Correction to: The Italian Dystonia Registry: rationale, design and preliminary findings.

In the original article, Gina Ferrazzano was affiliated to Department of Neurology and Psychiatry, Neuromed Institute IRCCS, Sapienza University of Rome, Pozzilli, Italy.The corrected affiliation should be: Neuromed Institute IRCCS, Pozzilli, IS, Italy.

The Italian Dystonia Registry: rationale, design and preliminary findings.

The Italian Dystonia Registry is a multicenter data collection system that will prospectively assess the phenomenology and natural history of adult-onset dystonia and will serve as a basis for future etiological, pathophysiological and therapeutic studies. In the first 6 months of activity, 20 movement disorders Italian centres have adhered to the registry and 664 patients have been recruited. Baseline historical information from this cohort provides the first general overview of adult-onset dystonia in Italy. The cohort was characterized by a lower education level than the Italian population, and most patients were employed as artisans, builders, farmers, or unskilled workers. The clinical features of our sample confirmed the peculiar characteristics of adult-onset dystonia, i.e. gender preference, peak age at onset in the sixth decade, predominance of cervical dystonia and blepharospasm over the other focal dystonias, and a tendency to spread to adjacent body parts, The sample also confirmed the association between eye symptoms and blepharospasm, whereas no clear association emerged between extracranial injury and dystonia in a body site. Adult-onset dystonia patients and the Italian population shared similar burden of arterial hypertension, type 2 diabetes, coronary heart disease, dyslipidemia, and hypothyroidism, while hyperthyroidism was more frequent in the dystonia population. Geographic stratification of the study population yielded no major difference in the most clinical and phenomenological features of dystonia. Analysis of baseline information from recruited patients indicates that the Italian Dystonia Registry may be a useful tool to capture the real world clinical practice of physicians that visit dystonia patients.

Switching from constant voltage to constant current in deep brain stimulation: a multicenter experience of mixed implants for movement disorders.

BACKGROUND AND PURPOSE: For many years deep brain stimulation (DBS) devices relied only on voltage-controlled stimulation (CV), but recently current-controlled devices have been developed and approved for new implants as well as for replacement of CV devices after battery drain. Constant-current (CC) stimulation has been demonstrated to be effective in new implanted parkinsonian and dystonic patients, but the effect of switching to CC therapy in patients chronically stimulated with CV implantable pulse generators (IPGs) has not been assessed. This report shows the results of a consecutive retrospective data collection performed at five Italian centers before and after replacement of constant-voltage with constant-current DBS devices, in order to verify the clinical efficacy and safety of this procedure. METHODS: Nineteen patients with Parkinson's disease or dystonic syndrome underwent DBS IPG CV/CC replacement. Clinical features and therapy satisfaction were assessed before surgery, 1 week after and 3 and 6 months after replacement. Programming settings and impedances were recorded before removing the CV device and when the CC IPGs were switched on. RESULTS: The clinical outcome of CC stimulation was similar to that obtained with CV devices and remained stable at 3 and 6 months of follow-up. Impedance values recorded for CV and CC IPGs were similar. Ninety-five percent of patients and physicians were satisfied with mixed implants. No adverse events occurred after IPG replacement. CONCLUSION: Replacing CV with CC IPGs is a safe and effective procedure. Longer follow-up is necessary to better clarify the impact of CC stimulation on clinical outcome after chronic stimulation in CV mode.

Impact of bridging with perioperative low-molecular-weight heparin on cardiac and bleeding outcomes of stented patients undergoing non-cardiac surgery.

When patients with coronary stents undergo non-cardiac surgery, bridging therapy with low-molecular-weight heparin (LMWH) is not infrequent in clinical practice. However, the efficacy and safety of this approach is poorly understood. This was a retrospective analysis of patients with coronary stent(s) on any antiplatelet therapy undergoing non-cardiac surgery between March 2003 and February 2012. The primary efficacy endpoint was the 30-day incidence of major adverse cardiac or cerebrovascular events (MACCE), defined as the composite of cardiac death, myocardial infarction, acute coronary syndrome leading to hospitalisation, or stroke. The primary safety endpoint was the 30-day composite of Bleeding Academic Research Consortium (BARC) bleedings ≥ 2. Among 515 patients qualifying for the analysis, LMWH bridging was used in 251 (49 %). At 30 days, MACCE occurred more frequently in patients who received LMWH (7.2 % vs 1.1 %, p=0.001), driven by a higher rate of myocardial infarction (4.8 % vs 0 %, p< 0.001). This finding was consistent across several instances of statistical adjustment and after the propensity matching of 179 pairs. Patients bridged with LMWH also experienced a significantly higher risk of BARC bleedings ≥ 2 (21.9 % vs 11.7 %, p=0.002) compared to those who were not, which remained significant across different methods of statistical adjustment and propensity matching. In conclusion, LMWH bridging in patients with coronary stents undergoing surgery is a common and possibly harmful practice, resulting in worse ischaemic outcomes at 30 days, and a significant risk of bleeding.

