Patients Mean Disease Duration Research Articles

Blink reflex excitability in patients with Hemifacial spasm exhibiting different abnormal discharge patterns: from early isolated discharges to later grouped bursts or tonic spasms.

We studied blink reflex (BR) and BR excitability recovery (BRER) in patients with hemifacial spasm (HFS) exhibiting different abnormal discharge patterns. We hypothesized that patients with groups of clonic or tonic burst activities appear later in the disease course and may have more excitability of the BR circuit at the brainstem compared to patients with isolated twitchings, which occur earlier. We included 124 patients with botulinum toxin-naive HFS (mean age 50.6 ± 13.3years) and 40 healthy subjects. We performed surface polymyography on facial muscles in patients and classified them according to the abnormal discharge pattern: isolated discharges, grouped bursts forming random sequences, tonic spasms, and a combination of these activities. Then, we recorded BR and BRER at 200, 600, and 1000ms interstimulus intervals. We compared disease duration, R1 and R2 latencies, R2 area-under-the-curve (AUC), and BRER% (i) between healthy subjects and patients and (ii) among groups of patients with different abnormal discharge patterns. There were isolated discharges in 28 patients, grouped bursts forming random sequences in 42, and continuous muscle activity with tonic spasms in one. The remaining patients had combinations. Mean R1 and R2 latencies were significantly longer, and mean R2 AUC was significantly higher on the symptomatic side of patients compared to healthy subjects. The mean BRER was enhanced on both sides in patients than in healthy subjects (p < 0.001). However, it was similar among patient groups with different abnormal discharge patterns (p > 0.05). The mean disease duration in patients with isolated discharges was shorter (3.3 ± 2.0years) than those with grouped bursts or tonic spasms (p = 0.002; Kruskal-Wallis test). Our study observed that excitability at the brainstem was similar in HFS patients with different abnormal discharge patterns, suggesting that the difference in discharge patterns in HFS may be due to a reason other than the difference in BR excitability.

P757 Visceral adiposity and not subcutaneous adiposity or body mass index is more strongly associated with relapse in inflammatory bowel disease

Abstract Background The role of body composition in inflammatory bowel disease (IBD) has still not been established due to heterogenous evidence. This study aimed to assess if abdominal body composition parameters were associated with outcomes in inflammatory bowel disease. Methods In this retrospective study abdominal body composition was evaluated using routinely acquired computed tomography (CT) from eligible patients with IBD between January 2014 and September 2021. Cross sectional areas of total fat (TFA), subcutaneous fat (SFA), visceral fat (VFA) and skeletal muscle (SMA) including their indices and ratios were measured at lumbar 3 (L3) vertebrae level using appropriate software. The outcome measure was relapse defined as acute flaring of symptoms and/or worsening biochemical markers, endoscopic recurrence requiring dose intensification of current therapy and surgical resection. Spearman’s correlations between body composition parameters were analysed. Binary logistic regression adjusting for demographics, smoking, disease characteristics (type, duration, location, behaviour) and medications was used to test for the relative significance of SFA, VFA, SMA and body mass index (BMI) on the outcome measures. A forward logarithmic likelihood method was used to test for it. Results Fifty-three IBD patients (58% male; 42% female) with 22 (42%) in relapse were included for abdominal CT analysis. Mean age of patients with relapse was 41.8 ± 16.2 years while those without relapse was 48.4 ± 12.5 years (p = 0.360). Mean BMI of patients with relapse was 25.6 ± 4.4 while those without relapse was 28.1 ± 7.0 (p = 0.457). Mean disease duration of patients with relapse was 15.3 ± 9.2 years while those without relapse was 21.3 ± 10.7 years (p = 0.583) [see Table 1]. Mean VFA was not significantly lower in relapse than remission (mean: 95.3 ± 99.5 cm² vs 119.7 ± 89.6 cm²; logarithmic p = 0.222), whereas mean SFA was significantly higher in relapse than remission (mean: 129.0 ± 64.3 cm² vs 225.3 ± 156.8 cm²; logarithmic p = 0.016) [see Table 2]. VFA and BMI correlated strongly with all other body composition parameters according to Spearman’s model (p &amp;lt; 0.001) [see Table 3]. After performing binary logistic regression and accounting for variables including age, gender, smoking status, type of IBD, disease characteristics (duration, location, behaviour), corticosteroid use, biologic therapy, anti-TNF level, VFA (p = 0.013) was significantly associated with relapse in comparison to SFA (p = 0.063) and BMI (p = 0.563). Conclusion Visceral adiposity and not BMI or subcutaneous adiposity is more strongly associated with relapse in IBD.

