Aura attacks from acute convexity subarachnoid haemorrhage not due to cerebral amyloid angiopathy

Background and purpose: Convexity subarachnoid haemorrhage (cSAH) has recently been recognised as a cause of recurrent aura-like symptoms, mimicking transient ischaemic attacks (TIAs). Subarachnoid haemorrhage and recurrent aura-like episodes can occur in patients with cerebral amyloid angiopathy (CAA), which has been the presumed cause in the majority of reported cases. However, this syndrome can occur following cSAH secondary to other conditions, and it is important for clinicians to investigate and manage such patients appropriately. Method: Case series. Results: We describe two patients who presented with recurrent stereotyped transient neurological symptoms in the setting of acute cSAH identified on MRI. In one patient, SAH occurred secondary to cerebral venous sinus thrombosis. In the other, SAH was due to extension of a traumatic subdural haematoma. Conclusions: Conditions other than CAA can cause the clinicoradiological syndrome of cSAH with recurrent TIA-like events. Gradient echo or susceptibility-weighted imaging should be included in the diagnostic work-up of patients presenting with such events. When cSAH is detected, the full differential diagnosis for this should be considered. Aetiologies other than CAA can cause this syndrome and management can vary greatly depending on the underlying cause.

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Erratum to: Convexity subarachnoid haemorrhage has a high risk of intracerebral haemorrhage in suspected cerebral amyloid angiopathy

Journal of Neurology ◽

10.1007/s00415-017-8428-9 ◽

2017 ◽

Vol 264 (4) ◽

pp. 674-674 ◽

Cited By ~ 1

Author(s):

D. Wilson ◽

I. C. Hostettler ◽

G. Ambler ◽

G. Banerjee ◽

H. R. Jäger ◽

...

Keyword(s):

High Risk ◽

Subarachnoid Haemorrhage ◽

Cerebral Amyloid Angiopathy ◽

Intracerebral Haemorrhage ◽

Amyloid Angiopathy ◽

Cerebral Amyloid

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Familial Cerebral Cavernous Malformation Mimicking Cerebral Amyloid Angiopathy

Neurology Clinical Practice ◽

10.1212/cpj.0000000000001055 ◽

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).

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Convexity subarachnoid haemorrhage has a high risk of intracerebral haemorrhage in suspected cerebral amyloid angiopathy

Journal of Neurology ◽

10.1007/s00415-017-8398-y ◽

2017 ◽

Vol 264 (4) ◽

pp. 664-673 ◽

Cited By ~ 17

Author(s):

D. Wilson ◽

I. C. Hostettler ◽

G. Ambler ◽

G. Banerjee ◽

H. R. Jäger ◽

...

Keyword(s):

High Risk ◽

Subarachnoid Haemorrhage ◽

Cerebral Amyloid Angiopathy ◽

Intracerebral Haemorrhage ◽

Amyloid Angiopathy ◽

Cerebral Amyloid

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Isolated Central Sulcus Hemorrhage: A Rare Presentation Most Frequently Associated with Cerebral Amyloid Angiopathy

Case Reports in Radiology ◽

10.1155/2012/574849 ◽

2012 ◽

Vol 2012 ◽

pp. 1-5

Author(s):

Murthy R. Chamarthy ◽

Yogesh Kumar ◽

Michael D. Meszaros ◽

Ankit Shah ◽

Mark A. Rosovsky

Keyword(s):

Computed Tomography ◽

Elderly Patient ◽

Cerebral Amyloid Angiopathy ◽

Imaging Finding ◽

Amyloid Angiopathy ◽

Central Sulcus ◽

Rare Presentation ◽

Cerebral Amyloidosis ◽

Cerebral Amyloid ◽

Work Up

Central sulcus hemorrhage is a rare imaging finding that can be related to cerebral amyloidosis in a normotensive non-traumatic elderly patient and present as an isolated finding or in association with other areas of involvement. We report a case presenting with an isolated central sulcus hemorrhage on computed tomography. Further imaging work-up excluded other potential causes of peripheral hemorrhages and established a putative diagnosis of cerebral amyloidosis.

