Cerebral amyloid angiopathy-related transient focal neurological episodes: A transient ischemic attack mimic with an increased risk of intracranial hemorrhage

Background and Purpose: The general cardiovascular Framingham risk score (FRS) identifies adults at increased risk for stroke. We tested the hypothesis that baseline FRS is associated with the presence of postmortem cerebrovascular disease (CVD) pathologies. Methods: We studied the brains of 1672 older decedents with baseline FRS and measured CVD pathologies including macroinfarcts, microinfarcts, atherosclerosis, arteriolosclerosis, and cerebral amyloid angiopathy. We employed a series of logistic regressions to examine the association of baseline FRS with each of the 5 CVD pathologies. Results: Average age at baseline was 80.5±7.0 years and average age at death was 89.2±6.7 years. A higher baseline FRS was associated with higher odds of macroinfarcts (odds ratio, 1.10 [95% CI, 1.07–1.13], P <0.001), microinfarcts (odds ratio, 1.04 [95% CI, 1.01–1.07], P =0.009), atherosclerosis (odds ratio, 1.07 [95% CI, 1.04–1.11], P <0.001), and arteriolosclerosis (odds ratio, 1.04 [95% CI, 1.01–1.07], P =0.005). C statistics for these models ranged from 0.537 to 0.595 indicating low accuracy for predicting CVD pathologies. FRS was not associated with the presence of cerebral amyloid angiopathy. Conclusions: A higher FRS score in older adults is associated with higher odds of some, but not all, CVD pathologies, with low discrimination at the individual level. Further work is needed to develop a more robust risk score to identify adults at risk for accumulating CVD pathologies.

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Intracranial hemorrhage: cerebral amyloid angiopathy or nasal decongestants?

JAMA ◽

10.1001/jama.264.17.2213 ◽

1990 ◽

Vol 264 (17) ◽

pp. 2213-2214

Author(s):

M. I. Weintraub

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Intracranial Hemorrhage ◽

Amyloid Angiopathy ◽

Nasal Decongestants ◽

Cerebral Amyloid

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Amyloid Spells and High Blood Pressure: Imminent Danger

Case Reports in Neurology ◽

10.1159/000369922 ◽

2014 ◽

Vol 7 (1) ◽

pp. 39-43 ◽

Cited By ~ 1

Author(s):

Andre Caetano ◽

Miguel Pinto ◽

Sofia Calado ◽

Miguel Viana-Baptista

Keyword(s):

Blood Pressure ◽

High Blood Pressure ◽

Cerebral Amyloid Angiopathy ◽

Antiplatelet Agents ◽

Pressure Profile ◽

Clonic Seizure ◽

Superficial Siderosis ◽

Amyloid Angiopathy ◽

Increased Risk ◽

Cerebral Amyloid

We present the case of a 71-year-old male, admitted after a generalized tonic-clonic seizure, with a history of recurrent left arm and face paresthesias, associated with sulcal cortical subarachnoid hemorrhages. During the next 48 h, he remained agitated with a high blood pressure profile; he also suffered a cardiac arrest in relation to a severe left fronto-parietal and a smaller right parietal parenchymal hemorrhage that developed over the subarachnoid hemorrhage locations. There were no intracranial vascular abnormalities. Three months later, an MRI revealed disseminated superficial siderosis. He was discharged with a modified Rankin scale of 4. He died 1 month later of unknown cause. A diagnosis of probable cerebral amyloid angiopathy was assumed. Patients with pathologically proven cerebral amyloid angiopathy that present with transient focal neurological symptoms in relation to cortical bleeds, the so-called ‘myloid spells' seem to be at an increased risk of future parenchymal hemorrhages. Avoiding antiplatelet agents in these cases has been proposed. Our case suggests that these patients should be monitored closely in the hyperacute phase , and tight blood pressure control should be considered as the immediate risk of bleeding may be high, even without a definitive diagnosis of cerebral amyloid angiopathy.

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Cerebral amyloid angiopathy–associated intracerebral hemorrhage: pathology and management

Neurosurgical FOCUS ◽

10.3171/2012.1.focus11370 ◽

2012 ◽

Vol 32 (4) ◽

pp. E7 ◽

Cited By ~ 46

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.

