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).

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.

scholarly journals Early MRI imaging and follow-up study in cerebral amyloid angiopathy

Open Medicine ◽  
2021 ◽  
Vol 16 (1) ◽  
pp. 257-263
Author(s):  
Shan-chun Zhang ◽  
Jian-jun Jia ◽  
Heng-li Zhao ◽  
Bo Zhou ◽  
Wei Wang ◽  
...  
Keyword(s):  
Recurrence Rate ◽  
Cerebral Amyloid Angiopathy ◽  
Superficial Siderosis ◽  
Control Group ◽  
Amyloid Angiopathy ◽  
Imaging Features ◽  
Follow Up Study ◽  
Imaging Characteristics ◽  
Cerebral Amyloid

Abstract Aim To study the imaging features of leukoaraiosis (LA) and hemorrhage in cerebral amyloid angiopathy (CAA) patients. Methods The earliest MRI images of probable CAA patients and non-CAA patients were collected. The characteristics of LA in the two groups were analyzed. Cerebral micro bleeding (CMB), superficial siderosis (SS), and intracranial hemorrhage (ICH) were recorded in the follow-up study. The space relationship between CMB or SS and ICH was assessed. Results We found that 10/21 (47.6%) patients had occipital prominent LA and 14/21 (66.7%) patients had subcortical punctate LA before the ICH, which was higher than that of the ones in the control group (p = 0.015 and 0.038, respectively). The recurrence rate of ICH was 100% (3/3) in patients with diffuse SS and 36.4% (4/11) in patients without. The recurrence rate of ICH was 60% (3/5) in patients with multiple-lobe CMBs and 44.4% (4/9) in those without. The location of the ICH and CMB was inconsistent. ICH occurred in the ipsilateral cerebral hemisphere of SS in three patients with diffuse SS. Conclusion LA, diffuse SS, and multiple-lobe CMBs are important imaging characteristics of CAA, which may help make early diagnosis and predict the recurrence of ICH.

scholarly journals A Case of Cerebral Amyloid Angiopathy-related Inflammation with Multiple Microbleeds: Diagnosis by Brain Biopsy

2017 ◽  
Vol 106 (4) ◽  
pp. 820-827
Author(s):  
Seitaro Nohara ◽  
Akiko Ishii ◽  
Atsushi Ueda ◽  
Shoko Seki ◽  
Eiichi Oguni ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Brain Biopsy ◽  
Amyloid Angiopathy ◽  
Cerebral Amyloid

scholarly journals Cerebral Amyloid Angiopathy: Multiple neurolgic problems

2017 ◽  
Vol 1 (3) ◽  
pp. 01-01
Author(s):  
Paul Gilbert
Keyword(s):  
White Matter ◽  
Cerebral Amyloid Angiopathy ◽  
Clinical Symptoms ◽  
Brain Biopsy ◽  
Acute Onset ◽  
Amyloid Angiopathy ◽  
Migraine Headaches ◽  
Diffuse White Matter ◽  
Cerebral Amyloid ◽  
Intravenous Steroids

A 72-Year-old female with a history of migraine headaches presented with an acute onset of expressive aphasia, difficulty with memory and worsening of her headaches. An MRI of the brain was done which revealed diffuse white matter T2 hyperintensities (Figures 1). Due to worsening of the patient’s clinical symptoms a repeat MRI was performed four days later that revealed multiple micro-bleeds (Figure 2), as well as a lobar hemorrhage in left temporal lobe (Figure 2). An extensive workup including HIV testing, CSF examination for infectious etiology including protein 14-3-3 and demylineating disease was negative. Paraneoplastic and autoimmune workup was also non-diagnostic. A brain biopsy was performed due to the extensive white matter disease, which revealed Cerebral Amyloid Angiopathy (CAA) with focal granulomatous angiitis. The patient was treated with intravenous steroids with no significant improvement clinically. Two months after diagnosis, her disease course has remained static, without improvement or deterioration.