Increased Expression of Extracellular Vesicles Is Associated With the Procoagulant State in Patients With Established Rheumatoid Arthritis.

This study sought to identify different subpopulations of extracellular vesicles (EVs) in plasma from female patients with established rheumatoid arthritis (RA) in relation to the activation of coagulation and fibrin formation in these patients. Forty women were included in the study, 20 patients and 20 age-matched healthy controls. The mean disease duration in patients was 13.0 (5.0–25.0) years, with medium to high disease activity despite ongoing treatment with low-dose prednisolone and methotrexate. There were no differences between the investigated groups regarding the presence of traditional cardiovascular risk factors. The concentration of phosphatidylserine-positive (PS+) EVs; platelet (CD42a+), leucocyte (CD45+), monocyte (CD14+), and endothelial (CD144+)-derived EVs; and EVs-expressing tissue factor (CD142+), P-selectin (CD62P+), and E-selectin (CD62E+) were determined by flow cytometry analysis. Overall hemostasis potential (OHP) was assessed to follow the hemostatic disturbances, including the parameters for overall coagulation potential (OCP) and overall fibrinolytic potential (OFP). Fibrin clot turbidity was measured together with clot lysis time, and scanning electron microscopy was performed. Increased concentrations of PS+, CD42a+, CD142+, CD45+, CD14+, and CD62P+ EVs were found in plasma from patients with RA compared to healthy controls, and the concentrations of PS+, CD42a+, CD14+, and CD62P+ EVs were positively correlated with the inflammatory parameters in RA patients. Positive correlations were also found between the levels of PS+ and CD42a+ EVs and OCP as well as between the levels of PS+, CD42a+, and CD62P+EVs and OHP. The levels of PS+, CD42a+, CD14+, CD62P+, and CD62E+ EVs were negatively correlated with OFP. Elevated levels of circulating EVs of different cell origins were found in patients with established RA, in relation to the inflammatory burden and coagulation activation in the disease.

Iron-sensitive magnetic resonance imaging in Parkinson's disease: a systematic review and meta-analysis.

To evaluate the diagnostic performance of iron-sensitive sequences targeting the substantia nigra for distinguishing patients with Parkinson's disease from control participants and to identify factors causing heterogeneity. A systematic literature search in the Ovid-MEDLINE and EMBASE databases was performed for studies reporting the relevant topic before March 6, 2020. The pooled sensitivity and specificity values with their 95% confidence intervals were calculated using bivariate random-effects modeling. Subgroup and meta-regression analyses were also performed to determine factors influencing heterogeneity affecting the diagnostic performance among the clinical, MRI, and analytic characteristics. A total of 22 articles including 1126 patients with Parkinson's disease and 933 control participants were enrolled in this systematic review and meta-analysis. Of those, 12 studies used objective analyses of quantitative susceptibility measurements, and 10 visually assessed the nigrosome-1 in subjective analyses. Iron-sensitive nigral magnetic resonance imaging showed a pooled sensitivity of 92% (95% confidence interval 88-95%) and a pooled specificity of 90% (95% confidence interval 81-95%). According to subgroup and meta-regression analyses, a longer mean disease duration in patients with Parkinson's disease (≥ 5years), subjective analysis, a smaller size of pixel (< 0.6 mm2), a larger flip angle (> 15°), a smaller slice thickness (≤ 1mm), and specific targeting of the substantia nigra pars compacta improved the diagnostic performance. Iron-sensitive nigral magnetic resonance imaging had a favorable diagnostic performance in discriminating patients with Parkinson's disease from control participants. Subjective analytic methods remain superior to objective approaches. Further improvements of the spatial resolution and contrast-to-noise ratio to specifically target the nigrosome-1 with objective analytic methods will be needed.

