The diagnosis challenge of cerebral amyloid angiopathy: case series

Amyloid ◽  
2011 ◽  
Vol 18 (sup1) ◽  
pp. 211-213
Author(s):  
S. Vollaro ◽  
D. Landi ◽  
M. Di Girolamo ◽  
F. Passarelli ◽  
P. M. Rossini ◽  
...  
2012 ◽  
Vol 32 (4) ◽  
pp. E7 ◽  
Author(s):  
Prachi Mehndiratta ◽  
Sunil Manjila ◽  
Thomas Ostergard ◽  
Sylvia Eisele ◽  
Mark L. Cohen ◽  
...  

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.


Stroke ◽  
2012 ◽  
Vol 43 (suppl_1) ◽  
Author(s):  
Ah-Ling Cheng ◽  
Cheryl R McCreary ◽  
M. L Lauzon ◽  
Richard Frayne ◽  
Mayank Goyal ◽  
...  

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.


2021 ◽  
Vol 429 ◽  
pp. 118793
Author(s):  
Fulvio Pasquin ◽  
Valentina Tommasini ◽  
Alessandro Dinoto ◽  
Francesco Biaduzzini ◽  
Laura D'Acunto ◽  
...  

2020 ◽  
Vol 19 (2) ◽  
pp. 74
Author(s):  
Yun Jeong Hong ◽  
Si Baek Lee ◽  
Seong Hoon Kim ◽  
Dong Woo Ryu ◽  
Yongbang Kim ◽  
...  

Cephalalgia ◽  
2010 ◽  
Vol 31 (3) ◽  
pp. 368-371 ◽  
Author(s):  
DK Field ◽  
TJ Kleinig

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.


2021 ◽  
pp. 1-5
Author(s):  
Laura Michiels ◽  
Donatienne Van Weehaeghe ◽  
Rik Vandenberghe ◽  
Jelle Demeestere ◽  
Koen Van Laere ◽  
...  

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.


PM&R ◽  
2017 ◽  
Vol 9 ◽  
pp. S259-S260
Author(s):  
Atira H. Kaplan ◽  
Maria A. Jouvin-Castro ◽  
Gary N. Inwald

Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Sanjay Mittal ◽  
Karan Tarasaria

Background: Inflammatory Cerebral Amyloid Angiopathy (iCAA) is a rare cause of rapidly progressive cognitive decline. Prior studies have proposed radiological diagnostic criteria and demonstrated evidence of inflammation related to Aβ. Though lacking any therapeutic trials, various authors have suggested a single pulse IV Methyl Prednisolone (MPS) therapy as curative in most patients. Methods: We studied clinical, radiological and neuropathological features of 2 of biopsy proven iCAA cases over the past 5 years correlating their neuropathological and radiological features. We also reviewed published literature for radiological findings of proven cases of ICAA Clinical features Case 1: 75/female presented with visual hallucinations, her neurological examination was un-remarkable >Case 2: 84/female presented with language and memory difficulties her neurological examination was remarkable for word finding and object naming difficulties Radiological Features: Gadolinium enhanced brain MRI with fluid attenuated inversion recovery (FLAIR) and susceptibility weighted imaging (SWI) were obtained Patient 1: SWI artifact s in bilateral cerebral hemispheres basal ganglia and cerebellum suggestive of cortical micro-bleeds (CMBs ) there was a cluster of CMBs in the right parietal region associated with FLAIR changes and gadolinium enhancement Patient 2 numerous scattered cortical micro-bleeds (CMBs). There was a large cluster of CMBs in left temporal lobe along with FLAIR changes and gadolinium enhancement Biopsy: B oth patient underwent lesional biopsy corresponding to the area of clustered CMBs Neuropathology: demonstrated histopathological and electron microscopic evidence of perivascular inflammation and amyloid deposition consistent with iCAA Review of literature : In a total of 7 brain MRI s in 3 recently published case series 4 had reported CMBs of which 3 patients were identified representing MRI findings consistent with Grouped CMBs associated with FLAIR changes Conclusion: iCAA is a rare and treatable cause of subacute cognitive decline. We suggest grouped cortical micro bleeds (GCMBs) associated with FLAIR changes as a unique radiological feature of this disorder.


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