scholarly journals Spontaneous ARIA-like Events in Cerebral Amyloid Angiopathy–Related Inflammation: A Multicenter Prospective Longitudinal Cohort Study

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012778
Author(s):  
Laura Antolini ◽  
Jacopo C. DiFrancesco ◽  
Marialuisa Zedde ◽  
Gianpaolo Basso ◽  
Andrea Arighi ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Cerebral Amyloid Angiopathy ◽  
Pulse Therapy ◽  
Amyloid Angiopathy ◽  
Longitudinal Cohort ◽  
History Of ◽  
Corticosteroid Pulse Therapy ◽  
Cerebral Amyloid

Background and Objectives:To investigate the natural history and outcomes following treatment for spontaneous amyloid-related imaging abnormalities (ARIA)-like in cerebral amyloid angiopathy-related inflammation (CAA-ri).Methods:A multicenter, hospital-based, longitudinal, prospective observational study of inpatients meeting CAA-ri diagnostic criteria, recruited through the iCAβ International Network, in the period January 2013 - March 2017. A protocol for systematic data collection at first-ever presentation and at subsequent in-person visits, including T1-weighted, GRE-T2*, fluid-suppressed T2-weighted (FLAIR), and T1 post-gadolinium contrast-enhancement images aquired on 1.5T MRI, was employed at 3, 6, 12, 24-months follow-up. Centralized reads of MRI images were performed blinded to clinical, therapeutic, and time-points information. Main outcomes were survival, clinical and radiological recovery, intracerebral hemorrhage (ICH), and recurrence of CAA-ri.Results:The study enrolled 113 participants (10.6% definite, 71.7% probable, and 17.7% possible CAA-ri), mean age 72.9 years, 43.4% female, 37.1% APOEε4 carriers. 36.3% had a history of Alzheimer’s disease, 33.6% of ICH. A history of ICH, as well as the occurrence of new ICH at follow-up, was more common in patients with cortical superficial siderosis at baseline (52.6% vs 14.3%; p< 0.0001 and 19.3% vs 3.6%; p<0.009, respectively). After the first-ever presentation of CAA-ri, 70.3% (95% CI, 61.6-78.5) and 84.1% (95% CI, 76.2-90.6) clinically recovered within three and twelve months, followed by radiological recovery in 45.1% (95% CI, 36.4 - 54.8) and 77.4% (95% CI, 67.7 - 85.9), respectively. After clinicoradiological resolution of the first-ever episode, 38,3% (95% CI, 22.9 - 59.2) had at least one recurrence within the following 24 months. Recurrence was more likely if intravenous high dose corticosteroid pulse therapy was suddenly stopped compared to slow oral tapering-off (Hazard Ratio 4.68; 95% CI, 1.57-13.93; p=0.006).Discussion:These results from the largest longitudinal cohort registry of patients with CAA-ri support the transient and potentially relapsing inflammatory nature of the clinical-radiological acute manifestations of the disease and the effectiveness of slow oral tapering-off after intravenous corticosteroid pulse therapy in preventing recurrences. Our results highlight the importance of differential diagnosis for spontaneous ARIA-like events in Aβ-driven diseases, including treatment-related ARIA in Alzheimer’s disease patients exposed to immunotherapy drugs.

scholarly journals 204 Clinicoradiological Diagnosis of Cerebral Amyloid Angiopathy-Related Inflammation (CAA-ri) - a Non-Invasive Approach to Diagnosis and Management

2019 ◽  
Vol 48 (Supplement_3) ◽  
pp. iii17-iii65
Author(s):  
Deirdre McCartan ◽  
David Williams ◽  
Barry Moynihan ◽  
Karl Boyle
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Epileptiform Activity ◽  
Brain Biopsy ◽  
Cognitive Change ◽  
Cognitive Testing ◽  
Amyloid Angiopathy ◽  
Related Disorder ◽  
Invasive Approach ◽  
Increased Risk ◽  
Cerebral Amyloid

