scholarly journals Pediatric autoimmune encephalitis

2020 ◽  
Vol 7 (3) ◽  
pp. e682 ◽  
Author(s):  
Marienke A.A.M. de Bruijn ◽  
Arlette L. Bruijstens ◽  
Anna E.M. Bastiaansen ◽  
Agnes van Sonderen ◽  
Marco W.J. Schreurs ◽  
...  
Keyword(s):  
Autoimmune Encephalitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Incidence Rates ◽  
Study Cohort ◽  
Current Guideline ◽  
Clinical Criteria ◽  
Aspartate Receptor ◽  
Hashimoto Encephalopathy ◽  
Neurologic Disorders

ObjectiveThe aims of this study were (1) to describe the incidence of autoimmune encephalitis (AIE) and acute disseminated encephalomyelitis (ADEM) in children, (2) to validate the currently used clinical criteria to diagnose AIE, and (3) to describe pitfalls in the diagnosis of pediatric autoimmune (AI) and inflammatory neurologic disorders.MethodsThis study cohort consists of 3 patient categories: (1) children with antibody-mediated AIE (n = 21), (2) children with ADEM (n = 32), and (3) children with suspicion of an AI etiology of their neurologic symptoms (n = 60). Baseline and follow-up clinical data were used to validate the current guideline to diagnose AIE. In addition, patient files and final diagnoses were reviewed.ResultsOne-hundred three of the 113 included patients fulfilled the criteria of possible AIE. Twenty-one children had antibody-mediated AIE, of whom 19 had anti-N-methyl-D-aspartate receptor (NMDAR), 1 had anti–α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, and 1 had anti–leucine-rich glioma-inactivated protein 1 encephalitis. Finally, 34 children had ADEM, and 2 children had Hashimoto encephalopathy. Mean incidence rates were 1.54 children/million (95% CI 0.95–2.35) for antibody-mediated AIE and 2.49 children/million (95% CI 1.73–3.48) for ADEM. Of the other 48 children, treating physicians' diagnoses were reviewed. In 22% (n = 6) of children initially diagnosed as having an AI/inflammatory etiology (n = 27), no support for AI/inflammation was found.ConclusionBesides anti-NMDAR encephalitis and ADEM, other AIEs are rare in children. The current guideline to diagnose AIE is also useful in children. However, in children with nonspecific symptoms, it is important to review data critically, to perform complete workup, and to consult specialized neuroinflammatory centers.

SKIN MELANOMA INCIDENCE IN WORKERS OCUPATIONALLY EXPOSED TO IONIZING RADIATION OVER A PROLONGED PERIOD

2019 ◽  
Vol 65 (3) ◽  
pp. 441-446
Author(s):  
Valentina Rybkina ◽  
Tamara Azizova ◽  
Yevgeniya Grigoreva
Keyword(s):  
Ionizing Radiation ◽  
Gamma Rays ◽  
Disease Risk ◽  
Incidence Rates ◽  
Study Cohort ◽  
Calendar Period ◽  
Skin Melanoma ◽  
Melanoma Incidence ◽  
Occupationally Exposed

Purpose of the study. The study is aimed to investigate skin melanoma incidence in workers occupationally exposed to radiation over a prolonged period. Materials and methods. Skin melanoma incidence was studied in a cohort of workers first employed at nuclear facility Mayak Production Association (PA) between 1948 and 1982 who had been followed up till 31.12.2013 (22,377 individuals). Mean cumulative doses from external gamma-rays over the whole follow-up period were 0.54±0.001 Sv in males and 0.44±0.002 Sv in females. Incident rates for skin melanoma were analyzed by sex, attained age, calendar period of diagnostics and radiation dose using worldwide standard and the direct standardization technique. Results. 60 skin melanoma cases (37 in males and 23 in females) were registered in the study cohort over the whole follow-up period. The standardized skin melanoma incident rate was 8.51±1.46 in males and 8.78±2.27 in females per 100000 workers revealing statistically higher rates compared to corresponding rates for general populations of the Russian Federation, Urals Federal District and Chelyabinsk region. Skin melanoma incidence was significantly increased in the period of 1994 - 2013 as compared to the period of 1974 - 1993. Skin melanoma incidence excess in females was greater than that for males. Skin melanoma incidence increment in females was mostly driven by modifications of disease occurrence risk while in males it was driven by a combined effect of age pattern modifications in the study cohort and increase of disease risk. Conclusions. Skin melanoma incidence rates in the cohort of workers occupationally exposed to ionizing radiation over a prolonged period were associated with sex and attained age workers and the calendar period of diagnostics. No significant association of skin melanoma incidence with dose from external gamma-rays was observed. A significantly increasing trend was observed for skin melanoma incidence by the end of the follow-up in both males and females.

