ABSTRACT
In recent decades, the possibility that use of mobile communicating devices, particularly wireless (mobile and cordless) phones, may increase brain tumour risk, has been a concern, particularly given the considerable increase in their use by young people. MOBI-Kids, a 14-country (Australia, Austria, Canada, France, Germany, Greece, India, Israel, Italy, Japan, Korea, the Netherlands, New Zealand, Spain) case-control study, was conducted to evaluate whether wireless phone use (and particularly resulting exposure to radiofrequency (RF) and extremely low frequency (ELF) electromagnetic fields (EMF)) increases risk of brain tumours in young people. Between 2010 and 2015, the study recruited 899 people with brain tumours aged 10 to 24 years old and 1,910 controls (operated for appendicitis) matched to the cases on date of diagnosis, study region and age. Participation rates were 72% for cases and 54% for controls. The mean ages of cases and controls were 16.5 and 16.6 years, respectively; 57% were males. The vast majority of study participants were wireless phones users, even in the youngest age group, and the study included substantial numbers of long-term (over 10 years) users: 22% overall, 51% in the 20-24-year-olds. Most tumours were of the neuroepithelial type (NBT; n = 671), mainly glioma. The odds ratios (OR) of NBT appeared to decrease with increasing time since start of use of wireless phones, cumulative number of calls and cumulative call time, particularly in the 15-19 years old age group. A decreasing trend in ORs was also observed with increasing estimated cumulative RF specific energy and ELF induced current density at the location of the tumour. Further analyses suggest that the large number of ORs below 1 in this study is unlikely to represent an unknown causal preventive effect of mobile phone exposure: they can be at least partially explained by differential recall by proxies and prodromal symptoms affecting phone use before diagnosis of the cases. We cannot rule out, however, residual confounding from sources we did not measure. Overall, our study provides no evidence of a causal association between wireless phone use and brain tumours in young people. However, the sources of bias summarised above prevent us from ruling out a small increased risk.
Subject(s)
Brain Neoplasms , Cell Phone , Glioma , Adolescent , Adult , Brain Neoplasms/epidemiology , Brain Neoplasms/etiology , Case-Control Studies , Child , Electromagnetic Fields/adverse effects , Glioma/etiology , Humans , Male , Radio Waves/adverse effects , Young AdultABSTRACT
ABSTRACTThe aim of the present study is to investigate the impact of benzodiazepine use on cognitive performance in primary care patients with first cognitive complaints. The association between the exposition to benzodiazepines (short and long half-life) and cognitive performance, evaluated through the Mini Mental State Examination (MMSE), was tested through analysis of the covariance and logistic regression models. Within the 4,249 participants (mean age 77.0 ± 8.2, 66.4% women), 732 (17%) were on benzodiazepines. When compared with non-users, short- and long-acting benzodiazepine users presented overlapping adjusted MMSE mean scores (respectively, mean MMSE score: 25.3, 95%CI 25.2-25.5; 25.4, 95%CI 25.1-25.7, and 25.9, 95%CI 25.3-26.4; p = 0.156). When tested according to the logistical regression model, after adjusting for potential confounders, no association was found between short and long acting benzodiazepine use and a MMSE < 24 (respectively, OR 0.9, 95%CI 0.7-1.2; OR 0.8, 95%CI 0.7-1.3) as compared with non-users. In conclusion, according to the results of our study, benzodiazepine use seems not to impact on cognitive performance- as assessed with the MMSE- of primary care patients referring to GPs for first cognitive complaints.
