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Biomedical subjects

E Beghi

Publications and source records attributed to E Beghi.

At least 19 recordsLinked to original sources
PubMed2007

Incidence of ALS in Lombardy, Italy.

OBJECTIVE: To assess the incidence and trends of ALS in a large population at risk. METHODS: This study was performed in nine provinces of Lombardy in Northern Italy (population 4,947,554). Patients with newly diagnosed ALS were enrolled during the period 1998 to 2002 through a prospective regional register. For each patient, the main demographic and clinical information was collected by the caring physicians and reviewed by a panel of experts according to the original and revised El Escorial diagnostic criteria. Overall, age- and sex-specific and standardized annual incidence rates were calculated for the entire population and for each year and province separately. RESULTS: We studied 517 patients (M:F ratio 1.3) aged 18 to 92 years (mean 63.6). Onset of symptoms was bulbar in 29% of cases. ALS was definite in 45%, probable in 27%, probable laboratory supported in 3.5%, possible in 15%, and suspected in 10%. Mean disease duration at diagnosis was 10.6 months. The standardized incidence rate was 2.09 per 100,000/year (95% CI: 1.17 to 3.18). The rate, which was 2.43 in men and 1.76 in women, tended to increase up to ages 65 to 74 and to decrease thereafter. The rate was unchanged over time and presented moderate variations across provinces. The incidence rate of definite ALS was 0.93 (spinal-onset ALS 1.35; bulbar-onset ALS 0.74) and was consistently higher in men with spinal-onset ALS vs men with bulbar-onset ALS and women. CONCLUSIONS: The incidence of ALS varied according to age, sex, and site of onset. No temporal and geographic clusters were detected over a 5-year period.

Adolescent↗
PubMed2006

The effectiveness of hospitalization in the treatment of paediatric idiopathic headache patients.

BACKGROUND: Headache is a disease that has a high social impact in the paediatric as well as in the adult population, often resulting in a significant reduction in the young patient's quality of life, reflected primarily in a greater number of days off school and increasingly frequent recourse to symptomatic drugs. The idea for this study came from the clinical impression that some paediatric headache patients might benefit more from inpatient than outpatient care. AIM: The aim of our study was to compare the effectiveness of hospitalization to outpatient care of patients with newly diagnosed frequent and disabling headache. METHODS: A pragmatic randomized open-label trial was conducted at the Child Neurology Clinic of the University of Pavia, Italy. Children and adolescents with a 2- to 6-month moderate-to-severe migraine or tension-type headache history were randomized to hospital admission or outpatient assessment and followed for 6 months. The efficacy of the two therapeutic strategies was measured by counting the number of responders in each arm. Other end points included the mean frequency and duration of attacks, the number of drug prescriptions taken to control pain, and the number of patients and physicians expressing satisfaction with treatment. RESULTS: The study population included 27 girls and 23 boys aged 8 through 18 years with migraine (23 cases) or tension-type headache (27 cases). Compared to outpatient assessment, hospital admission was correlated to a significant increase in the number of responders: 0 vs. 44% (1 month), 0 vs. 68% (3 months), and 12 vs. 68% (6 months). The mean frequency and duration of attacks were significantly lower in hospitalized patients (p < 0.0001). Hospitalization was correlated with a significant reduction of patients with severe headache (p < 0.005), a reduction of drug use, and a higher number of satisfied patients and physicians (p < 0.05). Logistic regression analysis confirmed the higher responder rate among hospitalized patients after adjusting for age, sex, diagnosis, and headache characteristics or admission. CONCLUSIONS: We think hospitalization reduces the emotional mechanisms that provoke stress in children and often induce or favour headache attacks. If these mechanisms can be interrupted, the management of disease may become easier and with enduring benefits.

Adolescent↗
PubMed2006

Hemodynamic instability after extracranial carotid stenting.