Clinical outcome of deep brain stimulation for dystonia: constant-current or constant-voltage stimulation? A non-randomized study.

BACKGROUND AND PURPOSE: Bilateral globus pallidus deep brain stimulation (GPi-DBS) represents an effective and relatively safe therapy for different forms of refractory dystonia. The aim of this study was to assess, retrospectively, the effect of two different stimulation settings during GPi-DBS in 22 patients affected by primary generalized or multi-segmental dystonia. METHODS: Thirteen patients were stimulated using a voltage-controlled setting whilst in the other nine patients a current-controlled setting was used. Clinical features were evaluated for each patient at baseline, 6 months and 12 months after surgery by means of the Burke-Fahn-Marsden Dystonia Rating Scale. RESULTS: Globus pallidus deep brain stimulation was effective in all patients. However, comparing constant-current and constant-voltage stimulation, a better outcome was found in the current-controlled group during the last 6 months of follow-up. CONCLUSIONS: Current-controlled stimulation is effective during GPi-DBS for primary dystonia and it could be a better choice than voltage-controlled stimulation over long-term follow-up.

Systematic quality control for management of ST elevation acute myocardial infarction in setting of local network.

AIM: Adherence to evidence based guidelines, assessed by measuring key indicators, allows to detect, evaluate and improve quality of care. Since 2004 in Carlo Poma Hospital, following the introduction of a network for ST-elevation myocardial infarction (STEMI) management, the authors carried out a clinical database in order to measure quality of care in STEMI patients. MATERIALS AND METHODS: A real time upgradable database was developed, to assess clinical practice in myocardial infarction management. The authors evaluated prevalence and control of risk factors, pharmacological therapies and interventional procedures, pathways and delays to care. RESULTS: From 1 February 2004 to 31 January 2008, 1,714 consecutive patients with myocardial infarction were admitted in the Intensive Care Unit (ICU). Primary percutaneous coronary intervention (PCI) was performed in 85% of STEMI patients. Door to balloon time was greater than 90 min in only 17% of patients, of whom 88% coming from emergency department and 12% transferred by 118. CONCLUSION: In the authors' experience quality indicators proved useful in the management of myocardial infarction. Implementation of 118 and improvement of pre-hospital diagnosis in setting of local network can reduce time to treatment.

Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant.

Pulmonary hypertension (PH) may complicate idiopathic pulmonary fibrosis (IPF) but the prevalence of PH in IPF remains undefined. The present authors sought to describe the prevalence of PH in IPF. The lung transplant registry for the USA (January 1995 to June 2004) was analysed and IPF patients who had undergone right heart catheterisation (RHC) were identified. PH was defined as a mean pulmonary arterial pressure ((Ppa)) > or =25 mmHg and severe PH as a (Ppa) >40 mmHg. Independent factors associated with PH were determined. Of the 3,457 persons listed, 2,525 (73.0%) had undergone RHC. PH affected 46.1% of subjects; approximately 9% had severe PH. Variables independently associated with mild-to-moderate PH were as follows: need for oxygen, pulmonary capillary wedge pressure (P(pcw)) and forced expiratory volume in one second (FEV(1)). Independent factors related to severe PH included the following: carbon dioxide tension, age, FEV(1), P(pcw), need for oxygen and ethnicity. A sensitivity analysis in subjects with P(pcw) <15 mmHg did not appreciably alter the present findings. Pulmonary hypertension is common in idiopathic pulmonary fibrosis patients awaiting lung transplant, but the elevations in mean pulmonary arterial pressure are moderate. Lung volumes alone do not explain the pulmonary hypertension. Given the prevalence of pulmonary hypertension and its relationship with surrogate markers for quality of life (e.g. activities of daily living), future trials of therapies for this may be warranted.

No reflow in patients undergoing primary angioplasty for acute myocardial infarction at high risk: incidence and predictive factors.