Hearing status in patients with rheumatoid arthritis.

Background:Previous studies showed that one of the complications of rheumatoid arthritis disease was auditory disorder. The goal of the present study was to compare the auditory status in patients with rheumatoid arthritis and healthy individuals.Methods:In the present case-control study, 30 normal persons and 60 persons with rheumatoid arthritis with mean age of 46.72 and standard deviation of 6.76 of both genders were appraised using pure tone audiometry, tympanometry and speech audiometry. The mean disease duration in patients with rheumatoid arthritis was 12.51±6.09 years.Results:The frequency of hearing loss in rheumatoid arthritis group was significantly more than the control group (p=0.001). All patients had sensorineural hearing loss. Only in 5% of rheumatoid arthritis group, abnormal tympanometry (as type) was reported. Speech discrimination score analysis showed significant difference between the patients with rheumatoid arthritis and controls. In terms of hearing threshold level, the mean hearing threshold level (in 2000, 4000 and 8000 Hz frequencies) of the patients with rheumatoid arthritis was significantly higher than control group in both ears (p<0.05). A positive significant correlation was found among mean hearing threshold level in 4000 and 8000 Hz frequencies and rheumatoid arthritis duration in both ears.Conclusion:The frequency of hearing loss and the average hearing threshold in RA patients were higher than healthy individuals. The most common type hearing loss is sensorineural.

Access to End-of Life Parkinson's Disease Patients Through Patient-Centered Integrated Healthcare.

Background: Palliative care in Parkinson's Disease (PD) patients considerably differs from palliative care in oncology patients. Integrated care models are a concept to support patients and improve management of PD symptoms. However, it is not known if the access to PD patients at the end of life can be achieved through integrated care models.Aim: To analyze an integrated model of care for PD patients with the aim to identify if this integrated model of care has access to PD patients at the end of life.Material and Methods: The Cologne Parkinson's network was designed as a randomized, controlled prospective clinical trial in order to increase quality of life of PD patients. This innovative model of care integrated a neurologist in private practice, a movement disorder specialist of the University Hospital and a PD nurse. Mortality rates of PD patients during the study period of 6 months were registered and compared with mortality rates of the general population of Germany according to the Federal Statistical Office of Germany. The retrospective post-hoc analysis was conducted after completion of the initial study at the University Hospital and neurologists' practices in the greater area of Cologne, Germany. Eligible patients had a diagnosis of idiopathic PD and were aged 25–85 years.Results: Parkinson's Disease patients in this trial had an even slightly lower mortality rate as the general population (1.66 v. 2.1%). These results are contradictory and speak for a substantial proportion of late-stage disease patients, who have not been adequately included in this study or have been better treated within this trial. The mean disease duration of patients in this study was around 6 years which resembles the lower range of the mean disease duration at death of PD patients in general.Conclusions: The results of our post-hoc analysis show, that accessing PD patients in the last phase of their disease is extremely difficult and nearly fails in spite of an integrated care approach. Reasons for poor access and loss of follow-up at the end of life have to be identified and care models for PD patients until the end of life should be developed urgently.

Evaluation of the liver condition in chronic hepatitis C virus patients with and without vasculitis