OBJECTIVE: Hemodynamic instability (hypertension, hypotension and bradycardia) is a well-known complication of carotid endarterectomy. Carotid angioplasty and stenting (CAS) is becoming a valuable alternative treatment for patients with severe carotid stenosis and increased surgical risk. CAS implies instrumentation of the carotid bulb, so baroceptor dysfunction may provoke hemodynamic instability. The aim of this work was to calculate the incidence of this complication and to detect factors to predict it. METHODS: Medical records and angiograms of 51 consecutive patients submitted to CAS for severe atherosclerotic stenosis (40 cases) or postsurgical restenosis (11 cases) were retrospectively reviewed in order to detect the occurrence of intra- and post-procedural hypertension (systolic blood pressure >160 mmHg), hypotension (systolic blood pressure <90 mmHg) and bradycardia (heart rate <60 beats/min). The relationship between clinical, procedural and angiographic factors and the occurrence of hemodynamic instability was assessed with univariate and multivariate analysis (logistic regression). RESULTS: Transient mild systolic post-procedural hypertension occurred in five cases (10%); preprocedural hypertension, asymptomatic stenosis and ipsilateral post-surgical restenosis predicted this. Hypotension with bradycardia also occurred in five cases (10%), one with neurological sequelae. Transient periprocedural bradycardia occurred in 19 cases (37%). Severe bradycardia without hypotension arose in one case only. Factors predicting post-procedural hypotension included the presence of a fibrous plaque and the ratio between the pre- and post-stenting diameter of the internal carotid artery. Peri-procedural bradycardia predicted post-procedural bradycardia. None of these factors were confirmed by multivariate analysis as a significant prognostic predictor. CONCLUSION: Mild systolic hypertension may occur after CAS, but is resolved by medical treatment. Prolonged hypotension and bradycardia may also arise and this can be dangerous because it may cause neurological deterioration due to hypoperfusion. These complications cannot be predicted by clinical, procedural, and angiographic factors.

Aged↗
PubMed2006

Prevalence and characteristics of peripheral neuropathy in hepatitis C virus population.

OBJECTIVE: To assess the prevalence of peripheral neuropathy (PN) and its correlation with cryoglobulinemia (CG) in an unselected, untreated referral hepatitis C virus (HCV) population. PATIENTS AND METHODS: Two hundred and thirty four patients (120 women and 114 men) with untreated HCV infection were consecutively enrolled by seven Italian centres. Clinical neuropathy was diagnosed when symptoms and signs of peripheral sensory or motor involvement were present. Median, ulnar, peroneal, and sural nerves were explored in all patients and distal symmetric polyneuropathy was diagnosed when all explored nerves or both lower limb nerves were affected. Mononeuropathy and mononeuropathy multiplex were diagnosed when one nerve or two non-contiguous nerves with asymmetrical distribution were affected. Screening for CG was done in 191 unselected patients. RESULTS: Clinical signs of PN were observed in 25 of the 234 patients (10.6%). Electrophysiological PN was found in 36 (15.3%). CG was present in 56/191 patients (29.3%). The prevalence of CG increased significantly with age (p<0.001) and disease duration (p<0.05). PN was present in 12/56 (21%) patients with CG and 18/135 (13%) without CG (p=NS). PN increased significantly with age (p<0.001) and logistic regression analysis confirmed age as the only independent predictor of PN (OR 1.10 for each year; 95% CI 1.04 to 1.15; p<0.001). CONCLUSIONS: Electrophysiological examination detected subclinical neuropathy in 11 patients (4.7%). Statistical analysis showed that CG was not a risk factor for PN whereas PN prevalence increased significantly with age.

Adolescent↗
PubMed2006

Signs and symptoms at diagnosis of amyotrophic lateral sclerosis: a population-based study in southern Italy.

Amyotrophic lateral sclerosis (ALS) diagnostic criteria are used to select patients for clinical trials based on different levels of diagnostic certainty, according to the spread of upper (UMN) and lower motoneuron (LMN) signs in different anatomic regions. However, the clinical presentation of ALS patients is extremely variable and this can delay the time to diagnosis and decrease the likelihood for trial entry. The aims of the study were to describe the signs and symptoms of diagnosis in a population-based incident cohort of ALS cases, using the El Escorial (EEC) and the Revised Airlie Diagnostic Criteria (AHC). The source of the study was a prospective population-based registry established in Puglia, southern Italy, in 1997. The diagnosis and the classification of the cases were based on EEC and AHC. All incident ALS cases during the period 1998-1999 were enrolled and followed up. During the surveillance period, we identified 130 ALS incident cases, and bulbar-ALS represented 20% of our cohort. The highest risk for bulbar onset was among subjects aged >75 years [RR: 20.1, 95% confidence interval (CI) 3.4-118.0] compared with subjects aged <55 years and among females compared with males (Relative risk (RR): 2.75, 95% CI: 1-7.3). The vast majority of patients (72%) referred progressive muscle weakness in the limbs as the presenting symptom. Eighty percent of cases presented contemporary bulbar or spinal involvement; UMN signs in the bulbar region were present in 24% of cases and any motoneuronal sign in thoracic region in only 15% of the cases. In this population-based series, progressive muscle weakness was the most common presenting sign; bulbar onset was associated with advanced age and female sex. UMN signs in the bulbar region and any motoneuronal sign in the thoracic region were observed in 20% of our case series. This may represent the main limitation to show the spread of signs during diagnostic assessment for inclusion in epidemiological studies and clinical trials.

