Abstract WMP102: Prevalence and Natural History of Superficial Siderosis: A Population-based Study

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Michael Pichler ◽  
Kelly Flemming ◽  
Alejandro Rabinstein ◽  
Robert Brown ◽  
Kejal Kantarci ◽  
...  

Introduction: Cortical superficial siderosis (cSS) refers to deposition of blood breakdown products along the cerebral cortex, causing characteristic staining patterns seen with iron-sensitive MRI techniques. Cortical superficial siderosis is a relatively rare disorder, but has been linked to cerebral amyloid angiopathy and Alzheimer’s disease. The objective of this study was to determine the frequency and natural history of cSS in the general elderly population. Methods: MRI scans from the Mayo Clinic Study of Aging (MCSA), an ongoing population-based study of elderly residents in Olmsted County, Minnesota, were reviewed by neuroradiologists. Participants with cSS were identified based on linear pattern of hypointensity on gradient recalled echo imaging consistent with cSS. Exclusion criteria were: 1) MRI findings not consistent with cSS or 2) alternative explanation for MRI findings (such as aneurysmal subarachnoid hemorrhage, intracranial surgery, or trauma). Additional data abstracted included extent of cSS, presence of cerebral microbleeds, and clinical outcome. Results: Eleven out of 1,441 participants had MRI scans showing cSS (0.8%). When stratified by age, the frequency was 0.4% in those 50 to 70 years old and 1.1% in those over 70 years old. Six participants had only focal involvement of cSS (restricted to three or fewer sulci) and five had disseminated involvement (affecting more than three sulci). Microbleeds were seen in four of five (80%) participants with disseminated cSS, but none with focal cSS. Five participants (2 focal, 3 disseminated cSS) had follow up MRI scans, with an average follow up of 25 months. There was no further hemorrhage in those with focal cSS. However, all three participants with disseminated cSS experienced additional hemorrhage: one with new microbleeds, one with new microbleeds and lobar hemorrhage, and one with sulcal subarachnoid hemorrhage and lobar hemorrhage. Conclusion: Although rare, cSS may be encountered in the general elderly population. Extent of involvement of cSS and concomitant microbleeds may be important risk factors for progression of disease and intracerebral hemorrhage. The clinical significance of focal cSS occurring in the absence of microbleeds requires further investigation.

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Christina Bergqvist ◽  
François Hemery ◽  
Arnaud Jannic ◽  
Salah Ferkal ◽  
Pierre Wolkenstein

AbstractNeurofibromatosis 1 (NF1) is an inherited, autosomal-dominant, tumor predisposition syndrome with a birth incidence as high as 1:2000. A patient with NF1 is four to five times more likely to develop a malignancy as compared to the general population. The number of epidemiologic studies on lymphoproliferative malignancies in patients with NF1 is limited. The aim of this study was to determine the incidence rate of lymphoproliferative malignancies (lymphoma and leukemia) in NF1 patients followed in our referral center for neurofibromatoses. We used the Informatics for Integrated Biology and the Bedside (i2b2) platform to extract information from the hospital’s electronic health records. We performed a keyword search on clinical notes generated between Jan/01/2014 and May/11/2020 for patients aged 18 years or older. A total of 1507 patients with confirmed NF1 patients aged 18 years and above were identified (mean age 39.2 years; 57% women). The total number of person-years in follow-up was 57,736 (men, 24,327 years; women, 33,409 years). Mean length of follow-up was 38.3 years (median, 36 years). A total of 13 patients had a medical history of either lymphoma or leukemia, yielding an overall incidence rate of 22.5 per 100,000 (0.000225, 95% confidence interval (CI) 0.000223–0.000227). This incidence is similar to that of the general population in France (standardized incidence ratio 1.07, 95% CI 0.60–1.79). Four patients had a medical history leukemia and 9 patients had a medical history of lymphoma of which 7 had non-Hodgkin lymphoma, and 2 had Hodgkin lymphoma. Our results show that adults with NF1 do not have an increased tendency to develop lymphoproliferative malignancies, in contrast to the general increased risk of malignancy. While our results are consistent with the recent population-based study in Finland, they are in contrast with the larger population-based study in England whereby NF1 individuals were found to be 3 times more likely to develop both non-Hodgkin lymphoma and lymphocytic leukemia. Large-scale epidemiological studies based on nationwide data sets are thus needed to confirm our findings.