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The diagnosis challenge of cerebral amyloid angiopathy: case series

Amyloid ◽

10.3109/13506129.2011.574354079 ◽

2011 ◽

Vol 18 (sup1) ◽

pp. 211-213

Author(s):

S. Vollaro ◽

D. Landi ◽

M. Di Girolamo ◽

F. Passarelli ◽

P. M. Rossini ◽

...

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Case Series ◽

Amyloid Angiopathy ◽

Cerebral Amyloid

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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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Finger-Like Projections in Lobar Haemorrhage on Early Magnetic Resonance Imaging Is Associated with Probable Cerebral Amyloid Angiopathy

Cerebrovascular Diseases ◽

10.1159/000499032 ◽

2019 ◽

Vol 47 (3-4) ◽

pp. 121-126 ◽

Cited By ~ 1

Author(s):

Dimitri Renard ◽

Teodora Parvu ◽

Eric Thouvenot

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Field Strength ◽

Subarachnoid Haemorrhage ◽

Cerebral Amyloid Angiopathy ◽

Amyloid Angiopathy ◽

Resonance Imaging ◽

Mri Scans ◽

Cerebral Amyloid ◽

Magnetic Resonance Imaging Mri

Background: Recently, finger-like projections (FLP) and subarachnoid haemorrhage extension (SAHE) of lobar intracerebral haemorrhage (LH) on acute CT together with ApoE4 genotype have been used in a prediction model for histopathologically proven cerebral amyloid angiopathy (CAA). Our aim was to analyse FLP and SAHE on acute/early subacute magnetic resonance imaging (MRI) and to assess the association with probable CAA diagnosis according to modified Boston criteria. Methods: We retrospectively studied MRI scans (and CT if available) performed <7 days in a cohort of consecutive acute LH patients >55 years. Results: Forty-six patients (24 men and 22 women; mean age 73; 28 probable and 18 possible CAA patients) were analysed. Mean symptom onset-MRI delay was 1.3 days (including 26 patients with MRI <24 h). Both probable and possible CAA groups were comparable regarding age, sex, time MRI and CT performance, MRI field strength, and LH volume. On MRI, both FLP and SAHE were observed more frequently in probable than in possible CAA (FLP 43 vs. 6%, p = 0.0073; SAHE 79 vs. 44%, p = 0.027), and associated with larger LH volumes (FLP, p = 0.011; SAHE, p = 0.047). FLP was associated with earlier performed MRI (mean 0.3 vs. 1.75 days, p = 0.025). In the subgroup of 35 patients with available CT (performed a mean of 2.2 days before or after MRI), FLP presence on CT was observed more frequently in probable than in possible CAA (57 vs. 7%, p = 0.0039). Concordance of MRI and CT for FLP presence/absence was 89%. Conclusions: In acute LH patients, FLP and SAHE on acute/early subacute phase MRI are associated with probable CAA diagnosis. Larger LH volumes are associated with FLP and SAHE on MRI, and early performed MRI with FLP.

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Cerebral amyloid angiopathy with related inflammation masquerading as crescendo transient ischaemic attacks

Practical Neurology ◽

10.1136/practneurol-2021-003223 ◽

2021 ◽

pp. practneurol-2021-003223

Author(s):

Duncan Maddox ◽

Kayla Ward ◽

Thomas Robertson ◽

Mike Boggild

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Neurological Disorders ◽

Antithrombotic Therapy ◽

Neurological Symptoms ◽

Amyloid Angiopathy ◽

Transient Ischaemic Attacks ◽

Cerebral Biopsy ◽

Cerebral Amyloid ◽

Capsular Warning Syndrome

Cerebral amyloid angiopathy with related inflammation (CAA-RI) is an uncommon inflammatory subtype of CAA, with a variety of presentations that can mimic other focal and diffuse neurological disorders. We present a 63-year-old man with recurrent stereotyped focal neurological symptoms, who was initially diagnosed as capsular warning syndrome and treated with antithrombotic therapy. Atypical imaging led to further investigation including a cerebral biopsy, which confirmed CAA-RI; he improved clinically and radiologically with immunosuppression. This case highlights how CAA-RI is often under-recognised and that patients risk receiving inappropriate anticoagulation and delay in starting immunosuppression.

