scholarly journals NEW INSIGHTS IN THE NATURAL HISTORY OF EARLY-ONSET MULTIPLE SCLEROSIS

2016 ◽  
Vol 15 (3) ◽  
pp. 127-134
Author(s):  
Romana Homorodean ◽  
◽  
Lacramioara Perju-Dumbrava ◽  
◽  

Background. The early onset forms of Multiple Sclerosis (Paediatric MS, P-MS) have a low frequency, occurring in 2% to 10% of all MS cases. Age at diagnosis is a key feature for disease progression and prognosis in MS patients. Objective. To evaluate the clinical features and course of MS in patients having the disease onset before the age of 18, according to age at diagnosis. Methods. Patients with MS onset before age of 18 identified from Basse-Normandie MS regional registry, were included in the study. Patients were divided in two groups according to the age at MS diagnosis: the first group (group A) of patients in whom MS diagnosis was set before age of 18 and the second group (group B) including patients older than 18 years at MS diagnosis. Data related to clinical features and disease course were evaluated and compared between the two groups and also with some clinical data of adult MS patients included in the same regional database. Results. A total of 41 patients with P-MS were included in the study, represented by 20 patients in group A and 21 patients in group B. Patients from group A had a more rapid progression to mild handicap (EDSS 3.0) than patients from group B (time to reach EDSS 3.0 of 6.34 vs 14.36 years; p=0.02) and they reached this level of disability at a younger age (21.17 vs 29.79 years; p=0.002). The duration of the first remission was significantly shorter in group A (1.44 vs 5.14 years; p<0.001) and the evolution to secondary-progressive (SP) form was faster when compared to group B (14.97 vs 27.57 years; p=0.01). Patients from group A had a greater risk to reach EDSS 3.0 or EDSS 6.0 as well as a greater probability to reach SP form (p<0.001) than patients from group B. Progression index (PI) of the entire paediatric group was significantly lower than mean PI in the adult group (0.29 vs 0.45; p=0.003) but not significantly different between the two groups (0.37 in group A vs 0.21 in group B). Conclusion. P-MS forms show some clinical peculiarities, especially those with the age of diagnosis less than 18 years. They seem to have a more severe clinical MS course and a faster progression of disability.

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Hyemoon Chung ◽  
Geu-Ru Hong ◽  
Jungwoo Son ◽  
In-Jeong Cho ◽  
Chi Young Shim ◽  
...  

Background: Apical hypertrophic cardiomyopathy (ApHCM) might have different morphological and clinical features according to the age of disease onset, probably result from the distinct mechanism of pathogenesis. The aim of this study was to evaluated the morphological and clinical differences according to the age of disease onset in patients with ApHCM. Methods: This was a retrospective, observational study of 56 ApHCM patients (31 males, mean 65±9 years). Among 426 patients with diagnosed ApHCM from 2000 until 2013, we selected 56 patients who met the inclusion criteria: [[Unable to Display Character: &#9332;]] patiens who diagnosed with ApHCM before 60 years old and current age >60 years old, (group A, early onset, n=16); and [[Unable to Display Character: &#9333;]] who diagnosed with ApHCM after 60 years old who had no evidence of apical hypertrophy confirmed by previous echocardiography (group B, late onset, n=40). Morphological LV feature was assessed by transthoracic echocardiography, which were divided into pure and mixed type. Furthermore, hemodynamic parameters and clinical events were also evaluated. Results: There were no differences in the presence of hypertension, LV systolic function and left atrial volume index between group A and B. However, early onset group A had more increased maximal wall thickness of LV apex (17.08±2.02 vs 15.43±1.68mm, p=0.005), and more incidence of pure type (100% (16/16) vs 56.8% (21/40), p=0.001) compare than late onset group B. Group B had older age (65±5 vs 72±4 years, p=0.001), increased LV end-diastolic dimension (51.25±2.32 vs 48.58±3.37mm, p=0.006), increased E/E’ (11.24±2.94 vs 14.60±6.37, p=0.049) with decreased E’ (5.61±1.34 vs 4.66±1.38cm/s, p=0.024) respectively.There were no significant differences in the risk of cardiovascular complication such as atrial fibrillation and stoke between group A and B. Conclusions: There was obvious different clinical characteristics according to the age of disease onset. Early onset ApHCM has typical morphological feature and relatively preserved LV diastolic function, compared with late onset ApHCM. Our result supports that late onset ApHCM was related to decreased LV diastolic function and increased LV wall thickness, which reflects the potential myocardial remodeling with aging.


