scholarly journals Midbrain/pons area ratio and clinical features predict the prognosis of Progressive Supranuclear Palsy

2020 ◽  
Author(s):  
shishuang cui ◽  
huawei ling ◽  
juanjuan du ◽  
yiqi lin ◽  
jing pan ◽  
...  

Abstract Backgrounds: Progressive Supranuclear Palsy (PSP) is a rare movement disorder with poor prognosis. This retrospective study aims to characterize the natural history of PSP and to find predictors of shorter survival and faster decline of activity of daily living. Method: All patients recruited fulfilled the movement disorder society (MDS) clinical diagnostic criteria for PSP (MDS-PSP criteria) for probable and possible PSP with median 12 years. Data were obtained including age, sex, date of onset, age at onset (AAO), symptoms reported at first visit and follow-up, date of death and date of institutionalization. Magnetic resonance imaging was collected at the first visit. Endpoints were death and institutionalization. Kaplan-Meier method and Cox proportional hazard model were used to explore factors associated with early death and institutionalization. Results: 59 patients fulfilling MDS-PSP criteria were enrolled in our study. 19 patients (32.2%) had died and 31 patients (52.5%) were institutionalizedl by the end of the follow-up. Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. Predictors associated with earlier institutionalization were older AAO and decreased M/P area ratio. Conclusion: older and decreased M/P area ratio were predictors for earlier dearth and institutionalization in PSP. The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP.

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Shi-Shuang Cui ◽  
Hua-Wei Ling ◽  
Juan-Juan Du ◽  
Yi-Qi Lin ◽  
Jing Pan ◽  
...  

Abstract Background Progressive supranuclear palsy (PSP) is a rare movement disorder with poor prognosis. This retrospective study aimed to characterize the natural history of PSP and to find predictors of shorter survival and faster decline of activity of daily living. Method All patients recruited fulfilled the movement disorder society (MDS) clinical diagnostic criteria for PSP (MDS-PSP criteria) for probable and possible PSP with median 12 years. Data were obtained including age, sex, date of onset, age at onset (AAO), symptoms reported at first visit and follow-up, date of death and date of institutionalization. Magnetic resonance imaging was collected at the first visit. Endpoints were death and institutionalization. Kaplan-Meier method and Cox proportional hazard model were used to explore factors associated with early death and institutionalization. Results Fifty-nine patients fulfilling MDS-PSP criteria were enrolled in our study. Nineteen patients (32.2%) had died and 31 patients (52.5%) were institutionalized by the end of the follow-up. Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. Predictors associated with earlier institutionalization were older AAO and decreased M/P area ratio. Conclusion Older AAO and decreased M/P area ratio were predictors for earlier dearth and institutionalization in PSP. The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP.


2020 ◽  
Author(s):  
Shishuang Cui ◽  
Huawei Ling ◽  
Juanjuan Du ◽  
Yiqi Lin ◽  
Jing Pan ◽  
...  

Abstract Backgrounds: Progressive Supranuclear Palsy (PSP) is a rare movement disorder with poor prognosis. This retrospective study aims to characterize the natural history of PSP and to find predictors of shorter survival and faster decline of activity of daily living. Method: All patients recruited fulfilled the movement disorder society (MDS) clinical diagnostic criteria for PSP (MDS-PSP criteria) for probable and possible PSP with median 12 years. Data were obtained including age, sex, date of onset, age at onset (AAO), symptoms reported at first visit and follow-up, date of death and date of institutionalization. Magnetic resonance imaging was collected at the first visit. Endpoints were death and institutionalization. Kaplan-Meier method and Cox proportional hazard model were used to explore factors associated with early death and institutionalization. Results: 59 patients fulfilling MDS-PSP criteria were enrolled in our study. 19 patients (32.2%) had died and 31 patients (52.5%) were institutionalizedl by the end of the follow-up. Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. Predictors associated with earlier institutionalization were older AAO and decreased M/P area ratio. Conclusion: older and decreased M/P area ratio were predictors for earlier dearth and institutionalization in PSP. The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP.


2020 ◽  
Author(s):  
shishuang cui ◽  
huawei ling ◽  
juanjuan du ◽  
yiqi lin ◽  
jing pan ◽  
...  

Abstract Backgrounds: Progressive Supranuclear Palsy (PSP) is a rare movement disorder with poor prognosis. This retrospective study aims to characterize the natural history of PSP and to find predictors of shorter survival and faster decline of activity of daily living. Method: All patients recruited fulfilled the movement disorder society (MDS) clinical diagnostic criteria for PSP (MDS-PSP criteria) for probable and possible PSP with median 12 years. Data were obtained including age, sex, date of onset, age at onset (AAO), symptoms reported at first visit and follow-up, date of death and date of institutionalization. Magnetic resonance imaging was collected at the first visit. Endpoints were death and institutionalization. Kaplan-Meier method and Cox proportional hazard model were used to explore factors associated with early death and institutionalization. Results: 59 patients fulfilling MDS-PSP criteria were enrolled in our study. 19 patients (32.2%) had died and 31 patients (52.5%) were institutionalizedl by the end of the follow-up. Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. Predictors associated with earlier institutionalization were older AAO and decreased M/P area ratio. Conclusion: older and decreased M/P area ratio were predictors for earlier dearth and institutionalization in PSP. The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP.