Aim of the workThe association between hepatitis C virus (HCV)-related vasculitis and severe hepatic fibrosis is a controversial issue. In this study, we aimed to evaluate the liver affection in a group of patients with HCV-related vasculitis and a control group with chronic HCV infection without vasculitis. Patients and methodsTwenty-six HCV associated vasculitis patients (22 females, 4 males) with a mean age of 51.9±8.5years (range 36–72years) and a control group including 20 age- and sex matched HCV infected patients without any extra-hepatic autoimmune manifestations were recruited in this study. All patients and controls were evaluated by routine biochemical tests, conventional ultrasonography and Fibroscan. ResultsThe mean disease duration in patients with vasculitis and the control group was 7.5±7.3 and 4.1±3.6years, respectively (p=0.062). Mean aspartate aminotransferase, bilirubin and international normalized ratio (INR) values were higher in the control group (p=0.036, 0.041 and 0.017, respectively). Hepatomegaly was found in 11 (42.3%) vasculitis patients and 17 (85%) controls (p=0.006), while portal hypertension was found in 4 (15.4%) vasculitis patients and 9 (45%) controls (p=0.046). On Fibroscan, eleven vasculitis patients (42.3%) had mild to moderate liver fibrosis (F1–2), and 10 (38.5%) had severe liver fibrosis (F3–4), while only one patient (5%) of the control group had mild, and 17 (85%) had severe liver fibrosis (p=0.002). ConclusionPatients with chronic HCV infection without vasculitis have worse liver functions and more advanced liver fibrosis than those with HCV related vasculitis.

L-2-Hydroxyglutaric Aciduria: Pattern of MR Imaging Abnormalities in 56 Patients

To describe the pattern of magnetic resonance (MR) imaging abnormalities in l-2-hydroxyglutaric aciduria (L2HGA) and to evaluate the correlation between imaging abnormalities and disease duration. MR images in 56 patients (30 male, 26 female; mean age +/- standard deviation, 11.9 years +/- 8.5) with genetically confirmed L2HGA were retrospectively reviewed, with institutional review board approval and waiver of informed consent. At least one complete series of transverse T2-weighted images was available for all patients. The images were evaluated by using a previously established scoring list. The correlation between MR imaging abnormalities and disease duration was assessed (Mann-Whitney or Kruskal-Wallis test). The cerebral white matter (WM) abnormalities preferentially affected the frontal and subcortical regions. The abnormal subcortical WM often had a mildly swollen appearance (37 patients). Initially, the WM abnormalities were at least partially multifocal (32 patients). In patients with longer disease duration, the WM abnormalities became more confluent and spread centripetally, but the periventricular rim remained relatively spared (41 patients). The mean disease duration in patients with WM atrophy (14.8 years) was significantly longer (P = .001) than that in patients without atrophy (6.7 years). Bilateral involvement of the globus pallidus (55 patients), caudate nucleus (56 patients), and putamen (56 patients) was seen at all stages. The cerebellar WM was never affected. The dentate nucleus was involved bilaterally in 55 of 56 patients. L2HGA has a distinct highly characteristic pattern of MR imaging abnormalities: a combination of predominantly subcortical cerebral WM abnormalities and abnormalities of the dentate nucleus, globus pallidus, putamen, and caudate nucleus. With increasing disease duration, WM abnormalities and basal ganglia signal intensity abnormalities become more diffuse and cerebral WM atrophy ensues.

Clinical characteristics, course and prognosis of spinal multiple sclerosis

Retrospective examination. To define the clinical characteristics and response to therapy of spinal multiple sclerosis (MS). Italy. Retrospective review was performed on 563 patients with clinical definite MS. Selection criteria were two or more spinal cord lesions in the presence of normal magnetic resonance imaging of the brain. Spinal MS was diagnosed in 13 patients (2.3%) out of 563 with clinical definite MS. There were seven female and six male patients; nine had a relapsing-remitting (RR) and four, a primary progressive (PP) course. All patients were treated with immunosoppressive or immunomodulatory therapy. Mean disease duration in patients with RR-MS was 13.1+/-10.1 years with a mean age at onset of 29.5+/-14.3 years; the mean Expanded Disability Status Scale (EDSS) at the time of the study was 3.5+/-2.5 with a progression index of 0.28. Mean disease duration in patients with PP course was 7+/-6.2 years with a mean age at onset of 56.7+/-10.4 years; the mean EDSS at the time of the study was 6.2+/-2.0 with a progression index of 1.48. Patients with spinal RR-MS are characterised by an early disease onset with minimal or moderate disability progression; patients with spinal PP-MS show a late disease onset and more rapid disability progression. In our series of spinal MS patients, disability progression seems to be mainly due to the disease course and age at onset rather than to the site of lesion.