Subject(s)
Aged, 80 and over/psychology , Benzodiazepines/therapeutic use , Cognition Disorders/chemically induced , Cognition Disorders/prevention & control , Cognition/drug effects , Dementia/drug therapy , Primary Health Care , Aged , Benzodiazepines/adverse effects , Cognition Disorders/epidemiology , Cognition Disorders/psychology , Dementia/diagnosis , Dementia/psychology , Female , Geriatric Assessment , Humans , Italy/epidemiology , Male , Middle Aged , Neuropsychological TestsABSTRACT
BACKGROUND AND PURPOSE: Drugs with anticholinergic properties might have a negative impact on cognition, but findings are still conflicting. The association was evaluated between anticholinergic drugs and cognitive performance in primary care patients with first cognitive complaints. METHODS: From April 2013 to March 2014, 353 general practitioners administered the Mini-Mental State Examination (MMSE) to patients presenting with first cognitive complaints. Drug history was collected and the anticholinergic cognitive burden (ACB) was scored and categorized as ACB 0, ACB 1 and ACB 2+. A mixed effect linear regression model was used to assess the association between ACB and MMSE score. RESULTS: Of 4249 subjects entering the study (mean age 77 ± 8.2 years, 66.4% women and mean years of schooling 8.9 ± 4.5), 25.8% received at least one drug with anticholinergic action. According to multivariate analysis, and after adjustment for several confounders, subjects with ACB 2+ had a statistically significant lower MMSE score compared with those with ACB 0 (ß -0.63; 95% confidence interval -1.19; -0.07). Subjects with ACB 1 had a non-statistically significant lower MMSE score than those with ACB 0 (ß -0.11; 95% confidence interval -0.37; 0.15). CONCLUSIONS: Anticholinergic medication might affect cognitive function in people with first cognitive complaints. Alternatives should be taken into account when possible, balancing the benefits and harms of these medications.
Subject(s)
Cholinergic Antagonists/adverse effects , Cognition Disorders/chemically induced , Aged , Aged, 80 and over , Body Burden , Cholinergic Antagonists/therapeutic use , Cognition Disorders/epidemiology , Cognition Disorders/psychology , Educational Status , Female , Humans , Male , Neuropsychological Tests , Primary Health Care , Psychomotor Performance , Socioeconomic Factors , Treatment OutcomeABSTRACT
MRI grading of grade II and III gliomas may have an important impact on treatment decisions. Occasionally,both conventional MRI (cMRI) and histology fail to clearly establish the tumour grade. Three cMRI features(no necrosis; no relevant oedema; absent or faint contrast enhancement) previously validated in 196 patients with supratentorial gliomas directed our selection of 68 suspected low-grade gliomas (LGG) that were also investigated by advanced MRI (aMRI), including perfusion weighted imaging (PWI), diffusion weighted imaging(DWI) and spectroscopy. All the gliomas had histopathological diagnoses. Sensitivity and specificity of cMRI preoperative diagnosis were 78.5 and 38.5 %, respectively, and 85.7 and 53.8 % when a MRI was included, respectively. ROC analysis showed that cut-off values of 1.29 for maximum rCBV, 1.69 for minimum rADC, 2.1 for rCho/Cr ratio could differentiate between LGG and HGG with a sensitivity of 61.5, 53.8, and 53.8 % and a specificity of 54.7, 43 and 64.3 %, respectively. A significantly longer OS was observed in patients with a maximum rCBV<1.46 and minimum rADC>1.69 (80 vs 55 months, p = 0.01; 80 vs 51 months, p = 0.002, respectively). This result was also confirmed when cases were stratified according to pathology (LGG vs HGG). The ability of a MRI to differentiate between LGG and HGG and to predict survival improved as the number of a MRI techniques considered increased. In a selected population of suspected LGG,classification by cMRI underestimated the actual fraction of HGG. aMRI slightly increased the diagnostic accuracy compared to histopathology. However, DWI and PWI were prognostic markers independent of histological grade.
Subject(s)
Brain Neoplasms/mortality , Brain Neoplasms/pathology , Glioma/mortality , Glioma/pathology , Magnetic Resonance Imaging/methods , Neoplasm Grading/methods , Adult , Diffusion Magnetic Resonance Imaging/methods , Female , Humans , Magnetic Resonance Angiography/methods , Male , Sensitivity and Specificity , Survival AnalysisABSTRACT
Microscopic Monte Carlo simulations of liquid sheets of copper and tin have been performed in order to study the dependence of the surface tension on the thickness of the sheet. It results that the surface tension is constant with the thickness as long as the sheet remains in one piece. When the sheet is getting thinner, holes start to appear, and the calculated surface tension rapidly decreases with thickness until the sheet becomes totally unstable and forms a cylinder. We assume here that this decrease is not due to a confinement effect as proposed by Werth et al. [Physica A 392, 2359 (2013)] on Lennard-Jones systems, but to the appearance of holes that reduces the energy cost of the surface modification. We also show in this work that a link can be established between the stability of the sheet and the local fluctuations of the surface position, which directly depends on the value of the surface tension. Finally, we complete this study by investigating systems interacting through different forms of Lennard-Jones potentials to check if similar conclusions can be drawn.