scholarly journals Effects of colchicine on risk of cardiovascular events and mortality among patients with gout: a cohort study using electronic medical records linked with Medicare claims

2015 ◽  
Vol 75 (9) ◽  
pp. 1674-1679 ◽  
Author(s):  
Daniel H Solomon ◽  
Chih-Chin Liu ◽  
I-Hsin Kuo ◽  
Agnes Zak ◽  
Seoyoung C Kim
Keyword(s):  
Cox Regression ◽  
Incidence Rates ◽  
Study Cohort ◽  
Beneficial Effects ◽  
Medicare Claims ◽  
Potential Association ◽  
All Cause Mortality ◽  
And Gender ◽  
Cv Disease

BackgroundColchicine may have beneficial effects on cardiovascular (CV) disease, but there are sparse data on its CV effect among patients with gout. We examined the potential association between colchicine and CV risk and all-cause mortality in gout.MethodsThe analyses used data from an electronic medical record (EMR) database linked with Medicare claims (2006–2011). To be eligible for the study cohort, subjects must have had a diagnosis of gout in the EMR and Medicare claims. New users of colchicine were identified and followed up from the first colchicine dispensing date. Non-users had no evidence of colchicine prescriptions during the study period and were matched to users on the start of follow-up, age and gender. Both groups were followed for the primary outcome, a composite of myocardial infarction, stroke or transient ischaemic attack. We calculated HRs in Cox regression, adjusting for potential confounders.ResultsWe matched 501 users with an equal number of non-users with a median follow-up of 16.5 months. During follow-up, 28 primary CV events were observed among users and 82 among non-users. Incidence rates per 1000 person-years were 35.6 for users and 81.8 for non-users. After full adjustment, colchicine use was associated with a 49% lower risk (HR 0.51, 95% CI 0.30 to 0.88) in the primary CV outcome as well as a 73% reduction in all-cause mortality (HR 0.55, 95% CI 0.35 to 0.85, p=0.007).ConclusionsColchicine use was associated with a reduced risk of a CV event among patients with gout.

scholarly journals Cerebrospinal Fluid IL-17A Could Predict Acute Disease Severity in Non-NMDA-Receptor Autoimmune Encephalitis

2021 ◽  
Vol 12 ◽  
Author(s):  
Michael Levraut ◽  
Véronique Bourg ◽  
Nicolas Capet ◽  
Adrien Delourme ◽  
Jérôme Honnorat ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Disease Severity ◽  
Disease Onset ◽  
Autoimmune Encephalitis ◽  
Demyelinating Diseases ◽  
Acute Disease ◽  
Aspartate Receptor ◽  
Initial Severity ◽  
Csf Concentration

IntroductionMost of our knowledge into autoimmune encephalitis (AE) comes from N-Methyl-D-Aspartate Receptor (NMDAR) encephalitis. The concentrations of cytokines in cerebrospinal fluid (CSF) including IL-17A have been found to be increased and associated with poor outcome. However, data on the cytokine concentration in CSF and its correlation with outcome is lacking for other types of AE.ObjectiveTo report the concentrations of CSF sIL-2R, IL-6, IL-8, IL-10 and IL-17A and to correlate it with acute disease severity and the 1-year outcome in non-NMDAR AE.MethodsWe measured the CSF concentration of each cytokine in 20 AE patients, and compared IL-6 and IL-17A concentrations with 13 patients with CNS demyelinating diseases and 20 non-inflammatory controls. Patients were > 18yr and had at least 1-year clinical follow-up. Intracellular and NMDAR antibody (Ab) -mediated encephalitis were excluded. A mRS ≤ 2 was retained as a 1-year good outcome.ResultsThe IL-17A concentration in CSF was higher in AE patients than in both control groups (p<0.01). No difference was observed in CSF concentration of IL-6 between groups. At disease onset, a high CSF IL-17A concentration correlated with a high modified Rankin Scale (p<0.05), a high Clinical Assessment Scale for Autoimmune Encephalitis score (p<0.001) and ICU admission (p<0.01). There was no correlation between the concentration of all CSF cytokines and the 1-year clinical outcome.ConclusionOur results show that CSF IL-17A could be interesting to assess initial severity in non-NMDAR AE. Thus, CSF IL-17A could be an interesting therapeutic target and be useful to assess early selective immunosuppressive therapy.

scholarly journals Breast cancer incidence rates in women exposed to occupational ionizing radiation