Open Medicine ◽  
2016 ◽  
Vol 11 (1) ◽  
pp. 509-517 ◽  
Author(s):  
Silvia Marola ◽  
Alessia Ferrarese ◽  
Enrico Gibin ◽  
Marco Capobianco ◽  
Antonio Bertolotto ◽  
...  

AbstractConstipation, obstructed defecation, and fecal incontinence are frequent complaints in multiple sclerosis. The literature on the pathophysiological mechanisms underlying these disorders is scant. Using anorectal manometry, we compared the anorectal function in patients with and without multiple sclerosis.136 patients referred from our Center for Multiple Sclerosis to the Coloproctology Outpatient Clinic, between January 2005 and December 2011, were enrolled. The patients were divided into four groups: multiple sclerosis patients with constipation (group A); multiple sclerosis patients with fecal incontinence (group B); non-multiple sclerosis patients with constipation (group C); non-multiple sclerosis patients with fecal incontinence (group D). Anorectal manometry was performed to measure: resting anal pressure; maximum squeeze pressure; rectoanal inhibitory reflex; filling pressure and urge pressure. The difference between resting anal pressure before and after maximum squeeze maneuvers was defined as the change in resting anal pressure calculated for each patient.ResultsGroup A patients were noted to have greater sphincter hypotonia at rest and during contraction compared with those in group C (p=0.02); the rectal sensitivity threshold was lower in group B than in group D patients (p=0.02). No voluntary postcontraction sphincter relaxation was observed in either group A or group B patients (p=0.891 and p=0.939, respectively).ConclusionsThe decrease in the difference in resting anal pressure before and after maximum squeeze maneuvers suggests post-contraction sphincter spasticity, indicating impaired pelvic floor coordination in multiple sclerosis patients. A knowledge of manometric alterations in such patients may be clinically relevant in the selection of patients for appropriate treatments and for planning targeted rehabilitation therapy.


2017 ◽  
Vol 19 (1) ◽  
pp. 11-14 ◽  
Author(s):  
Gary Birnbaum

Background: Current disease-modifying therapies (DMTs) are of benefit only in people with relapsing forms of multiple sclerosis (RMS). Thus, safely stopping DMTs in people with secondary progressive MS may be possible. Methods: Two groups of patients with MS were studied. Group A consisted of 77 patients with secondary progressive MS and no evidence of acute central nervous system inflammation for 2 to 20 years. These patients were advised to stop DMTs. Group B consisted of 17 individuals with RMS who stopped DMTs on their own. Both groups were evaluated at treatment cessation and for a minimum of 1 year thereafter. Multiple variables were assessed to determine those that predicted recurrent acute disease. Results: Nine patients in group A (11.7%) and ten patients in group B (58.8%) had recurrent acute disease, almost always within 1 to 2 years of stopping treatment. The only variable of significance in group A distinguishing stable and relapsing patients was age (P = .0003), with relapsing patients being younger. Group B patients were younger and had significantly lower Expanded Disability Status Scale scores than group A, with no significant differences in age between relapsed and stable patients. Conclusions: The DMTs can be stopped safely in older patients with MS (≥7 decades) with no evidence of acute disease for 2 years or longer, with an almost 90% probability of remaining free of acute recurrence. The high proportion of untreated patients with RMS experiencing recurrent acute disease is consistent with published data.