ABSTRACT
In this study, the thermodynamic properties of association of some inorganic ions (ClO4(-) and SO4(2-)) with ß-cyclodextrins (ß-CD) in aqueous solution are determined under both free ß-CD and surface confined ß-CD conditions using atomistic simulations. The potential of mean force (PMF) is calculated as a function of the environment and the thermodynamic properties of association are deduced by integrating the free energy profiles. No inclusion complex between SO4(2-) and ß-CD is detected. Nevertheless, the PMF curve obtained for gold-confined CD seems to evidence a small minimum at a larger separation distance that shows specific interactions such as hydrogen bonding outside the cavity. As concerns ClO4(-), our simulations reveal the formation of an inclusion complex with free ß-CD in perfect agreement with the available experimental results. Nevertheless, we do not detect any formation of the host-guest inclusion complex under heterogeneous conditions. Finally, the differences observed as a function of the anions are interpreted through an atomistic description. The general trend of weaker complex stabilities with the increasing free energy of hydration of the anions is found in homogeneous systems.
ABSTRACT
An outbreak of bovine besnoitiosis in three female, 15-18 months old beef cattle in central Italy is here described. All the animals were born in central Italy without any recent contact with imported animals. The animals were in poor body conditions and showed symptoms and clinical signs consistent with chronic besnoitiosis. The diagnosis was confirmed by histopathologic examinations of skin biopsies and whole body at necropsy, showing typical 50-100 µ cysts engulfing superficial dermis in skin and lamina propria in mucosae; lesions were confined to skin and respiratory mucosae, and cysts were not seen in any other tissue. Bovine besnoitiosis is rapidly spreading among European countries and in our case the affected animals were born in the farm and not recent admission was referred, so it is likely to consider this as an autoctone outbreak of the disease in Italy. This case, taken together with other recently reported ones, suggest to consider Italy among potentially endemic areas for bovine besnoitiosis.
Subject(s)
Cattle Diseases/parasitology , Coccidiosis/veterinary , Disease Outbreaks/veterinary , Animals , Cattle , Cattle Diseases/epidemiology , Chronic Disease , Coccidiosis/epidemiology , Coccidiosis/parasitology , Communicable Diseases, Emerging/epidemiology , Communicable Diseases, Emerging/veterinary , Female , Italy/epidemiologyABSTRACT
OBJECTIVES: to provide a revised version of earlier guidelines published in 2006. BACKGROUND: primary dystonias are chronic and often disabling conditions with a widespread spectrum mainly in young people. DIAGNOSIS: primary dystonias are classified as pure dystonia, dystonia plus or paroxysmal dystonia syndromes. Assessment should be performed using a validated rating scale for dystonia. Genetic testing may be performed after establishing the clinical diagnosis. DYT1 testing is recommended for patients with primary dystonia with limb onset before age 30, and in those with an affected relative with early-onset dystonia. DYT6 testing is recommended in early-onset or familial cases with cranio-cervical dystonia or after exclusion of DYT1. Individuals with early-onset myoclonus should be tested for mutations in the DYT11 gene. If direct sequencing of the DYT11 gene is negative, additional gene dosage is required to improve the proportion of mutations detected. A levodopa trial is warranted in every patient with early-onset primary dystonia without an alternative diagnosis. In patients with idiopathic dystonia, neurophysiological tests can help with describing the pathophysiological mechanisms underlying the disorder. TREATMENT: botulinum toxin (BoNT) type A is the first-line treatment for primary cranial (excluding oromandibular) or cervical dystonia; it is also effective on writing dystonia. BoNT/B is not inferior to BoNT/A in cervical dystonia. Pallidal deep brain stimulation (DBS) is considered a good option, particularly for primary generalized or cervical dystonia, after medication or BoNT have failed. DBS is less effective in secondary dystonia. This treatment requires a specialized expertise and a multidisciplinary team.