Neurology ◽  
2019 ◽  
Vol 93 (4) ◽  
pp. e358-e371 ◽  
Author(s):  
Andreas Charidimou ◽  
Hazel I. Zonneveld ◽  
Sara Shams ◽  
Kejal Kantarci ◽  
Ashkan Shoamanesh ◽  
...  

ObjectiveTo assess potential mechanisms of cortical superficial siderosis (cSS), a central MRI biomarker in cerebral amyloid angiopathy (CAA), we performed a collaborative meta-analysis of APOE associations with cSS presence and severity.MethodsWe pooled data from published studies reporting APOE genotype and MRI assessment of cSS in 3 distinct settings: (1) stroke clinic patients with symptomatic CAA (i.e., lobar intracerebral hemorrhage, transient focal neurologic episodes) according to the Boston criteria; (2) memory clinic patients; and (3) population-based studies. We compared cSS presence and severity (focal or disseminated vs no cSS) in participants with ε2+ or ε4+ genotype vs the ε3/ε3 genotype, by calculating study-specific and random effects pooled, unadjusted odds ratios (ORs).ResultsThirteen studies fulfilled inclusion criteria: 7 memory clinic cohorts (n = 2,587), 5 symptomatic CAA cohorts (n = 402), and 1 population-based study (n = 1,379). There was no significant overall association between APOE ε4+ and cSS presence or severity. When stratified by clinical setting, APOE ε4+ was associated with cSS in memory clinic (OR 2.10; 95% confidence interval [CI] 1.11–3.99) but not symptomatic CAA patients. The pooled OR showed significantly increased odds of having cSS for APOE ε2+ genotypes (OR 2.42, 95% CI 1.48–3.95) in both patient populations. This association was stronger for disseminated cSS in symptomatic CAA cohorts. In detailed subgroup analyses, APOE ε2/ε2 and APOE ε2/ε4 genotypes were most consistently and strongly associated with cSS presence and severity.ConclusionCAA-related vasculopathic changes and fragility associated with APOE ε2+ allele might have a biologically meaningful role in the pathophysiology and severity of cSS.


Neurology ◽  
2018 ◽  
Vol 91 (2) ◽  
pp. e132-e138 ◽  
Author(s):  
Solène Moulin ◽  
Barbara Casolla ◽  
Grégory Kuchcinski ◽  
Gregoire Boulouis ◽  
Costanza Rossi ◽  
...  

ObjectiveTo determine the prevalence of cortical superficial siderosis (cSS), its clinical and neuroimaging associated markers, and its influence on the risk of recurrent intracerebral hemorrhage (ICH) in a prospective observational ICH cohort.MethodsWe investigated clinical and radiologic markers associated with cSS using multivariable analysis. In survival analyses, we used Cox models to identify predictors of recurrent ICH after adjusting for potential confounders.ResultsOf the 258 patients included in the study, 49 (19%; 95% confidence interval [CI] 14%–24%) had cSS at baseline. Clinical factors independently associated with the presence of cSS were increasing age (odds ratio [OR] 1.03 per 1-year increase, 95% CI 1.001–1.06, p = 0.044), preexisting dementia (OR 2.62, 95% CI 1.05–6.51, p = 0.039), and history of ICH (OR 4.02, 95% CI 1.24–12.95, p = 0.02). Among radiologic biomarkers, factors independently associated with the presence of cSS were ICH lobar location (OR 24.841, 95% CI 3.2–14.47, p < 0.001), severe white matter hyperintensities score (OR 5.51, 95% CI 1.17–5.78, p = 0.019), and absence of lacune (OR 4.46, 95% CI 1.06–5.22, p = 0.035). During a median follow-up of 6.4 (interquartile range 2.9–8.4) years, recurrent ICH occurred in 19 patients. Only disseminated cSS (hazard ratio 4.69, 95% CI 1.49–14.71, p = 0.008), not the presence or absence of cSS or focal cSS on baseline MRI, was associated with recurrent symptomatic ICH.ConclusionIn a prospective observational cohort of spontaneous ICH, clinical and radiologic markers associated with cSS suggest the implication of underlying cerebral amyloid angiopathy. Disseminated cSS may become a key prognostic neuroimaging marker of recurrent ICH that could be monitored in future clinical trials dedicated to patients with ICH.


Neurology ◽  
2020 ◽  
pp. 10.1212/WNL.0000000000011052
Author(s):  
Qi Li ◽  
Maria Clara Zanon Zotin ◽  
Andrew D. Warren ◽  
Yuan Ma ◽  
Edip Gurol ◽  
...  