2019 ◽  
Author(s):  
shishuang cui ◽  
huawei ling ◽  
juanjuan du ◽  
yiqi lin ◽  
jing pan ◽  
...  

Abstract Introduction: Progressive Supranuclear Palsy (PSP) is a rare movement disorder with poor prognosis. The natural history of PSP has been described in a few studies. This retrospective study aims to characterize the natural history of PSP and to find predictors of shorter survival and faster decline of activity of daily living.Method: All patients recruited fulfilled the movement disorder society (MDS) clinical diagnostic criteria for PSP (MDS-PSP criteria) for probable and possible PSP with median 12 years. Data were obtained including age, gender, date of onset, age at onset (AAO), symptoms reported at first visit and follow-up, date of death and date of institutionalization. Magnetic resonance imaging was collected at the first visit. Endpoints were death and institutionalization. K-M method and Cox proportional hazard model were used to explore factors associated with early death and institutionalization.Results: 59 patients fulfilled MDS-PSP criteria were enrolled in our study. 19 patients (32.2%) had died and 31 patients (52.5%) had institutionalized by the end of the follow-up. Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. Predictors associated with earlier institutionalization were older AAO and decreased M/P area ratio.Conclusion: Older AAO and decreased M/P area ratio were predictors for earlier dearth and institutionalization in PSP. The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP.


2020 ◽  
Author(s):  
Yiqing Xia ◽  
R. Zoë Greenwald ◽  
M. Rachael Milwid ◽  
Claire Trottier ◽  
Michel Boissonnault ◽  
...  

AbstractBackgroundReducing HIV transmission using pre-exposure prophylaxis (PrEP) requires targeting individuals at high acquisition risk, such as men who have sex with men (MSM) with a history of non-occupational post-exposure prophylaxis (nPEP). This study aims to characterize longitudinal trends in PrEP uptake and its determinants among nPEP users in Montréal.MethodsEligible attendees at Clinique médicale l’Actuel were recruited prospectively starting in October 2000 (nPEP) and January 2013 (PrEP). Linking these cohorts, we characterized the PEP-to-PrEP cascade, examined the determinants of PrEP uptake after nPEP consultation using a Cox proportional-hazard model, and assessed whether PrEP persistence differed by nPEP history using Kaplan-Meier curves.ResultsAs of August 2019, 31% of 2,682 MSM nPEP cohort participants had two or more nPEP consultations. Subsequent PrEP consultations occurred among 36% of nPEP users, of which 17% sought nPEP again afterwards. Among 2,718 PrEP cohort participants, 46% reported previous nPEP use. Among nPEP users, those aged 25-49 years (Hazard Ratio (HR)=1.3, 95% confidence interval (CI): 1.1-1.7), with more nPEP episodes (HR=1.4, 95%CI: 1.3-1.5), reported chemsex (HR=1.3, 95%CI: 1.1-1.7), with a STI history (HR=1.5; 95%CI: 1.3-1.7), and who returned for their first nPEP follow-up visit (HR=3.4, 95%CI: 2.7-4.2) had higher rates of PrEP linkage. There was no difference in PrEP persistence between PEP-to-PrEP and PrEP only participants.ConclusionOver one-third of nPEP users were subsequently prescribed PrEP. However, the large proportion of men who repeatedly use nPEP calls for more efficient PrEP-linkage services and, among those that use PrEP, improved persistence should be encouraged.


Rheumatology ◽  
2018 ◽  
Vol 58 (4) ◽  
pp. 650-655 ◽  
Author(s):  
Alexander Oldroyd ◽  
Jamie C Sergeant ◽  
Paul New ◽  
Neil J McHugh ◽  
Zoe Betteridge ◽  
...  

Abstract Objectives To characterize the 10 year relationship between anti-transcriptional intermediary factor 1 antibody (anti-TIF1-Ab) positivity and cancer onset in a large UK-based adult DM cohort. Methods Data from anti-TIF1-Ab-positive/-negative adults with verified diagnoses of DM from the UK Myositis Network register were analysed. Each patient was followed up until they developed cancer. Kaplan–Meier methods and Cox proportional hazard modelling were employed to estimate the cumulative cancer incidence. Results Data from 263 DM cases were analysed, with a total of 3252 person-years and a median 11 years of follow-up; 55 (21%) DM cases were anti-TIF1-Ab positive. After 10 years of follow-up, a higher proportion of anti-TIF1-Ab-positive cases developed cancer compared with anti-TIF1-Ab-negative cases: 38% vs 15% [hazard ratio 3.4 (95% CI 2.2, 5.4)]. All the detected malignancy cases in the anti-TIF1-Ab-positive cohort occurred between 3 years prior to and 2.5 years after DM onset. No cancer cases were detected within the following 7.5 years in this group, whereas cancers were detected during this period in the anti-TIF1-Ab-negative cases. Ovarian cancer was more common in the anti-TIF1-Ab-positive vs -negative cohort: 19% vs 2%, respectively (P < 0.05). No anti-TIF1-Ab-positive case <39 years of age developed cancer, compared with 21 (53%) of those ≥39 years of age. Conclusion Anti-TIF1-Ab-positive-associated malignancy occurs exclusively within the 3 year period on either side of DM onset, the risk being highest in those ≥39 years of age. Cancer types differ according to anti-TIF1-Ab status, and this may warrant specific cancer screening approaches.