Subject(s)
Botulinum Toxins/therapeutic use , Deep Brain Stimulation , Dystonia/diagnosis , Dystonia/therapy , Dystonic Disorders/diagnosis , Dystonic Disorders/therapy , Dystonia/genetics , Dystonia/physiopathology , Dystonic Disorders/genetics , Dystonic Disorders/physiopathology , Globus Pallidus/physiopathology , Globus Pallidus/surgery , Humans , Molecular Chaperones/geneticsABSTRACT
BACKGROUND: Epidemiological studies on the association between allergic disorders, T-helper type 2 (Th2) mediated, and multiple sclerosis (MS), a T-helper type 1 (Th1)/Th17-mediated disease, provided conflicting results. OBJECTIVE: The aim of this study was to further examine the association between allergic disorders and MS. METHODS: The association between MS and previous medical history of any type of allergy has been investigated in a population-based case-control study conducted in Northern Italy, based on telephone interviews to 423 cases and 643 population controls (refusal rates 3.7% and 9.4%, respectively). Controls were a random sample of the general population. RESULTS: A history of atopic allergies seems to confer protection against MS (odds ratio [OR], 0.58; 95% confidence interval [CI], 0.38-0.89; P = 0.012). In particular, the prevalence of allergic asthma was 4.9% in people with MS and 12% in control subjects (OR = 0.38; 95% CI 0.22-0.66, P < 0.01). No association was found between MS and nonatopic allergies. CONCLUSIONS: Our findings are confirmatory of the putative protective effect of Th2-mediated disorders on Th1 immune responses associated with MS. A unifying theory on the mechanisms by which previous history of atopic allergies may modify the risk of MS is still lacking.
Subject(s)
Hypersensitivity/epidemiology , Multiple Sclerosis/epidemiology , Adolescent , Adult , Case-Control Studies , Female , Humans , Hypersensitivity/immunology , Italy/epidemiology , Logistic Models , Male , Middle Aged , Multiple Sclerosis/immunology , Multiple Sclerosis/prevention & control , Odds Ratio , Population Surveillance , Prevalence , Registries , Risk Assessment , Risk Factors , Th1 Cells/immunology , Th2 Cells/immunology , Young AdultABSTRACT
BACKGROUND: Azathioprine (AZA) is an immunosuppressive drug widely prescribed for the treatment of multiple sclerosis (MS) until the first half of the 1990s. It could be an alternative to interferon beta because it is less expensive. Concerns about its safety, mainly a possible increased risk of malignancy, have been raised. This systematic review aimed to determine the trade off between the benefits and risks of azathioprine in MS. OBJECTIVES: To compare azathioprine with placebo. To assess the effect of azathioprine on major clinical outcomes (ie, disability progression and relapses) in patients with MS, and to evaluate the drug's safety. METHODS: The Cochrane MS Group search strategy was adopted to identify relevant articles. All randomised controlled trials comparing azathioprine treatment of a least 1 year duration with placebo for patients with MS were eligible for the review. Cohorts, case controls, case series and case reports were also considered to assess adverse effects. Regulatory agencies were additional sources of information for adverse effects. More details are available in the full review.
Subject(s)
Azathioprine/therapeutic use , Immunosuppressive Agents/therapeutic use , Multiple Sclerosis/drug therapy , Azathioprine/adverse effects , Humans , Immunosuppressive Agents/adverse effects , Randomized Controlled Trials as Topic , Treatment OutcomeABSTRACT
BACKGROUND: Ultrasound guidance for central venous cannulation is advised by recent guidelines, but is not being applied in everyday practice. The purpose of this study was to determine the reduction in complications when applying an ultrasound locating device for internal jugular vein catheterization. METHODS: An observational study was conducted from November 2004 to October 2005 in a tertiary neurosurgical hospital on 300 patients undergoing internal jugular vein cannulation using an ultrasound technique. Patients were not randomized and operators were trained using theoretical and practical courses. Prior to the study, the investigators, who were consultant anaesthesiologists, had to perform at least 20 successful supervised cannulations. RESULTS: Cannulation was successful in all cases. The incidence of arterial puncture was 2.7%, and multiple venous punctures represented the main minor complication (14%). Bivariate analysis of the overall complications revealed no significant correlation with age group, American Society of Anesthesiologists' (ASA) classification, body mass index, or position and diameter of the vein. CONCLUSIONS: Ultrasound cannulation of the internal jugular vein minimized complications. These could be avoided when new ultrasound probes and specific needles are introduced.