AIM: Percutaneous coronary intervention (PCI) is a consolidated therapeutic strategy for the treatment of acute myocardial infarction (AMI), but achieving a TIMI 3 flow does not always correspond to true tissue reperfusion. The aim of the study was to evaluate the incidence and predictive factors of no reflow in patients undergoing primary angioplasty (PCI) for AMI at high risk, in the setting of a provincial cardiological emergency network. METHODS: We retrospectively analyzed the ECGs of 360 consecutive patients undergoing primary PCI, between 2001-2004, recorded before and 90 min after the procedure, and compared them with the angiographic data. RESULTS: The patients were divided into 2 groups: group A (reperfused) with a >50% reduction in ST and group B (no reflow) with a <50% reduction in ST but a TIMI 3 flow in the epicardial vessel. The 2 groups were comparable in terms of mean age, sex, diabetes and AMI site. However, there were statistically significant differences between the groups in terms of precoronary time, Killip class IV, ejection fraction, mean leukocyte count, C-reactive protein, and the periprocedural administration of abciximab. Total mortality was 6%: 14% in group B vs 3% in group A. CONCLUSIONS: Our data show that a fair percentage of patients (24%) with a TIMI 3 flow after PCI during ST-elevation MI do not show ECG signs of effective reperfusion and have a higher in-hospital mortality rate. Precoronary time and the inflammatory phlogistic substrate are important independent predictors of no reflow. Pretreatment with abciximab, particularly if it is not periprocedural, can prevent the occurrence of no reflow.

[Coronary disease: early intervention saves lives. Treatment of acute myocardial infarction in Mantova: results of thirty months of work after the implementation of a province network]. / Malattia coronarica: l'intervento precoce salva la vita. Terapia dell'infarto miocardico acuto a Mantova, risultati di trenta mesi di attivita' dopo l'attivazione di una rete provinciale.

BACKGROUND: Since June 2001, in the province of Mantova, we have undertaken a program for the management of acute myocardial infarction based on the early assessment of patient risk profiles, concerning telematic connections among care centers and on the optimization of in-hospital and out of hospital critical pathways for access to care. MATERIALS AND METHODS: Our network provides connections among the following centers: advanced life support ambulances, seven hospitals, three coronary care units, one cath lab on call 24 h a day for primary angioplasty, and one thoracic surgery division. This program, through its strong telematic platform, allows the early assessment of myocardial infarction and provides primary angioplasty to all high-risk patients, as fibrinolytic treatment is reserved only for low-risk patients admitted in peripheral hospitals. RESULTS: Two hundred and eighty patients with acute myocardial infarction were treated with angioplasty; 224 patients (80%) underwent primary angioplasty, 36 patients (13%) facilitated angioplasty and 20 patients (7%) rescue angioplasty. One hundred and thirty-two patients (47%) were first admitted to Mantova Hospital; 78 patients (28%) were referred to Mantova from peripheral hospitals and 70 patients (25%) were directly transported to the cath lab by advanced life support ambulances. Procedural success was obtained in 98% of patients, with 0.4% intraprocedural mortality. In-hospital mortality was 5.7%, while mortality in cardiogenic shock patients was 36%. The recurrence of acute myocardial infarction occurred in 1% of patients and major bleeding occurred in 2.2% of patients. One patient with cardiogenic shock died during transport. Mean door to balloon time was 67 min with a 42% reduction in the 3rd recruitment period. CONCLUSIONS: This program, developed in the setting of a provincial network for the management of acute myocardial infarction, provided primary angioplasty to all high-risk patients, with a high procedural success rate. Within a few months, time to treatment was minimized by the use of telematic facilities.

Goodpasture's syndrome: a case of delayed appearance of autoantibodies and renal disease.

Goodpasture's syndrome, or anti-glomerular basement membrane (anti-GBM) disease, is a rare pulmonary-renal syndrome that results from autoantibody-mediated destruction of alveolar and glomerular basement membranes. It is defined by the triad of pulmonary hemorrhage (hemoptysis), glomerulonephritis (hematuria), and circulating anti-GBM antibodies. Although kidney disease can occur with or without lung involvement, isolated alveolar hemorrhage as an incipient manifestation of the syndrome is rare. We report the case of a patient with anti-GBM disease who initially presented with seronegative hemoptysis and normal urine. It was not until relapse of his condition that we found acute glomerulonephritis and circulating antibodies. In this report, we briefly review the pathogenesis and clinical manifestations of anti-GBM disease and discuss the relevance of isolated alveolar hemorrhage.

Coronary artery bypass grafts in a patient with isolated cardiac dextroversion.

Despite several controversies, the term "dextrocardia" usually defines a rare type of intrinsic cardiac abnormality due to a rotation disorder and resulting in a right-sided direction of the cardiac axis. According to the majority of experts, the extent of a dextrocardia associated with a situs solitus is termed "dextroversion". In such a rare condition, therefore, the relationships between the cardiac chambers and the other structures (that is superior and inferior venae cavae, liver, stomach) are modified whereas in case of dextrocardia with situs inversus, the relationships between the cardiac chambers and neighboring structures are preserved and the classical "mirror image" is shown. In 95% of cases with dextroversion, an associated cardiac abnormality has been described and, therefore, acquired heart diseases in patients with isolated dextroversion are extremely rare. To our knowledge, the present is the first case report describing a coronary artery bypass graft performed in a patient with isolated dextroversion. The technical aspects of the surgical procedure are also discussed.

Health care rationing in the aged: ethical and clinical perspectives.