2021 ◽  
Vol 65 (3) ◽  
pp. 245-250
Author(s):  
Anna V. Rumyantseva ◽  
Tamara V. Azizova ◽  
Mariya V. Bannikova ◽  
Ol’ga V. Zubkova
Keyword(s):  
Breast Cancer ◽  
Ionizing Radiation ◽  
Incidence Rate ◽  
Incidence Rates ◽  
Study Cohort ◽  
Female Population ◽  
Study Objective ◽  
Incidence And Mortality ◽  
Increasing Trend

Introduction. Breast cancer (BC) is one of the most common malignancies within female incidence and mortality patterns in the RF. Study objective. To analyze BC incidence in females exposed to occupational ionizing radiation over prolonged periods. Materials and methods. The study cohort included 5689 females who had been occupationally exposed to ionizing radiation throughout 1948-1982 and followed up until 31.12.2018. Results. BC was on the top of the list among females of the study cohort (11.9%). BC prevalence rate in the female cohort understudy was 28.3% as of 31.12.2018. The standardized BrCa incidence rate was 0.88 ± 0.07 per 1000 person-years of the follow-up. The absolute BrCa case increase in the females was 1.29, with the rise of 232.48% by the end of the follow-up due to the rise in age categories of females and the elevating risk of the disease occurrence. Discussion. The study cohort of females demonstrated standardized BrCa incidence rates consistent with the corresponding estimates for the general female population of the RF. Meanwhile, the observed rates were lower than those estimated for the female population of the Chelyabinsk region. The increasing trend of the standardized BC incidence rate by the end of the follow-up period (31.12.2018) was consistent with WHO statistics. The extended follow-up for an updated assessment of ionizing radiation effect on BC incidence is required to develop new cancer prevention strategies. Conclusion. The study demonstrated that BC incidence rates in the study cohort varied by age and calendar period of the follow-up. The increasing trend of the standardized rates of BC incidence was observed by the end of the follow-up.

Leptospira Triggered Anti-N-Methyl-d-Aspartate Receptor Encephalitis

2020 ◽  
Author(s):  
Prateek Kumar Panda ◽  
Indar Kumar Sharawat ◽  
Rishi Bolia
Keyword(s):  
Sleep Disturbances ◽  
Early Recognition ◽  
Case Reports ◽  
Case Series ◽  
Autoimmune Encephalitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Neurological Symptoms ◽  
Aspartate Receptor ◽  
Systemic Symptoms ◽  
Extrapyramidal Features

Abstract Primary neuroleptospirosis although rare but has been reported in the literature in the form of case reports and case series. However, there are no reports of autoimmune encephalitis triggered by leptospirosis in the literature, although four cases of acute disseminated encephalomyelitis, which is also considered to have autoimmune etiology have been reported. We are reporting an adolescent girl, who developed anti-N-methyl-d-aspartate receptor encephalitis after the resolution of systemic symptoms of leptospirosis. Her symptoms including neuropsychiatric and extrapyramidal features and sleep disturbances resolved completely after immunotherapy. As recently autoimmune encephalitis triggered by various infections are getting reported more frequently around the world, the clinicians need to consider this clinical possibility, even in patients with leptospirosis, who develop neurological symptoms while systemic clinical features are subsiding. Early recognition and timely administration of immunotherapy have the potential to completely reverse the neurological symptoms.

scholarly journals Bidirectional association between migraine and fibromyalgia: retrospective cohort analyses of two populations

BMJ Open ◽  
2019 ◽  
Vol 9 (4) ◽  
pp. e026581 ◽  
Author(s):  
I-Wen Penn ◽  
Eric Chuang ◽  
Tien-Yow Chuang ◽  
Cheng-Li Lin ◽  
Chia-Hung Kao
Keyword(s):  
Cohort Study ◽  
Incidence Rates ◽  
Study Cohort ◽  
Control Cohort ◽  
Healthcare Database ◽  
Bidirectional Association ◽  
Using Data ◽  
Two Populations ◽  
Cohort Analyses