Subject(s)
Catheterization/adverse effects , Jugular Veins/diagnostic imaging , Female , Humans , Male , Treatment Outcome , UltrasonographyABSTRACT
BACKGROUND: Azathioprine is the most widely used immunosuppressive treatment in multiple sclerosis (MS). It is an alternative to interferon beta for treating MS also because it is less expensive. Concerns about its safety, mainly a possible increased risk of malignancy, has limited its use. This systematic review aimed to determine the trade off between the benefits and risks of azathioprine in multiple sclerosis. OBJECTIVES: To compare azathioprine versus placebo. To determine the effect of azathioprine on major clinical outcomes, i.e., disability progression and relapses in patients with multiple sclerosis. SEARCH STRATEGY: The Multiple Sclerosis Group's Trials Register, The Cochrane Central Register of Controlled Trials (CENTRAL- Issue 4, 2006), Cochrane Database of Systematic Reviews (CDSR - Issue 4, 2006), Database of Abstracts of Reviews of Effectiveness (DARE - searched 28.12.06) MEDLINE (PubMed) (1966 to December 2006), EMBASE (1980 to December 2006). Journals and reference lists were hand searched for relevant articles both to benefit and adverse effects. Regulatory agencies were additional sources of information for adverse effects. SELECTION CRITERIA: All parallel group randomised controlled trials (RCTs) comparing azathioprine treatment of a least one year duration with placebo for patients with multiple sclerosis. Cohorts, case controls, case series and case reports were also used to assess adverse effects. DATA COLLECTION AND ANALYSIS: Potentially relevant references were evaluated and all data extracted by two independent authors. MAIN RESULTS: The five trials that met our criteria included 698 randomised patients: data from 499 (71.5%) were available for analysis of relapse frequency in patients at one year's, from 488 (70%) at two years' and from 415 (59.5%) at three years' follow-up. Azathioprine reduced the number of patients who had relapses during the first year of treatment (relative risk reduction [RRR] =20%; 95% CI = 5% to 33%), at two years' (RRR =23%; 95% CI = 12% to 33%) and three years' (RRR =18%; 95% CI = 7% to 27%) follow-up. These results were consistent in sensitivity analysis. There was no heterogeneity among the studies. Data from only three small trials with a total of 87 patients were available to calculate the number of patients who progressed during the first two to three years. There was a statistically significant benefit (RRR = 42%; 95% CI = 7% to 64%) of azathioprine therapy at three years' follow-up; this result was robust after sensitivity analyses and there was no heterogeneity among the trials. Gastrointestinal disturbances, bone marrow suppression and hepatic toxicity were greater in the azathioprine group rather than in the placebo group; they were anticipated, and, by monitoring and dosage adjustment, were easily managed. Withdrawals due to adverse effects were few, occurring mostly during the first year of azathioprine treatment and mainly due to gastrointestinal intolerance (5%). Data from the trials and from cohort and case controls studies available in the literature did not show an increase in risk of malignancy from azathioprine. A possible long-term risk of cancer from azathioprine may be related to a treatment duration above ten years and cumulative doses above 600 g. AUTHORS' CONCLUSIONS: Azathioprine is an appropriate maintenance treatment for patients with multiple sclerosis who frequently relapse and require steroids. Cumulative doses of 600 g should not be exceeded in relation to a possible increased risk of malignancy. Considering the trade off between the benefits and harms, azathioprine is a fair alternative to interferon beta for treating multiple sclerosis. A logical next step for future trials would seem the direct comparison of azathioprine and interferon beta. In fact the direct comparison between these two widely used treatments in multiple sclerosis has not been made.