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Abstract 3208: Sensitivity and Reliability of SWI Compared to T2* GRE MRI for Detection of Microbleeds in Cerebral Amyloid Angiopathy

Stroke ◽

10.1161/str.43.suppl_1.a3208 ◽

2012 ◽

Vol 43 (suppl_1) ◽

Author(s):

Ah-Ling Cheng ◽

Cheryl R McCreary ◽

M. L Lauzon ◽

Richard Frayne ◽

Mayank Goyal ◽

...

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Small Vessel Disease ◽

Intraclass Correlation ◽

Case Series ◽

Cerebral Small Vessel Disease ◽

Small Vessel ◽

Amyloid Angiopathy ◽

Healthy Controls ◽

Vessel Disease ◽

Cerebral Amyloid

Introduction: Case examples and small case series suggest that MRI susceptibility weighted imaging (SWI) may be more sensitive for cerebral microbleed (CMB) detection compared to MRI T2* gradient-recalled echo (GRE). However, there are few data on CMB counts measured by SWI vs. GRE, or inter-rater reliability, in groups of patients with cerebral small vessel disease. We used data from a prospective cohort study of cerebral amyloid angiopathy (CAA), a cerebral small-vessel disease marked by high numbers of CMBs, to quantify the sensitivity and reliability of SWI vs. GRE for CMB detection. Methods: Nine patients with symptomatic CAA (mean age 71±8.3; 7 males and 2 females) and 21 healthy non-CAA controls (mean age 68±6.3; 10 M/11 F) underwent T2* GRE and SWI on a 3.0T MR scanner. Probable CAA was diagnosed according to the Boston criteria prior to study entry using information from clinical MRI with GRE sequences. Two raters (labeled 1 and 2) independently interpreted the GRE and SWI scans blinded to clinical information. The phase-filtered magnitude image was used for SWI interpretation. Agreement reliability was assessed using the kappa coefficient (where a kappa of ≥0.60 indicates good agreement) or the intraclass correlation coefficient (ICC). Results: Overall, the raters identified 1,432 CMBs in the 9 CAA cases (range 1-434 per patient) and 8 CMBs in the healthy controls (range 0-3). Rater 1 identified CMBs in 5/21 healthy controls on SWI and 5/21 on GRE, while rater 2 identified CMBs in 4/21 on SWI and 3/21 on GRE (kappa 0.70 for GRE and 0.57 for SWI). In CAA cases more CMBs were seen on SWI compared to the GRE sequence but the difference was significant only for rater 1 (rater 1: on average 85% more per patient on SWI than on GRE, p=0.008; rater 2: 19% more, p=0.25). Among CAA cases the reliability between raters was poor for GRE (ICC 0.36) but excellent for SWI (0.94, p<0.05 for comparison with GRE). Review suggested that the differing reliability was because rater 1 was less likely than rater 2 to identify faint lesions on GRE as CMB, whereas these lesions were more conspicuous on SWI. If SWI rather than GRE were used to determine CAA status according to the Boston criteria, all 9 CAA cases would remain classified as probable CAA but 2/21 controls would be reclassified as either possible (n=1) or probable (n=1) asymptomatic CAA based on the detection of one or more lobar microbleeds on SWI. Conclusions: SWI confers greater reliability as well as greater sensitivity for CMB detection compared to GRE, and should be the preferred sequence for quantifying CMBs. SWI may more frequently identify lobar microbleeds that could represent asymptomatic CAA. Further research is needed to determine whether the Boston criteria require revision to take into account the greater sensitivity of SWI for CMB detection.

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