ObjectiveFibromyalgia (FM) and migraine are common pain disorders that tend to coexist. This study determined whether these two conditions exhibited any mutual influences.SettingCohort study.ParticipantsA retrospective, longitudinal cohort study was conducted using data obtained from a nationwide healthcare database. This study had two arms. Arm 1 comprised 33 216 patients with FM and arm 2 consisted of 7420 patients with migraine; all of these patients were diagnosed between 2000 and 2010. Using the aforementioned database, control subjects who had neither FM nor migraine and were matched with the FM and migraine patients by sex, age and index date of diagnosis were recruited. Each control cohort was four times the size of the corresponding study cohort. Follow-up for the control and study cohorts was conducted until the end of 2011.ResultsThe incidence rates of FM and migraine were calculated in arms 1 and 2, respectively. The overall incidence of migraine was greater in the FM cohort than in the corresponding control cohort (4.39 vs 2.07 per 1000 person-years (PY)); crude HR=2.12, 95% CI=1.96 to 2.30; adjusted HR (aHR)=1.89, 95% CI=1.75 to 2.05). After adjustment for sex, age and comorbidities, the overall incidence of FM in the migraine cohort was 1.57 times greater than that in the corresponding control cohort (7.01 vs 4.49 per 1000 PY; aHR=1.52, 95% CI=1.39 to 1.65).ConclusionsThe present study revealed a bidirectional link between FM and migraine.

Seizure Incidence Rates in Children and Adults With Familial Cerebral Cavernous Malformations

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012569
Author(s):  
Christine K. Fox ◽  
Jeffrey Nelson ◽  
Charles E. McCulloch ◽  
Shantel Weinsheimer ◽  
Ludmila Pawlikowska ◽  
...  
Keyword(s):  
Incidence Rate ◽  
Cavernous Malformation ◽  
Incidence Rates ◽  
Cerebral Cavernous Malformation ◽  
Care Utilization ◽  
Study Cohort ◽  
Cavernous Malformations ◽  
Increased Risk ◽  
Unit Increase

Background and Objectives:Seizure incidence rates related to Familial Cerebral Cavernous Malformation (FCCM) are not well described, especially for children. To measure the seizure incidence rate, examine seizure predictors and characterize epilepsy severity, we studied a cohort of children and adults with FCCM enrolled in the Brain Vascular Malformation Consortium (BVMC).Methods:Seizure data were collected from participants with FCCM in the BVMC at enrollment and during follow-up. We estimated seizure probability by age, and tested whether cerebral cavernous malformation (CCM) counts or genotype were associated with earlier seizure onset.Results:The study cohort included 479 FCCM cases. Median age at enrollment was 42.5 years (Interquartile Range [IQR] 22.5-55.0) and 19% were children (<18 years old). Median large CCM count was 3 (IQR: 1-5). Among 393 with genotyping, mutations were: CCM1-Common Hispanic Mutations (88%), another CCM1 mutation (5%), CCM2 mutations (5%), and CCM3 mutations (2%). Prior to or during the study, 202 (42%) had a seizure. The cumulative incidence of a childhood seizure was 20.3% (95% CI 17.0 – 23.4) and by age 80 years was 60.4% (95% CI 54.2-65.7). More total CCMs (Hazard Ratio [HR] 1.24 per SD unit increase, 95% CI 1.1 – 1.4) or more large CCMs (HR=1.5 per SD unit increase, 95% CI 1.2-1.9) than expected for age and sex increased seizure risk. A CCM3 mutation also increased risk compared to other mutations (HR 3.11, 95% CI 1.15-8.45). Individuals with a seizure prior to enrollment had increased hospitalization rates during follow-up (Incidence Rate Ratio 10.9, 95% CI 2.41 – 49.32) compared to patients without a seizure history.Discussion:Individuals with FCCM have a high seizure incidence, and those with more CCMs or CCM3 genotype are at greater risk. Seizures increase health care utilization in FCCM.

scholarly journals DOP44 Cancer risk in a high-incidence inflammatory bowel disease population: a Faroese IBD cohort study

2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S081-S082
Author(s):  
J M M Midjord ◽  
A G Vang ◽  
T Hammer ◽  
J Burisch ◽  
K R Nielsen
Keyword(s):  
Inflammatory Bowel Disease ◽  
Cohort Study ◽  
Cancer Risk ◽  
Bowel Disease ◽  
Incidence Rates ◽  
Study Cohort ◽  
Background Population ◽  
Inflammatory Bowel ◽  
Risk Of Cancer