2006 ◽  
Vol 134 (9-10) ◽  
pp. 414-419
Author(s):  
Aleksandar Djurovic ◽  
Mirjana Zivotic-Vanovic ◽  
Dejan Popovic

Introduction: Early rehabilitation of patients with cementless total hip arthroplasty (cTHA) includes different physical modalities and pulsed low frequency magnetic field (PLFMF), which effects have not been explored yet. Objective: To investigate the effects of PLFMF which was applied in different doses in early rehabilitation of patients with cTHA. Method: Prospective, controlled, clinical study included 90 patients, divided in three groups with 30 patients each. First two groups were treated with high (group A) or low (group B) doses of PLFMF, in addition to kinesitherapy. Control group C was treated only with kinesitherapy. Study was completed in three weeks. Results: Subjects of group A had significantly lower pain than group B (p<0.01) and group C (p<0.001) subjects in the first postoperative week. Pain in group B subjects was significantly lower than in group C in all three postoperative weeks (p<0.01). In relation to other two groups, subjects of group A had higher hip Harris score values at the end of the third postoperative week (p<0.05), and they were faster on 10-meter distance at the end of the first postoperative week (p<0.01). Conclusion: PLFMF used in low and high doses for patients with cTHA had significant effects on pain abatement, especially at higher doses. Improvement of function was earlier and more manifested in the group treated with high doses of PLFMF.


2008 ◽  
Vol 115 (7) ◽  
pp. 219-224 ◽  
Author(s):  
Makrina D. Savvidou ◽  
Alexandros Sotiriadis ◽  
Christine Kaihura ◽  
Kypros H. Nicolaides ◽  
Naveed Sattar

Adiponectin and leptin, two adipose-tissue-derived proteins, have been reported to be elevated in women with established PE (pre-eclampsia). The aim of the present study was to investigate whether alterations in adiponectin and leptin levels predate the development of PE and FGR (fetal growth restriction) in women at increased risk of these complications, as assessed by Doppler examination of the uterine arteries during the second trimester of pregnancy. We also sought to investigate the circulating levels of adiponectin and leptin in women with established severe early-onset FGR. The study included three groups of pregnant women at 23–25 weeks: Group A (n=44) with normal uterine artery Doppler waveforms, Group B (n=49) with abnormal Doppler waveforms and normal fetal growth at the time of the examination, and Group C (n=15) with established severe FGR and abnormal Doppler waveforms. All women had plasma adiponectin and leptin measured by sensitive immunoassays. In Group B, 19 women had a normal outcome, 17 delivered infants with FGR and 13 developed PE. The women who developed PE delivered smaller babies earlier than women with a normal outcome (P<0.001). There were no significant differences in adiponectin levels between any of the groups (overall P=0.3). Leptin concentrations, expressed as MoM (multiples of the median) of Group A, were higher in women in Group C, i.e. established severe FGR at 2.5 (1.2–2.7) MoMs (overall P<0.001), compared with all of the other groups and subgroups. In conclusion, we found that, in pregnancies complicated by severe early-onset FGR, the maternal plasma concentration of leptin is twice as high as in normal pregnancies. However, the second trimester levels of maternal plasma adiponectin and leptin in pregnancies that subsequently develop PE and/or FGR are not significantly different from normal and, consequently, it is unlikely that these markers will be useful as predictors of these pregnancy complications.


2020 ◽  
Vol 2020 ◽  
pp. 1-10
Author(s):  
Jie Liu ◽  
Hulin Chen ◽  
Dewei Wu ◽  
Ruiling Wei ◽  
Chaolan Lv ◽  
...  