Objective:To investigate whether acute convexity subarachnoid hemorrhage (cSAH) detected on CT in lobar intracerebral hemorrhage (ICH) related to cerebral amyloid angiopathy (CAA) is associated with recurrent ICH.Methods:We analyzed data from a prospective cohort of consecutive acute lobar ICH survivors fulfilling the Boston criteria for possible or probable CAA who had both brain CT and MRI at index ICH. Presence of cSAH was assessed on CT blinded to MRI data. Cortical superficial siderosis (cSS), cerebral microbleeds and white matter hyperintensities were evaluated on MRI. Cox proportional hazard models were used to assess the association between cSAH and the risk of recurrent symptomatic ICH during follow-up.Results:A total of 244 ICH survivors (76.4 ± 8.7 years; 54.5% female) were included. cSAH was observed on baseline CT in 99 patients (40.5%). Presence of cSAH was independently associated with cSS, hematoma volume and pre-existing dementia. During a median follow-up of 2.66 years, 49 patients (20.0%) had a recurrent symptomatic ICH. Presence of cSAH was associated with recurrent ICH (hazard ratio [HR] 2.64; 95% CI 1.46-4.79; p=0.001), after adjusting for age, antiplatelet use, warfarin use, history of previous ICH.Conclusion:cSAH was detected on CT in 40.5% of patients with acute lobar ICH related to CAA and heralds an increased risk of recurrent ICH. This CT marker may be widely used to stratify the ICH risk in patients with CAA.Classification of evidence:This study provides Class II evidence that cSAH accurately predicts recurrent stroke in patients with CAA.


Author(s):  
Chieh-Sen Chuang ◽  
Kai-Wei Yang ◽  
Chia-Ming Yen ◽  
Cheng-Li Lin ◽  
Chia-Hung Kao

Objective: Previous research has demonstrated that patients with a history of organophosphate poisoning tend to have a higher risk of neurological disorder. However, research on the rate of seizure development in patients after organophosphate poisoning is lacking. This study examined whether individuals with organophosphate poisoning have an increased risk of seizures through several years of follow-up. Patients and Methods: We conducted a retrospective study on a cohort of 45,060 individuals (9012 patients with a history of organophosphate poisoning and 36,048 controls) selected from the Taiwan National Health Insurance Research Database. The individuals were observed for a maximum of 12 years to determine the rate of new-onset seizure disorder. We selected a comparison cohort from the general population that was randomly frequency-matched by age, sex, and index year and further analyzed the risk of seizures using a Cox regression model adjusted for sex, age, and comorbidities. Results: During the study period, the risk of seizure development was 3.57 times greater in patients with organophosphate poisoning compared with individuals without, after adjustments for age, sex, and comorbidities. The absolute incidence of seizures was highest in individuals aged 20 to 34 years in both cohorts (adjusted hazard ratio = 13.0, 95% confidence interval = 5.40−31.4). A significantly higher seizure risk was also observed in patients with organophosphate poisoning and comorbidities other than cirrhosis. Conclusions: This nationwide retrospective cohort study demonstrates that seizure risk is significantly increased in patients with organophosphate poisoning compared with the general population.


2020 ◽  
pp. 107780122090832
Author(s):  
Ingrid J. Rowlands ◽  
Carl Holder ◽  
Peta M. Forder ◽  
Kelsey Hegarty ◽  
Annette J. Dobson ◽  
...  

We examined reporting of lifetime intimate partner violence (IPV) among 7,917 young women who completed two surveys, 12 months apart. At the first survey, 32% reported a history of IPV with a current or former partner. Of these, one third of women did not report IPV 12 months later (inconsistently reported IPV). Compared with women who consistently reported a history of IPV, women who inconsistently reported a history of IPV were less likely to report suicidal ideation, self-harm, illicit drug use, and smoking at the 12-month follow-up. A deeper understanding of what influences young women’s reporting of IPV is needed.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5318-5318
Author(s):  
Ronald S. Go ◽  
Jacob D Gundrum ◽  
Joan M Neuner