Subject(s)
Azathioprine/therapeutic use , Immunosuppressive Agents/therapeutic use , Multiple Sclerosis/drug therapy , Azathioprine/adverse effects , Humans , Immunosuppressive Agents/adverse effects , Neoplasms/chemically induced , Randomized Controlled Trials as TopicABSTRACT
BACKGROUND: Clinical and experimental data suggest that certain dietary regimens, particularly those including polyunsaturated fatty acids (PUFAs) and vitamins might improve outcomes in people with multiple sclerosis (MS). Diets and dietary supplements are much used by people with MS in the belief that they might improve disease outcomes. OBJECTIVES: We performed a Cochrane review of all randomised trials of dietary regimens for MS with the aim of answering MS consumers' questions regarding the efficacy and safety of these interventions. SEARCH STRATEGY: We searched the Cochrane MS Group trial register (February 2006), Cochrane Central Register of Controlled Trials (CENTRAL), Cochrane Library, Issue 1, 2006, MEDLINE (PubMed) (1966 to March 2006), EMBASE (1974 to March 2006) and the bibliographies of papers found. SELECTION CRITERIA: All randomised controlled trials comparing a specific dietary intervention, diet plan or dietary supplementation, with no dietary modification or placebo, were eligible. DATA COLLECTION AND ANALYSIS: Two reviewers independently selected articles, assessed trial quality and extracted data. Trial quality was poor, particularly as regards descriptions of randomisation, blinding and adverse event reporting. Some studies had large numbers of drop-outs; dropouts were never included in the analyses. MAIN RESULTS: PUFAs did not have a significant effect on disease progression, measured as worsening of Disability Status Scale. Omega-6 fatty acids (11-23 g/day linoleic acid) had no benefit in 75 relapsing remitting (RR) MS patients (progression at two years: relative risk (RR)=0.78, 95% CI [0.45 to 1.36]) or in 69 chronic progressive (CP) MS patients (RR=1.67, 95% CI [0.75 to 3.72]. Linoleic acid (2.9-3.4 g/day) had no benefit in CPMS (progression at two years: RR=0.78, 95% CI [0.43 to 1.42]). Slight decreases in relapse rate and relapse severity were associated with omega-6 fatty acids in some small studies, however these findings are limited by the limited validity of the endpoints.Omega-3 fatty acids had no benefit on progression at 12 months in 14 RRMS patients or at 24 months in 292 RRMS patients (RR=0.15, 95% CI [0.01 to 3.11], p= 0.22 at 12 months, and 0.82 95% CI [0.65 to 1.03], p=0.08, at 24 months). The low frequency of reported adverse events suggests no major toxicity associated with PUFA administration. No studies on vitamin supplementation and allergen-free diets were analysed as none met the eligibility criteria. AUTHORS' CONCLUSIONS: PUFAs seem to have no major effect on the main clinical outcome in MS (disease progression), and does not substantially affect the risk of clinical relapses over 2 years. However, the data available are insufficient to assess any potential benefit or harm from PUFA supplementation. Evidence bearing on the possible benefits and risks of vitamin supplementation and antioxidant supplements in MS is lacking. More research is required to assess the effectiveness of diets interventions in MS.
Subject(s)
Dietary Supplements , Fatty Acids, Unsaturated/administration & dosage , Multiple Sclerosis/diet therapy , Fatty Acids, Omega-3/administration & dosage , Humans , Randomized Controlled Trials as TopicABSTRACT
Heritability parameters of resistance to gastro-intestinal strongylids, measured as FEC (Faecal Egg Count), were evaluated in the Appenninica sheep breed. FEC heritability coefficient was 0.11 +/- 0.061 while FEC repeatability coefficients were 0.58 +/- 0.085 and 0.76 +/- 0.223 in adult females and lambs respectively. Subjects were classified, based on FEC, into three different levels of resistance to strongylids. Ewes belonging to the 'resistant class' should be conveniently exploited in mating schemes, in order to provide a method, alternative to drug administration, for a long-term parasite control; this would result particularly helpful under those production systems, such as organic farming, where the use of drugs is not allowed or limited.
Subject(s)
Intestinal Diseases, Parasitic/veterinary , Sheep Diseases/genetics , Strongylida Infections/veterinary , Animals , Breeding , Female , Genetic Predisposition to Disease , Immunity, Innate/genetics , Intestinal Diseases, Parasitic/genetics , Intestinal Diseases, Parasitic/parasitology , Seasons , Sheep Diseases/parasitology , Species Specificity , Strongylida Infections/genetics , Strongylida Infections/parasitologyABSTRACT
Four hundred and sixty records of patients with primary torsion dystonia (296 women and 164 men) were evaluated. The mean age at disease onset was 48.3 +/- 17.7 years; 13 patients carried the DYT1 CAG deletion. The distribution of age at onset was represented by a bi-modal curve, with a nadir at 21 year separating early onset from late onset cases. In 15.9% of cases there was a positive family history of dystonia. Cranial, cervical or lower limb onset was more common amongst women (M:F ratios were 1:2.7, 1:1.9, and 1:3); by contrast, onset in the upper limb was more common in men (M:F ratio 2.2:1). As expected, disease progression was more pronounced in cases with early onset; it was reckoned that onset at or above 32 years was associated with a negligible likelihood to progress to a generalized form. The mean age at onset of familial cases was 44.8 +/- 11.2 years, significantly lower than the mean age at onset of sporadic cases (53.5 +/- 13.4 years). Familial cases were characterized by more sites involved throughout disease course. Familial cases had a higher tendency to progress to a segmental or generalized form than sporadic cases.