Background. Stimulant laxatives are still considered the most common treatment for functional outlet obstruction constipation (FOOC). However, the effectiveness of laxatives is unsatisfactory, and the long-term use of laxatives may cause certain adverse events. With this in mind, it is, however, paramount that novel complementary treatment(s) and/or other forms of alternative medicine are adequately investigated. Aims. The study aims to explore the effects and potential mechanism(s) of transcutaneous electrical acustimulation (TEA) combined with adaptive biofeedback training (ABT) on FOOC. Methods. A total of forty-five patients with FOOC were recruited and were randomly assigned to receive either Macrogol 4000 Powder (MAC, 10 g bid) (group A, n = 15) only, ABT + MAC + Sham-TEA (group B, n = 15), or TEA + ABT + MAC (group C, n = 15) in a six-week study. Individual patients' constipation-symptoms (PAC-SYM) and constipation-quality of life (PAC-QOL) were both assessed and scored. Serum acetylcholine (Ach) and nitric oxide (NO) were measured from drawn blood samples while individual patients' heart rate variability (HRV) was calculated at baseline and after each corresponding therapy. Anorectal manometry and balloon expulsion test were both performed before and after treatment. Results. Firstly, participants in group C had significantly lower scores of PAC-SYM, PAC-QOL, and a decreased anal defecating pressure (ADP) as compared to participants in group B (all p<0.050). These results, however, suggest the TEAs effect. Secondly, the low-frequency band (LF)/(LF + HF) ratio in groups B and C were decreased as compared to group A (p=0.037, p=0.010, respectively) regarding HRV. On the other hand, the high-frequency band (HF)/(LF + HF) ratio in groups B and C showed an opposite outcome. Finally, the serum Ach in groups B and C was significantly higher as compared to group A (p=0.023, p=0.012, respectively). Of significant importance, the serum NO in groups B and C were notably low as compared to group A (p=0.001, p<0.001, respectively). Conclusions. TEA, combined with ABT, effectively improves constipation symptoms as well as QOL in FOOC patients. It is, however, achieved by decreasing ADP, which mechanisms are mediated via the autonomic and enteric mechanisms.


2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Antonio Carotenuto ◽  
◽  
Marcello Moccia ◽  
Teresa Costabile ◽  
Elisabetta Signoriello ◽  
...  

AbstractDifferently from the adult multiple sclerosis (MS) population, the predictive value of cognitive impairment in early-onset MS is still unknown. We aim to evaluate whether cognitive performances at disease onset predict disease progression in young people with MS. This is a retrospective study on early onset (<25 years) MS patients, who had a baseline cognitive evaluation at disease onset. Demographic and longitudinal clinical data were collected up to 7 years follow up. Cognitive abilities were assessed at baseline through the Brief Repeatable Battery. Associations between cognitive abilities and clinical outcomes (occurrence of a relapse, and 1-point EDSS progression) were evaluated with stepwise logistic and Cox regression models. We included 51 patients (26 females), with a mean age at MS onset of 17.2 ± 3.9 years, and an EDSS of 2.5 (1.0–6.0). Over the follow-up, twenty-five patients had at least one relapse, and 7 patients had 1-point EDSS progression. Relapse occurrence was associated with lower 10/36 SPART scores (HR = 0.92; p = 0.002) and higher WLG scores (HR = 1.05; p = 0.01). EDSS progression was associated with lower SDMT score (OR: 0.70; p = 0.04). Worse visual memory and attention/information processing were associated with relapses and with increased motor disability after up to 7-years follow-up. Therefor, specific cognitive subdomains might better predict clinical outcomes than the overall cognitive impairment in early-onset MS.


1993 ◽  
Vol 11 (12) ◽  
pp. 2330-2341 ◽  
Author(s):  
R Ladenstein ◽  
C Lasset ◽  
O Hartmann ◽  
D Frappaz ◽  
A Garaventa ◽  
...  

PURPOSE Relapse from stage 4 neuroblastoma usually carries a poor prognosis. A retrospective study using the European Bone Marrow Transplant (EBMT) Solid Tumor Registry was undertaken to define the role of megatherapy (MGT) in relapsed patients. PATIENTS AND METHODS After relapse, 33 boys and 15 girls with previous stage 4 neuroblastoma received intensification by MGT followed by either autologous (n = 42) or allogeneic (n = 6) bone marrow rescue in 11 European institutions. The median age at diagnosis was 47 months (range, 14 to 134) and the median interval from diagnosis to relapse was 16 months (range, 4 to 94). Thirty patients had received only conventional-dose primary treatments (group A), whereas 18 patients had previously received intensification with MGT (group B). The median follow-up time of the total group is 95 months (range, 25 to 185). RESULTS The actuarial overall survival rate at 2 years after MGT for relapse is 27% for group A and 0% for group B (P = .02). Three adverse, independent prognostic factors were confirmed by multivariate analysis using the Cox proportional hazards regression model: an interval of less than 12 months between diagnosis and relapse (P < .0001), nonresponding or untreated relapse (P = .0002), and previous MGT during primary treatments (P = .055). None of the other variables analyzed, such as sex, age, bone or bone marrow involvement at diagnosis or at relapse, and type of MGT at relapse, influenced outcome in this patient cohort. CONCLUSION Responding patients who relapse more than 12 months from diagnosis who had not received previous MGT appear to benefit from consolidation MGT. Relapse patients who do not fulfill these criteria gain no advantage from this cost-intensive procedure and should be treated differently.