Adult↗
PubMed2005

Survival of patients with amyotrophic lateral sclerosis in a population-based registry.

OBJECTIVE: To evaluate the survival of patients with amyotrophic lateral sclerosis (ALS) in an Italian population and to assess the effects of selected prognostic indicators on survival. BACKGROUND: Median survival of ALS patients has been reported to range between 12 and 23 months from diagnosis and between 23 and 36 months from onset of symptoms. Although several negative prognostic factors have been identified, the overall picture still needs clarification. METHODS: We included patients enrolled in an Italian ALS Regional Register (population 4,529,003) during the calendar year 1998. The diagnosis was confirmed by an ad hoc committee using the original El Escorial criteria. Each case was regularly followed up until death or December 31, 2002, whichever came first. Survival was assessed with the Kaplan-Meier method in the whole sample, by level of diagnostic certainty, and by selected prognostic indicators (age, sex, bulbar or spinal onset, and disease duration). Multivariate analysis was done with the Cox proportional hazard function. RESULTS: The sample comprised 79 patients (33 female; 46 male) aged 28-85 years (mean age 64.4 years). Onset of symptoms was bulbar in 30% of cases. Mean symptom duration at diagnosis was 13.3 months. ALS was definite in 43%, probable in 29%, possible in 6%, and suspected in 22%. By December 31, 2002, 56 cases (71%) had died. The cumulative probability of surviving after diagnosis was 78% at 12 months, 56% at 24 months, and 32% at 48 months. Median survival from onset was 39.2 months and from diagnosis 30.6 months. Multivariate analysis confirmed definite ALS at diagnosis and older age as adverse prognostic factors. CONCLUSIONS: Survival of ALS patients in the present sample was slightly longer than previously reported. Better palliative care and supportive treatment may explain the difference. Older age and the presence of definite ALS at diagnosis are poor prognostic predictors.

Adult↗
PubMed2005

Early invasive treatment (endarterectomy vs. stenting) of moderate-to-severe carotid stenosis in patients with transient ischaemic attack or minor stroke.

Carotid endarterectomy (CA) is an effective treatment for the secondary prevention of stroke in patients with carotid stenosis >50%. More recently, carotid angioplasty with stenting (CAS) has been introduced and found to be effective in case series and small clinical trials. Although CAS has been shown not to be inferior to CA, the comparative effects of early treatment (i.e., during the first month after transient ischaemic attack (TIA) or minor stroke) with these techniques are unknown. Early treatment is advocated, as recurrent stroke tends to present frequently in this time period. On this background, we designed a randomised clinical trial comparing the efficacy and safety of CA vs. CAS in patients who had suffered TIA or minor stroke in the antecedent month. The study design and methods of this multicentre pragmatic randomised parallel-group open trial are presented here.

Carotid Stenosis↗
PubMed2005

Classification of amyotrophic lateral sclerosis cases at presentation in epidemiological studies.

Amyotrophic lateral sclerosis (ALS) diagnosis is based exclusively on clinical grounds because of the absence of biological markers and of specific neuroradiological and neurophysiological diagnostic features. A clinical classification system of cases has been introduced (El Escorial Criteria, EEC) and then revised after the inclusion of the neurophysiologic assessment (Airlie House Criteria, AHC) for enrollment of patients in clinical trials. The aim of this study is to present cases at presentation in the early stages of the disease that have difficult allocation both in EEC and AHC. All cases were subjects enrolled through SLAP, a population-based registry based in Puglia, Southern Italy. Although differential diagnosis excluded ALS-mimic syndromes, we identified four cases (out of 130 cases, 3.1%) that did not meet the EEC and AHC at the first visit. Even though the number of unclassifiable cases is small, both EEC and AHC may be restrictive. This precludes the enrollment of ALS cases at an early stage both in observational studies and clinical trials.

Aged↗
PubMed2005

A post-marketing study on immunomodulating treatments for relapsing-remitting multiple sclerosis in Lombardia: preliminary results.