Abstract Background The association between inflammatory bowel disease and malignancy is still controversial despite many observational studies. The Faroese population exhibits the highest occurrence of inflammatory bowel disease (IBD) in the world. The aim of this study was to investigate the risk of cancer in Faroese IBD patients within the nationwide Faroese IBD cohort. Methods This was a nationwide cohort study of all IBD patients diagnosed in the Faroe Islands from 1960 to 2014. Clinical demographics and cancer diagnosis were retrieved from patient files and from the Faroese cancer registry. Patients were followed until the event of cancer, death or emigration. Patients diagnosed with cancer prior to the IBD diagnosis were excluded. Observed numbers of cancer were compared with expected numbers based on ASR(N) (Nordic age- and sex-specific incidence rates) from Nordcan, by multiplying ASR(N) with person-years and follow-up in the study cohort and presented as standardised incidence ratios (SIRs) with 95 % intervals (CIs). Results The cohort consisted of 664 incidence IBD patients. After excluding 12 patients with cancer prior to IBD diagnosis, 652 patients with a total follow-up length of 11 476 person-years were included (414 UC with 7.494 patient-years, 128 IBDU with 2.038 patient-years and 110 CD with 1.944 patient-years). A total of 56 patients developed cancer during the follow-up period compared with 39.2 expected cases in the background population. We observed no gender difference. In UC, 33 observed compared with 25.7 expected patients developed cancer (SIR 1.28; 95% CI, 0.88–1.80). In IBDU, 13 observed compared with 6.9 expected patients developed cancer (SIR 1.88; 95% CI 1.00–0.22). In CD, 10 cases were observed compared with 6.6 expected (SIR 1.51; 95% CI 0.73–2.79). The most common types of cancers observed are shown in Table 1. Conclusion In this nationwide cohort study, we found no overall risk of cancer in IBD patients in the geographic isolated Faroe Island. However, skin cancer occurred more than expected in IBDU patients. This finding needs to be investigated further including the influence of treatment on cancer risk.

Clinical long term follow-up of children presenting with a severe acute disseminated encephalomyelitis (ADEM)

2008 ◽  
Vol 39 (05) ◽  
Author(s):  
A Nagl ◽  
S Lütjen ◽  
S Zotter ◽  
A Blaschek ◽  
C Korenke ◽  
...  
Keyword(s):  
Acute Disseminated Encephalomyelitis ◽  
Long Term Follow Up

Management and outcomes of isolated interhemispheric subdural hematomas associated with falx syndrome

2019 ◽  
Vol 131 (6) ◽  
pp. 1920-1925
Author(s):  
Daniel A. Tonetti ◽  
William J. Ares ◽  
David O. Okonkwo ◽  
Paul A. Gardner
Keyword(s):  
Neurological Deterioration ◽  
Neurological Deficits ◽  
Motor Deficits ◽  
Study Cohort ◽  
Venous Infarction ◽  
Nonsurgical Management ◽  
Subdural Hematomas ◽  
Poor Outcomes ◽  
Contemporary Management

OBJECTIVELarge interhemispheric subdural hematomas (iSDHs) causing falx syndrome are rare; therefore, a paucity of data exists regarding the outcomes of contemporary management of iSDH. There is a general consensus among neurosurgeons that large iSDHs with neurological deficits represent a particular treatment challenge with generally poor outcomes. Thus, radiological and clinical outcomes of surgical and nonsurgical management for iSDH bear further study, which is the aim of this report.METHODSA prospectively collected, single-institution trauma database was searched for patients with isolated traumatic iSDH causing falx syndrome in the period from January 2008 to January 2018. Information on demographic and radiological characteristics, serial neurological examinations, clinical and radiological outcomes, and posttreatment complications was collected and tallied. The authors subsequently dichotomized patients by management strategy to evaluate clinical outcome and 30-day survival.RESULTSTwenty-five patients (0.4% of those with intracranial injuries, 0.05% of those with trauma) with iSDH and falx syndrome represented the study cohort. The average age was 73.4 years, and most patients (23 [92%] of 25) were taking anticoagulants or antiplatelet medications. Six patients were managed nonoperatively, and 19 patients underwent craniotomy for iSDH evacuation; of the latter patients, 17 (89.5%) had improvement in or resolution of motor deficits postoperatively. There were no instances of venous infarction, reaccumulation, or infection after evacuation. In total, 9 (36%) of the 25 patients died within 30 days, including 6 (32%) of the 19 who had undergone craniotomy and 3 (50%) of the 6 who had been managed nonoperatively. Patients who died within 30 days were significantly more likely to experience in-hospital neurological deterioration prior to surgery (83% vs 15%, p = 0.0095) and to be comatose prior to surgery (100% vs 23%, p = 0.0031). The median modified Rankin Scale score of surgical patients who survived hospitalization (13 patients) was 1 at a mean follow-up of 22.1 months.CONCLUSIONSiSDHs associated with falx syndrome can be evacuated safely and effectively, and prompt surgical evacuation prior to neurological deterioration can improve outcomes. In this study, craniotomy for iSDH evacuation proved to be a low-risk strategy that was associated with generally good outcomes, though appropriately selected patients may fare well without evacuation.

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