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World Neurosurgery Jun 2020Management of cerebral amyloidomas has lacked consensus owing to their rarity. We present a case and review 39 reported cases of amyloidomas in the literature, comparing...
BACKGROUND
Management of cerebral amyloidomas has lacked consensus owing to their rarity. We present a case and review 39 reported cases of amyloidomas in the literature, comparing their initial presentation, imaging characteristics, treatment, and progression.
CASE DESCRIPTION
A 56-year-old woman presented with a history of intractable headaches and progressive speech difficulty. Imaging demonstrated a 1.7 × 2.6 cm left frontal subcortical hemorrhagic mass with an associated developmental venous anomaly, characteristics initially suggestive of a cavernous malformation. Diffusion tractography imaging was employed to avoid interference with the intact arcuate fasciculus and corticospinal tracts to minimize neurologic deficits.
CONCLUSIONS
To our knowledge, this is the first documented case of the use of diffusion tractography in preoperative planning for cerebral amyloidoma resection. Our systematic review of 39 reported cases of amyloidomas in the literature provided insight regarding how these rare lesions have manifested and progressed and further understanding of current theories regarding their etiology and pathophysiology. Cerebral amyloidomas are rare, localized, proteinaceous aggregates with variable presentation and prognosis and no apparent relationship to systemic amyloidosis. Biopsy may be useful in determination of progression. Diffusion tractography is a valuable tool for minimizing complications associated with resection.
Topics: Amyloidosis; Brain Diseases; Female; Frontal Lobe; Humans; Middle Aged; Minimally Invasive Surgical Procedures; Neurosurgical Procedures
PubMed: 32084617
DOI: 10.1016/j.wneu.2020.02.072 -
Neurology Dec 2019To assess the association of cortical superficial siderosis (cSS) presence and extent with future bleeding risk in cerebral amyloid angiopathy (CAA). (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To assess the association of cortical superficial siderosis (cSS) presence and extent with future bleeding risk in cerebral amyloid angiopathy (CAA).
METHODS
This was a meta-analysis of clinical cohorts of symptomatic patients with CAA who had T2*-MRI at baseline and clinical follow-up for future intracerebral hemorrhage (ICH). We pooled data in a 2-stage meta-analysis using random effects models. Covariate-adjusted hazard ratios (adjHR) from multivariable Cox proportional hazard models were used.
RESULTS
We included data from 6 eligible studies (n = 1,239). cSS pooled prevalence was 34% (95% confidence interval [CI] 26%-41%; 87.94%; < 0.001): focal cSS prevalence was 14% (95% CI 12%-16%; 6.75%; = 0.37), and disseminated cSS prevalence was 20% (95% CI 13%-26%; 90.39%; < 0.001). During a mean follow-up of 3.1 years (range 1-4 years), 162/1,239 patients experienced a symptomatic ICH-pooled incidence rate 6.9% per year (95% CI 3.9%-9.8% per year; 83%; < 0.001). ICH incidence rates per year according to cSS status were 3.9% (95% CI 1.7%-6.1%; 70%; = 0.018) for patients without cSS, 11.1% (95% CI 7%-15.2%; 56.8%; = 0.074) for cSS presence, 9.1% (95% CI 5.5%-12.8%; 0%; = 0.994) for focal cSS, and 12.5% (95% CI 5.3%-19.7%; 73.2%; = 0.011) for disseminated cSS. In adjusted pooled analysis, any cSS presence was independently associated with increased future ICH risk (adjHR 2.14; 95% CI 1.19-3.85; < 0.0001). Focal cSS was linked with ICH risk (adjHR 2.11; 95% CI 1.31-2.41; = 0.002), while disseminated cSS conferred the strongest bleeding risk (adjHR 4.28; 95% CI 2.91-6.30; < 0.0001).
CONCLUSION
In patients with CAA, cSS presence and extent are the most important MRI prognostic risk factors for future ICH, likely useful in treatment planning.
CLASSIFICATION OF EVIDENCE
This study provides Class III evidence that in symptomatic CAA survivors with baseline T2*-MRI, cSS (particularly if disseminated, i.e., affecting >3 sulci) increases the risk of future ICH.
Topics: Aged; Brain Diseases; Cerebral Amyloid Angiopathy; Cerebral Cortex; Cerebral Hemorrhage; Female; Hemosiderosis; Humans; Male; Middle Aged; Prevalence; Risk Factors
PubMed: 31732564
DOI: 10.1212/WNL.0000000000008590 -
International Journal of Stroke :... Oct 2019We aimed to investigate cortical superficial siderosis as an MRI predictor of lobar intracerebral hemorrhage (ICH) recurrence risk in cerebral amyloid angiopathy (CAA),... (Meta-Analysis)
Meta-Analysis
BACKGROUND
We aimed to investigate cortical superficial siderosis as an MRI predictor of lobar intracerebral hemorrhage (ICH) recurrence risk in cerebral amyloid angiopathy (CAA), in a large prospective MRI cohort and a systematic review.
METHODS
We analyzed a single-center MRI prospective cohort of consecutive CAA-related ICH survivors. Using Kaplan-Meier and Cox regression analyses, we investigated cortical superficial siderosis and ICH risk, adjusting for known confounders. We pooled data with eligible published cohorts in a two-stage meta-analysis using random effects models. Covariate-adjusted hazard rations (adj-HR) from pre-specified multivariable Cox proportional hazard models were used.
RESULTS
The cohort included 240 CAA-ICH survivors (cortical superficial siderosis prevalence: 36%). During a median follow-up of 2.6 years (IQR: 0.9-5.1 years) recurrent ICH occurred in 58 patients (24%). In prespecified multivariable Cox regression models, cortical superficial siderosis presence and disseminated cortical superficial siderosis were independent predictors of increased symptomatic ICH risk at follow-up (HR: 2.26; 95% CI: 1.31-3.87, p = 0.003 and HR: 3.59; 95% CI: 1.96-6.57, p < 0.0001, respectively). Three cohorts including 443 CAA-ICH patients in total were eligible for meta-analysis. During a mean follow-up of 2.5 years (range: 2-3 years) 92 patients experienced recurrent ICH (pooled risk ratio: 6.9% per year, 95% CI: 4.2%-9.7% per year). In adjusted pooled analysis, any cortical superficial siderosis and disseminated cortical superficial siderosis were the only independent predictors associated with increased lobar ICH recurrence risk (adj-HR: 2.4; 95% CI: 1.5-3.7; p < 0.0001, and adj-HR: 4.4; 95% CI: 2-9.9; p < 0.0001, respectively).
CONCLUSIONS
In CAA-ICH patients, cortical superficial siderosis presence and extent are the most important MRI prognostic risk factors for lobar ICH recurrence. These results can help guide clinical decision making in patients with CAA.
Topics: Aged; Cerebral Amyloid Angiopathy; Cerebral Hemorrhage; Cohort Studies; Female; Humans; Magnetic Resonance Imaging; Male; Neuroimaging; Recurrence; Risk Factors
PubMed: 30785378
DOI: 10.1177/1747493019830065