The preliminary results of a post-marketing study on relapsing-remitting multiple sclerosis patients treated with immunomodulating agents attending the Lombardia Region's Multiple Sclerosis Centers are presented. A total of 294 patients treated with Betaferon (67), Avonex (115), Rebif 22 (45), Rebif 44 (18) and Copaxone (49) were included. Relapse frequency consistently decreases at 1 year and continues to decrease after 5 years of treatment, without differences between therapeutic groups. Eighty-seven out of 294 patients (29.6%) discontinued treatment for different reasons. Forty-eight of them shifted to a second therapeutic agent. A different trend, to lower or higher doses of interferon or immunosuppression, according to reasons of discontinuation, was observed.

Adjuvants, Immunologic↗
PubMed2005

The epidemiology of epilepsy in Europe - a systematic review.

Population-based epidemiological studies on epilepsy are available mainly from the UK and the Nordic, Baltic and western Mediterranean countries. No studies were identified from large areas of Europe, especially from the former eastern Europe (except the Baltic countries) and the eastern Mediterranean countries. Based on the prevalence of epilepsy in different studies and accounting for incomplete case identification the estimated number of children and adolescents in Europe with active epilepsy is 0.9 million (prevalence 4.5-5.0 per 1000), 1.9 million in ages 20-64 years (prevalence six per 1000) and 0.6 million in ages 65 years and older (prevalence seven per 1000). Approximately 20-30% of the epilepsy population have more than one seizure per month. Based on the age-specific incidence rates in European studies, the estimated number of new cases per year amongst European children and adolescents is 130,000 (incidence rate 70 per 100,000), 96,000 in adults 20-64 years (incidence rate 30 per 100,000) and 85,000 in the elderly 65 years and older (incidence 100 per 100,000). The proportion of both new and established cases with epilepsy in the young, adults and elderly in individual countries may differ substantially from total European distribution because of differences in age structure.

Age Distribution↗
PubMed2005

Validation of a questionnaire for the detection of cluster headache.

BACKGROUND: In the absence of biological markers, the diagnosis of cluster headache (CH) rests on clinical evidence as reported through ad-hoc interviews. OBJECTIVE: The aim of this study was to validate a 16-item self-administered questionnaire designed to screen CH cases. METHODS: The questions were based on the second edition of the International Headache Society classification (ICHD-II) criteria for CH (n = 12) and on specific disease features (n = 3). Answers to each question were either "Yes,""No," or "Don't know." The validity of this screening tool was assessed using a two-step procedure. In Step 1, the 16 questions were submitted to 30 healthy subjects with different cultural backgrounds to verify content clarity. In Step 2, the questionnaire was given to 71 patients (32 women and 39 men) aged 15 to 78 years (mean 37.5 years), who were seen at the University of Parma Headache Center with a diagnosis of CH (episodic, 17; chronic, 13), migraine (without aura, 18; with aura, 3), and tension-type headache (episodic, 16; chronic, 4) according to the ICHD-II criteria. Sensitivity, specificity, and the positive and negative predictive values were calculated for each question as measures of validity. RESULTS: Severity, unilaterality, and location of pain had the highest sensitivity (100%), but low (34.1%) or fairly low specificity (61.0% and 58.5%, respectively). In contrast, a positive response to lithium or verapamil had a 66.7% sensitivity and a 97.6% specificity. Sensitivity and specificity were 100% and 90.2% for duration of attacks (<180 to 240 minutes), and 90.0% and 92.7% for compulsory movements, respectively. The best discriminatory pattern for symptom detection was unilaterality of pain and the presence of at least five of the following seven features: pain severity and location, duration, frequency and daily recurrence of attacks, rhinorrhea and restlessness as accompanying symptoms. CONCLUSIONS: Our findings confirm that this questionnaire is a useful method for CH screening in epidemiological studies.

Adolescent↗
PubMed2005

Mortality of epilepsy in developing countries.

During the last two decades, there has been a renewed interest in studying epidemiology of epilepsy in developing countries. While there are data on prevalence of epilepsy from many developing countries, there is very little information on the mortality of epilepsy in these same populations. This is because incidence studies of epilepsy are difficult to perform, death certificates are unreliable and often unavailable, and the cause of death is difficult to determine. We report on several studies of mortality in epilepsy in developing countries: Ecuador; the Parsi community of Bombay; a semiurban community in Vasai, India; Mali; Martinique; and Africa. Overall, these studies in general illustrate excess mortality among people with epilepsy when compared with the general population.

Adult↗
PubMed2005

Incidence of amyotrophic lateral sclerosis in southern Italy: a population based study.

