Amyloid

By detecting β-amyloid ( Aβ) in the wall of cortical arterioles, amyloid positron emission tomography (PET) imaging might help diagnose cerebral amyloid angiopathy (CAA) in patients with lobar intracerebral hemorrhage (I-ICH). No previous study has directly assessed the diagnostic value of 11-Pittsburgh compound B (PiB) PET in probable CAA-related I-ICH against healthy controls (HCs). 11C-PiB-PET and magnetic resonance imaging (MRI) including T2* were obtained in 11 nondemented patients fulfilling the Boston criteria for probable CAA-related symptomatic I-ICH (sl-ICH) and 20 HCs without cognitive complaints or impairment. After optimal spatial normalization, cerebral spinal fluid (CSF)-corrected PiB distribution volume ratios (DVRs) were obtained. There was no significant difference in whole cortex or regional DVRs between CAA patients and age-matched HCs. The whole cortex DVR was above the 95% confidence limit in 4/9 HCs and 10/11 CAA patients (sensitivity = 91%, specificity = 55%). Region/frontal or occipital ratios did not have better discriminative value. Similar but less accurate results were found using visual analysis. In patients with sl-ICH, 11C-PiB-PET has low specificity for CAA due to the frequent occurrence of high 11C-PiB uptake in the healthy elderly reflecting incipient Alzheimer's disease (AD), which might also be present in suspected CAA. However, a negative PiB scan rules out CAA with excellent sensitivity, which has clinical implications for prognostication and selection of candidates for drug trials.

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