Abstract Background Cerebral Amyloid Angiopathy (CAA) is an age-related disorder characterised by deposition of beta-amyloid protein in the walls of small and medium cortical vessels leading to increased risk of intracranial bleeding. CAA-Related Inflammation (CAA-ri) is an under recognised subtype of CAA potentially responsive to immunosuppression and traditionally diagnosed by invasive brain biopsy. CAA-ri is associated with rapid cognitive decline but shows reversibility for some when treated with immunosuppression. We present the case of an 82 year old lady who presented with first seizure, a history of notable cognitive change and neuroimaging consistent with probable CAA-ri. Methods Validated clinicoradiological diagnostic criteria for CAA-ri were applied to MRI T2 FLAIR and SWI sequences. CSF, APOE genotyping, EEG and cognitive testing were performed. Interdisciplinary perspectives were sought from Neurology, Neurosurgery and Infectious Diseases colleagues. Consensus opinion opposed brain biopsy on strength of imaging evidence and pulsed intravenous steroid treatment was initiated. BP, anti-convulsant and bone protection were optimised and anti-thrombotics avoided. Repeat imaging and cognitive testing were repeated after four months. Results MRI T2-FLAIR revealed an asymmetric multifocal distribution of cortical and subcortical white matter hyperintensities (WMH) with leptomeningeal enhancement while SWI showed extensive multifocal microhaemorrhages with confluent haemorrhage in the right frontal and temporal regions. EEG demonstrated right frontal theta slowing and absence of epileptiform activity. CSF analysis reported raised protein at 53mg/dl. Normal WCC. Formal cognitive testing with ACEIII revealed a score of 79/100. EPOA was advised. Conclusion Clinicoradiological diagnosis of CAA-ri permits early initiation of immunosuppressive therapy and avoids invasive brain biopsy. In the absence of clinical suspicion and blood sensitive imaging sequences CAA-ri may be misdiagnosed as Acute Ischaemic Stroke or TIA where the addition of anti-thrombotic therapy could cause harm while early medical management offers potential reversibility.

Infant critical head injury could be a remote cause of middle-aged cerebral amyloid angiopathy

2019 ◽  
Author(s):  
Keisuke Tachiyama ◽  
Masahiro Nakamori ◽  
Yuki Hayashi ◽  
Hayato Matsushima ◽  
Eiji Imamura ◽  
...  
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
The Elderly ◽  
Amyloid Angiopathy ◽  
Resonance Imaging ◽  
Middle Aged ◽  
Case Presentation ◽  
Csf Analysis ◽  
History Of ◽  
Lobar Hemorrhage ◽  
Cerebral Amyloid

Abstract Background Cerebral amyloid angiopathy (CAA) is a sporadic condition in the elderly and is associated with Alzheimer’s disease. The younger cases, however, may have a history of traumatic brain injury (TBI) during infancy. Case Presentation We present a case of a 37-year-old man who had cerebral lobar hemorrhage. Magnetic resonance imaging revealed several lobar microbleeds, which along with the asymptomatic, lobar hemorrhages increased every year. At the age of 40 years, he developed mild cognitive impairment. Cerebrospinal fluid (CSF) analysis revealed a markedly decreased level of amyloid β42. Moreover, he had a subdural hematoma during infancy. Thus, we diagnosed him with CAA, which was related to the TBI at infancy. Conclusion TBI at infancy can be a remote cause of middle-aged CAA and dementia. This was supported by the low Aβ42 level in the CSF analysis.

scholarly journals A case of inflammatory cerebral amyloid angiopathy with white matter lesions appearing after brain biopsy

2021 ◽  
Vol 61 (3) ◽  
pp. 188-193
Author(s):  
Yosuke Takeuchi ◽  
Shuei Murahashi ◽  
Yasuyuki Hara ◽  
Makoto Nakajima ◽  
Mitsuharu Ueda
Keyword(s):  
White Matter ◽  
Cerebral Amyloid Angiopathy ◽  
White Matter Lesions ◽  
Brain Biopsy ◽  
Amyloid Angiopathy ◽  
Cerebral Amyloid
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