BACKGROUND: While the incidence of amyotrophic lateral sclerosis (ALS) is similar across the world (range, 1.0 to 2.5/100,000), a latitude gradient from north to south has been observed. OBJECTIVE: To determine the incidence of ALS in Puglia, a region of south eastern Italy, and to test the latitude gradient hypothesis comparing the present study with findings in studies conducted with the same design in a northern latitude. METHODS: Puglia (4,086,613 residents in 2001) is the site of a multicentre-multisource prospective population based registry established in 1997. All incident ALS cases during the period 1998-99 were enrolled and followed up. Cases were classified using the first and the revised El Escorial criteria. RESULTS: During the study period 130 cases were enrolled. The annual crude incidence for ALS in Puglia for the two year period 1998-99 was 1.6/100,000 (95% confidence interval, 1.3 to 1.9). The incidence was higher for men (incidence rate (IR) = 2.1 (1.7 to 2.7) than for women (IR = 1.2 (0.9 to 1.5)) in all age groups, with a male to female ratio of 1.6. For both men and women, the incidence increased through age 75 and declined rapidly afterwards. The mean annual incidence adjusted by age and sex to the 2001 Italian population was 1.7/100,000 (1.4 to 2.0). CONCLUSIONS: ALS incidence is within a narrow range across countries, with a peak between 65 and 75 years and a higher incidence in men. A north to south latitude gradient of ALS incidence is not supported by the results of cohort studies.

Adolescent↗
PubMed2004

Basic and clinical research on amyotrophic lateral sclerosis and other motor neuron disorders in Italy: recent findings and achievements from a network of laboratories.

An Italian collaborative group on motor neuron disorders, including amyotrophic lateral sclerosis (ALS) and its variants, has been recently created, combining various academic groups and laboratories involved in basic and clinical research. The aim is to exploit all the specific expertise and combine efforts at a national level to better understand and fight these fatal diseases. This review summarizes the achievements of the different groups and outlines prospects for future research. Basic research deals with the etiopathogenesis of motor neuron diseases. In vitro and in vivo models of superoxide dismutase 1 (SOD1) mutations are used to investigate the mechanisms of motor neuron death associated with this gene defect. The role of excitotoxicity, immune response, intracellular aggregates and mitochondrial alterations is studied with an integrated approach, at the molecular and cellular levels. Transgenic mice carrying the human mutated SOD1, and the wobbler mouse, a spontaneous model for motor neuron degeneration, offer unique opportunities for testing new therapies in vivo related or not to SOD1 mutations. Clinical research has focused mostly on the incidence and determinants of ALS in several areas of Italy. The incidence of the disease is now among the highest according to the results of population-based regional registries. Compared to earlier studies, more recent Italian investigations show an increase in the incidence and mortality related to ALS. Findings on the role of environmental risk factors are inconsistent. Methodological issues have also been raised by Italian groups regarding the diagnosis and treatment. The validity of the El Escorial diagnostic classification has been questioned where investigators and carers have not received formal training. Pitfalls and methodological drawbacks of randomized clinical trials have been highlighted based on the results of collaborative trials by Italian investigators. Information is now available on non-pharmacological treatments and palliative care, and the economic aspects and quality of life of ALS patients are being investigated.

Amyotrophic Lateral Sclerosis↗
PubMed2004

Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease: facts and uncertainties underlying the causal link between animal and human diseases.

Following an outbreak of bovine spongiform encephalopathy (BSE) in dairy cows in the United Kingdom (UK), 153 definite and probable human cases of new variant Creutzfeldt-Jakob disease (nvCJD) have been reported, almost exclusively in the UK. Although exposure to the BSE agent is the most plausible interpretation for the occurrence of nvCJD, the causal link between the BSE prion and nvCJD is still debated. This review discusses the pros and cons of nvCJD as a separate nosographic entity, the scientific basis for a correlation between BSE and nvCJD, the validity of the current diagnostic criteria for CJD and nvCJD, the contribution of epidemiology to the detection of a causal relation between BSE and nvCJD, and the present and future directions of the epidemiological research on BSE, CJD and nvCJD.

Animals↗
PubMed2004

Methodology of studies on the epidemiology of headache.

Headache is a common clinical condition. A correct evaluation of its frequency, characteristics and aetiology is a prerequisite for health care planning and for the implementation of preventive and treatment measures. Descriptive and analytic epidemiological studies are the basis to improve the knowledge of the burden of headache in the community. However, the results of the epidemiological studies are heavily dependent on the quality of the design, the correctness of the conduct and the appropriateness of the data analysis. A proper knowledge of the problems in the conduction and interpretation of the epidemiological studies helps the investigators to optimise the study protocols and the practicing physicians to critically view the published results.

Data Interpretation, Statistical↗