2007 ◽  
Vol 13 (9) ◽  
pp. 1138-1145 ◽  
Author(s):  
T. Kümpfel ◽  
M. Schwan ◽  
Th. Pollmächer ◽  
A. Yassouridis ◽  
M. Uhr ◽  
...  

During initiation of interferon-beta (IFN-β) therapy, many multiple sclerosis (MS) patients experience systemic side effects which may depend on the time point of IFN-β injection. We investigated the time course of plasma hormone-, cytokine- and cytokine-receptor concentrations after the first injection of IFN-β either at 8.00 a.m. (group A) or at 6.00 p.m. (group B) and quantified clinical side effects within the first 9 h in 16 medication free patients with relapsing-remitting MS. This investigation was repeated after 6-month IFN-β therapy. Plasma ACTH and cortisol concentrations followed their physiological rhythms, with lower levels in the evening compared to the morning, but raised earlier and stronger in group B after IFN-β administration. IFN-β injection in the evening led to a prompter increase of plasma IL-6 concentrations and temperature during the first hours and correlated to more intense clinical side effects compared to group A. Plasma IL-10 concentrations increased more in group A compared to group B, but sTNF-RI and sTNF-RII concentrations raised 7 h after IFN-β injection only in group B. Acute effects on plasma hormone and cytokine concentrations adapted after 6-month IFN-β treatment, while diurnal variations were still present. Baseline sTNF-RII concentrations were elevated after 6-month IFN-β therapy only in group A. Our results show that time point of IFN-β injection has differential effects on acute changes of plasma hormone and cytokine concentrations and is related to systemic side effects. This may have implications on the tolerability and effectiveness of IFN-β therapy. Multiple Sclerosis 2007; 13: 1138—1145. http://msj.sagepub.com


2013 ◽  
Vol 38 (2) ◽  
pp. 125-133 ◽  
Author(s):  
J Martin ◽  
E Fernandez ◽  
J Estay ◽  
VV Gordan ◽  
IA Mjor ◽  
...  

SUMMARY Replacement of dental restorations has been the traditional treatment for restorations that are defective. In this five-year randomized clinical trial, restorations with localized marginal defects were treated with sealants. Thirty-two patients (mean age, 26.8 years) with 126 Class I and Class II restorations with defective margins (amalgam n=69 and resin-based composite n=57) were recruited. Treatment was seal with pit and fissure sealant on localized marginal defects (group A: n=43) and was compared with total restoration replacement (group B: n=40) and untreated restorations (group C: n=43) as negative and positive controls. Restorations were assessed by two examiners using the modified US Public Health Service criteria, observing five clinical parameters: marginal adaptation, roughness, marginal stain, teeth sensitivity, and secondary caries at baseline and at five years after treatment. At the five-year recall examination, 23 patients with 90 restorations (71.4% recall rate) were examined. A significant improvement was observed in the marginal adaptation of the restorations in group A compared with group B. None of the treated group showed trends to downgrade in any parameter. Tooth sensitivity and secondary caries showed a low frequency in all groups. No significant difference in marginal adaptation of the restorations was found between amalgam and resin-based composite restorations (p=0.191). This study demonstrated that marginal sealing of restorations is a minimally invasive treatment that may be used instead of the replacement of restorations with localized marginal defects.


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