Subject(s)
Dystonia Musculorum Deformans/epidemiology , Dystonia Musculorum Deformans/genetics , Adolescent , Adult , Age of Onset , Aged , Aged, 80 and over , Child , Child, Preschool , Disease Progression , Female , Gene Deletion , Humans , Infant , Male , Middle Aged , Predictive Value of Tests , Registries , Retrospective StudiesABSTRACT
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder involving both upper and lower motor neurons, leading inexorably to death within a few years. Although our understanding of the pathogenesis of this disease has grown at a very fast rate in recent years, we do not yet have effective treatment options that can positively impact the quality of life (QoL) of these patients. Interestingly, increasing experimental evidence suggests that oxidative stress is involved in the pathogenesis of ALS and that vitamin E could reduce neuronal damage. Hence, in this observational study we determined the QoL in 33 ALS patients taking or not taking vitamin E supplementation (600 mg/day), using the Italian version of the Short-Form 36-Item Health Survey (SF-36). No differences were seen between the two groups of patients, therefore we do not recommend routine use of vitamin E in ALS patients, at least in the absence of randomised clinical trials specifically designed for addressing this issue.
Subject(s)
Amyotrophic Lateral Sclerosis/drug therapy , Quality of Life , Vitamin E/therapeutic use , Vitamins/therapeutic use , Aged , Aged, 80 and over , Dietary Supplements , Female , Follow-Up Studies , Health Surveys , Humans , Male , Middle Aged , Neuroprotective Agents/therapeutic use , Riluzole/therapeutic useABSTRACT
To review the literature on primary dystonia and dystonia plus and to provide evidence-based recommendations. Primary dystonia and dystonia plus are chronic and often disabling conditions with a widespread spectrum mainly in young people. Computerized MEDLINE and EMBASE literature reviews (1966-1967 February 2005) were conducted. The Cochrane Library was searched for relevant citations. Diagnosis and classification of dystonia are highly relevant for providing appropriate management and prognostic information, and genetic counselling. Expert observation is suggested. DYT-1 gene testing in conjunction with genetic counselling is recommended for patients with primary dystonia with onset before age 30 years and in those with an affected relative with early onset. Positive genetic testing for dystonia (e.g. DYT-1) is not sufficient to make diagnosis of dystonia. Individuals with myoclonus should be tested for the epsilon-sarcoglycan gene (DYT-11). A levodopa trial is warranted in every patient with early onset dystonia without an alternative diagnosis. Brain imaging is not routinely required when there is a confident diagnosis of primary dystonia in adult patients, whereas it is necessary in the paediatric population. Botulinum toxin (BoNT) type A (or type B if there is resistance to type A) can be regarded as first line treatment for primary cranial (excluding oromandibular) or cervical dystonia and can be effective in writing dystonia. Actual evidence is lacking on direct comparison of the clinical efficacy and safety of BoNT-A vs. BoNT-B. Pallidal deep brain stimulation (DBS) is considered a good option, particularly for generalized or cervical dystonia, after medication or BoNT have failed to provide adequate improvement. Selective peripheral denervation is a safe procedure that is indicated exclusively in cervical dystonia. Intrathecal baclofen can be indicated in patients where secondary dystonia is combined with spasticity. The absolute and comparative efficacy and tolerability of drugs in dystonia, including anticholinergic and antidopaminergic drugs, is poorly documented and no evidence-based recommendations can be made to guide prescribing.
Subject(s)
Dystonia/diagnosis , Dystonia/therapy , Botulinum Toxins/therapeutic use , Brain/pathology , Counseling , Denervation , Dopamine Antagonists/therapeutic use , Dystonia/classification , Dystonia/genetics , Genetic Techniques , Humans , Magnetic Resonance Imaging , Practice Guidelines as Topic , SyndromeABSTRACT
A combined serological and PCR method for the detection of Listeria monocytogenes infection in symptomatic and asymptomatic ovine flocks was evaluated. Seventy-eight milk samples and 157 serum samples were analysed using a L. monocytogenes PCR detection kit and an anti-listeriolysin O IgG immunoassay kit. The combined use of these commercial kits allowed a rapid and effective detection of L. monocytogenes infection in both the early stage, before seroconversion, and in a later phase, even after antibiotic therapy.