2013 ◽  
Vol 65 (2) ◽  
pp. 447-453
Author(s):  
N. Lukic ◽  
A. Stankovic ◽  
E. Dincic ◽  
M. Bundalo ◽  
Z. Krsmanovic ◽  
...  

The function of peroxisome proliferator-activated receptor ? (PPAR?) in immune regulation, as well as in antiinflammatory and anti-proliferative actions towards T lymphocytes, has been reported. A potential role of PPARs in multiple sclerosis (MS) was suggested. The aim of this study was to investigate if there is an association of PPAR?-2 Pro12Ala polymorphism with MS in 361 patients from Serbia. The genotype and allele frequencies of Pro12Ala polymorphism were not significantly different between controls and patients, or between females and males. In contrast to controls, we detected a rare Ala/Ala genotype in patients with MS. We found that there is a significant association of Ala/Ala genotype with older age at onset (ANOVA, p=0.07; LSD post-hoc, Ala/Ala vs. Pro/Ala, p=0.03, Ala/Ala vs. Pro/Pro p=0.02). It would be useful to validate our results in other populations, as well as to perform follow-up of the disease progression in regard to PPAR? genotypes.


2021 ◽  
Vol 8 ◽  
Author(s):  
Dana Bielopolski ◽  
Ruth Rahamimov ◽  
Boris Zingerman ◽  
Avry Chagnac ◽  
Limor Azulay-Gitter ◽  
...  

Background: Microalbuminuria is a well-characterized marker of kidney malfunction, both in diabetic and non-diabetic populations, and is used as a prognostic marker for cardiovascular morbidity and mortality. A few studies implied that it has the same value in kidney transplanted patients, but the information relies on spot or dipstick urine protein evaluations, rather than the gold standard of timed urine collection.Methods: We revisited a cohort of 286 kidney transplanted patients, several years after completing a meticulously timed urine collection and assessed the prevalence of major cardiovascular adverse events (MACE) in relation to albuminuria.Results: During a median follow up of 8.3 years (IQR 6.4–9.1) 144 outcome events occurred in 101 patients. By Kaplan-Meier analysis microalbuminuria was associated with increased rate of CV outcome or death (p = 0.03), and this was still significant after stratification according to propensity score quartiles (p = 0.048). Time dependent Cox proportional hazard analysis showed independent association between microalbuminuria and CV outcomes 2 years following microalbuminuria detection (HR 1.83, 95% CI 1.07–2.96).Conclusions: Two years after documenting microalbuminuria in kidney transplanted patients, their CVD risk was increased. There is need for primary prevention strategies in this population and future studies should address the topic.


Neurology ◽  
2020 ◽  
Vol 94 (11) ◽  
pp. e1171-e1180 ◽  
Author(s):  
Elena Cortés-Vicente ◽  
Rodrigo Álvarez-Velasco ◽  
Sonia Segovia ◽  
Carmen Paradas ◽  
Carlos Casasnovas ◽  
...  

ObjectiveTo describe the characteristics of patients with very-late-onset myasthenia gravis (MG).MethodsThis observational cross-sectional multicenter study was based on information in the neurologist-driven Spanish Registry of Neuromuscular Diseases (NMD-ES). All patients were >18 years of age at onset of MG and onset occurred between 2000 and 2016 in all cases. Patients were classified into 3 age subgroups: early-onset MG (age at onset <50 years), late-onset MG (onset ≥50 and <65 years), and very-late-onset MG (onset ≥65 years). Demographic, immunologic, clinical, and therapeutic data were reviewed.ResultsA total of 939 patients from 15 hospitals were included: 288 (30.7%) had early-onset MG, 227 (24.2%) late-onset MG, and 424 (45.2%) very-late-onset MG. The mean follow-up was 9.1 years (SD 4.3). Patients with late onset and very late onset were more frequently men (p < 0.0001). Compared to the early-onset and late-onset groups, in the very-late-onset group, the presence of anti–acetylcholine receptor (anti-AChR) antibodies (p < 0.0001) was higher and fewer patients had thymoma (p < 0.0001). Late-onset MG and very-late-onset MG groups more frequently had ocular MG, both at onset (<0.0001) and at maximal worsening (p = 0.001). Although the very-late-onset group presented more life-threatening events (Myasthenia Gravis Foundation of America IVB and V) at onset (p = 0.002), they required fewer drugs (p < 0.0001) and were less frequently drug-refractory (p < 0.0001).ConclusionsPatients with MG are primarily ≥65 years of age with anti-AChR antibodies and no thymoma. Although patients with very-late-onset MG may present life-threatening events at onset, they achieve a good outcome with fewer immunosuppressants when diagnosed and treated properly.


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