Abstract Background We performed a population-based study to determine the natural history of monoclonal gammopathy-associated malignancies (MGAM) including multiple myeloma, lymphoplasmacytic lymphoma, and Waldenstrom's macroglobulinemia that were considered smoldering at the time of cancer diagnosis. We compared the clinical outcomes among those with and without a prior monoclonal gammopathy of undetermined significance (MGUS) follow-up. Methods Data were obtained from the Surveillance Epidemiology and End Results (SEER) database linked to Medicare claims. We considered patients age ≥ 67 years with MGAM diagnosed from 1994-2005. We excluded those who were diagnosed by autopsy or death certificate only, had invasive cancers within 5 years prior to MGAM diagnosis, prior diagnosis of MGAM, lacked date of either diagnosis or death, lacked complete Medicare parts A/B coverage 15 months prior to or 3 months after MGAM diagnosis (or to date of death, if death was within 3 months), and receiving dialysis more than 3 months before MGAM diagnosis. Major complications including acute kidney injury, cord compression, dialysis-requirement, fracture, and hypercalcemia at time of disease progression were obtained from diagnosis and procedure claims. MGUS follow-up was defined as having a diagnosis claim 4-15 months prior to smoldering MGAM diagnosis. Smoldering MGAM was defined as absence of all of the following criteria: any major complication claims, any treatment claims, and death because of MGAM within 12 months of MGAM diagnosis, while progression to active MGAM was defined by the presence of any of these criteria. Results At the time of MGAM diagnosis 2,189 had smoldering disease. Of these, 1,753 (80.1%) progressed to active MGAM with a median time-to-progression of 17 months. Progression to active MGAM were identified by claims reflecting the development of major cancer complications (34.9%), administration of cancer therapy (59.5%), both (0.7%), or report of death from MGAM without major complication or treatment claims (4.9%). The cumulative probability of progression by Kaplan-Meier analysis was 40.2%, 72.9%, 85.7%, and 96.1%, at years 1, 3, 5, and 10, respectively. Among those who progressed, 35.6% developed one or more major complications including acute kidney injury (13.0%), cord compression (1.8%), dialysis-requirement (2.0%), fracture (15.6%), and hypercalcemia (5.6%). A prior MGUS follow-up occurred in 199 (9.1%) smoldering MGAM patients. The time to progression for smoldering MGAM patients with a prior MGUS follow-up was similar to those without MGUS follow-up (P = 0.52). Multivariable analysis factoring in year of smoldering MGAM diagnosis, type of cancer, age, sex, race, place of residence, SEER site, median annual household income, Charlson co-morbidity index, and prior MGUS follow-up showed that older age, higher Charlson co-morbidity scores, and diagnosis of multiple myeloma as independent predictors of progression (Table). Conclusions Our population-based study shows that most patients with smoldering MGAM diagnosed at age ≥ 67 years progress to active disease within the first three years. Those with a prior history of MGUS follow-up progress at a similar rate compared to those without such a history. The determinants of progression at diagnosis are age, number of co-morbidities, and type of MGAM. Disclosures: No relevant conflicts of interest to declare.


2016 ◽  
Vol 10 (7) ◽  
pp. 805-811 ◽  
Author(s):  
Fernando Fernández-Bañares ◽  
Yamile Zabana ◽  
Montserrat Aceituno ◽  
Laura Ruiz ◽  
Antonio Salas ◽  
...  

Cancers ◽  
2020 ◽  
Vol 12 (11) ◽  
pp. 3100
Author(s):  
Zahra Pasdar ◽  
David T. Gamble ◽  
Phyo K. Myint ◽  
Robert N. Luben ◽  
Nicholas J. Wareham ◽  
...  

Purpose: The purpose was to determine the association between HDP and cancer in a UK cohort. Methods: Between 1993 and 1997, participants from the EPIC-Norfolk cohort attended baseline health-checks and completed questionnaires, where a history of HDP was collected. Incident cancer cases were identified through NHS record linkage until March 2016. Univariable and multivariable logistic regression analyses were employed to determine the association between HDP and odds of cancer, with adjustment for potential confounders including co-morbidities, sociodemographic, lifestyle and reproductive factors. Results: 13,562 women were included after excluding prevalent cancer cases and women with no pregnancies. 2919 (21.5%) reported HDP and 2615 incident cancers occurred during mean follow up of 19 years. Median age (IQR) at baseline for incident cancer was 60.8 (±14.8) years. Among incident cancer cases, 578 (22.1%) had HDP. In multivariable analyses, HDP had odds ratio (OR) 1.06; 95% CI 0.95–1.18 for incident cancer. The ORs (95% CIs) for common site-specific cancers including breast, colorectal, lung, ovarian and endometrial cancers were 1.06 (0.88–1.28), 1.15 (0.92–1.45), 0.96 (0.68–1.35), 1.30 (0.93–1.83) and 1.16 (0.80–1.67). Conclusion: We found no association between HDP and cancer risk. Further studies are required to confirm and account for any underlying genetic factors involved in pregnancy-related exposures and cancer risk.


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