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Cerebral Amyloid Angiopathy with Cortical Subarachnoid Hemorrhage as a Mimic for Transient Ischemic Attack: A Case Report

Archives of Neuroscience ◽

10.5812/ans.111362 ◽

2021 ◽

Vol 8 (2) ◽

Author(s):

Mazyar Hashemilar ◽

Nasrin Forghani

Keyword(s):

Subarachnoid Hemorrhage ◽

Cerebral Amyloid Angiopathy ◽

Clinical Findings ◽

Amyloid Angiopathy ◽

Cortical Region ◽

Transient Ischemic Attacks ◽

Life Threatening ◽

Cerebral Amyloid ◽

Ischemic Attack ◽

Frontal Cortical Region

Context: Given its cerebral amyloid angiopathy, subarachnoid hemorrhage might represent transient focal neurological episodes erroneously diagnosed as transient ischemic attacks. The earliest neuroimaging findings in emergency room brain computed tomography indicating subarachnoid hemorrhage in these patients might be very subtle and missed by the clinician. Case Presentation: An 80-year-old man referred with transient focal neurological episodes, suggestive of transient ischemic attacks. In general, except for some cognitive dysfunctions, no remarkable point was noticed in his neurological examination. Non-enhanced brain-computer tomography and magnetic resonance imaging revealed evidence indicating slight convexity subarachnoid hemorrhage at the left frontal cortical region. Conclusions: The transient focal neurological episodes uncommonly represent intracranial hemorrhage. Nevertheless, this clinical representation might occur in patients with subarachnoid hemorrhage due to cerebral amyloid angiopathy. In such cases, the neuroimaging findings play a major role in the differential diagnosis. The misdiagnosis of transient ischemic attacks in these cases might lead to the consumption of antiplatelet drugs and end in catastrophic hemorrhage and life-threatening complications. Close attention to patients' clinical findings and judicious use of further neuroimaging studies would help clinicians to avoid making such mistakes.

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

Age and Ageing ◽

10.1093/ageing/afz103.122 ◽

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.

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Abstract W P257: Predictors of the CT Angiography Spot Sign in Deep and Lobar Intracerebral Hemorrhage

Stroke ◽

10.1161/str.45.suppl_1.wp257 ◽

2014 ◽

Vol 45 (suppl_1) ◽

Author(s):

Farid Radmanesh ◽

Guido J Falcone ◽

Christopher D Anderson ◽

Thomas W Battey ◽

Alison M Ayres ◽

...

Keyword(s):

Logistic Regression ◽

Intracerebral Hemorrhage ◽

Ct Angiography ◽

Cerebral Amyloid Angiopathy ◽

Symptom Onset ◽

Hematoma Expansion ◽

Amyloid Angiopathy ◽

Spot Sign ◽

Increased Risk ◽

Cerebral Amyloid

Objectives: Intracerebral hemorrhage (ICH) patients with CT angiography (CTA) spot sign are at increased risk of hematoma expansion and poor outcome. Since ICH is often the acute manifestation of a chronic cerebral vasculopathy, we investigated whether different clinical or imaging characteristics predict spot sign presence in patients with different underlying vasculopathies. Using ICH location as a surrogate for hypertension-related ICH and cerebral amyloid angiopathy-related ICH, we identified risk factors associated with spot sign. METHODS: We retrospectively analyzed a prospective cohort of consecutive spontaneous ICH patients with available CTA. Spot sign presence was ascertained by two independent readers blinded to clinical data. We assessed potential predictors of spot sign be performing uni- and multivariable logistic regression, analyzing deep and lobar ICH separately. RESULTS: 649 patients were eligible, 291 (45%) deep and 358 (55%) lobar ICH. Median time from symptom onset to CTA was 4.5 (IQR 5.2) and 5.7 (IQR 7.4) hours in patients with deep and lobar ICH, respectively. At least one spot sign was present in 76 (26%) deep and 103 (29%) lobar ICH patients. In mutivariable logistic regression, independent predictors of spot sign in deep ICH were warfarin (OR 2.82 [95%CI 1.06-7.57]; p=0.03), time from symptom onset to CTA (OR 0.9 [95%CI 0.81-0.97]; p=0.02), and baseline ICH volume (OR 1.27 [95%CI 1.14-1.43]; p=2.5E-5; per 10 mL increase). Predictors of spot sign in lobar ICH were preexisting dementia (OR 2.7 [95%CI 1.15-6.43]; p=0.02), warfarin (OR 4.01 [95%CI 1.78-9.29]; p=0.009), and baseline ICH volume (OR 1.27 [95%CI 1.17-1.39]; p=5.4E-8; per 10 mL increase). As expected, spot sign presence was a strong predictor of hematoma expansion in both deep (OR 3.52 [95%CI 1.72-7.2]; p=0.0005) and lobar ICH (OR 6.53 [95%CI 3.23-13.44]; p=2.2E-7). CONCLUSIONS: The most potent associations with spot sign are shared by deep and lobar ICH, suggesting that ICH caused by different vasculopathic processes share biological features. The relationship between preexisting dementia and spot sign in lobar ICH, but not deep ICH, suggests that ICH occurring in the context of more advanced cerebral amyloid angiopathy may be more likely to have prolonged bleeding.

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