This article provides an ethical analysis of the question of whether aged patients' access to health care should be less than, the same or greater than, the access younger patients enjoy, when economic resources are limited. This topic is being urgently considered in the US because managed care is becoming more common and brings with it new challenges to traditional medical ethics, and because the prevalence of the aged is increasing as is the number of patients with Alzheimer's disease (AD). It is also critical throughout the world because new findings suggest that the progression of AD may be retarded or even reversed by providing patients with enriched interpersonal environments. If these findings are valid, it would be inhumane to not consider providing these resources to patients with AD, since these gains would be so meaningful and substantial. Deontological and consequential values influencing this question are presented and evaluated. The theories of Veatch, Callahan and Daniels in regard to allocating health care to the aged are discussed. It is argued that 2 subgroups of aged patients, the isolated and demented, are among the patients worst off because the capacity to enjoy meaningful relationships with others supercedes all others and both groups of patients have lost this capacity. We assert that, on the basis of the principle of justice according to need, these 2 groups of patients' exceptional needs should be prioritised. We then raise the question of whether the majority of the population would be willing to provide these isolated and demented aged patients this care if the new findings proved valid. We conclude that, in light of many people's fear of growing old and dying, and some peoples bias against the aged (particularly in the US), willingness to provide the necessary resources is open to debate. Finally, we provide specific examples of the kinds of interventions which might be optimal for each group of patients. For patients who are cognitively unimpaired, this might be providing home care so that they could remain closer to and in contact with their loved ones. For patients who are cognitively impaired, this might be providing interpersonal support when these patients begin to lose control, rather than applying restraints or using psychotropic medication.

[Heart arrest during dipyridamole scintigraphy: report of 3 cases]. / Arresto cardiaco durante scintigrafia al dipiridamolo: descrizione di tre casi.

Potentially life threatening rythm disturbances, such as third degree A-V block and sinus arrest without "escape rythm", represent infrequent and unexpected events during dipyridamole thallium scintigraphy. We report three cases of cardiac arrest requiring resuscitation maneuvers after dipyridamole infusion during myocardial thallium scan. On the basis of these clinical observations we suggest that a trained cardiologist and resuscitation kit should be available in nuclear lab. Moreover elderly patients may require careful screening for preexisting conduction defects and sick sinus syndrome. When dipyridamole infusion provokes severe bradyarrhythmias not related to myocardial ischemia, a transesophageal electrophysiological study should always be performed.

Left ventricular false aneurysm: an unusually prolonged natural history.

A case of left ventricular false aneurysm of postinfarctual etiology with an unusually prolonged natural history (12 years survival) is reported. A first diagnosis of this rare cardiac complication was made in 1982 on the basis of hemodynamic and echocardiographic results. At that time the patient rejected surgical therapy. Eleven years later the patient came back to our attention after resuscitation from a sudden cardiac death. Hemodynamic and echocardiographic (transesophageal) tests showed a remarkable impairment of left ventricular function and an abnormal enlargement (10 x 8 cm) of a pseudoaneurysmatic cavity full of thrombi. The patient died suddenly in April 1994. We emphasize the unusual, prolonged survival of our patient suffering from an unresected left ventricular false aneurysm of postinfarctual etiology.

[Coronary angioplasty in chronic total occlusion: angiography results, complications, and predictive factors]. / L'angioplastica coronarica nelle occlusioni totali croniche: risultati angiografici, complicanze e fattori predittivi.

The results of coronary angioplasty of chronic total occlusions are still controversial. We examined baseline clinical and angiographic parameters and the success and complication rates of 142 consecutive procedures of coronary angioplasty of chronic occlusions selected among 1084 total procedures performed between January 1989 and May 1993. Procedural success was obtained in 109 procedures (77%) with 4 major cardiac complications (2.8%) consisting in one cardiac death caused by left main artery occlusion following an attempt of dilatation of an occluded left anterior descending artery, two non Q wave myocardial infarctions caused by distal embolization and one cardiac tamponade following artery perforation, successfully treated by pericardiocentesis. Extensive coronary dissection occurred in 24 procedures (17%): 5 caused vessel reocclusion and 19 were successfully treated by prolonged balloon inflation and/or use of long balloons or stent implantation. Among 15 baseline clinical and angiographic variables, none was predictive of cardiac complications or coronary artery dissection. In contrast, procedural success was significantly related with absence of bridging collaterals, duration of occlusion < 3 months and a tapered or thrombotic morphology of occlusion. Presence or absence of collaterals was the most significant variable selected by multivariate discriminant analysis. When patients with bridging collaterals were excluded from the analysis, occlusion morphology was the most important determinant. Success rates were 95% and 82% in patients with a tapered morphology lesion with < 3 months and > 3 months duration, respectively (ns); in contrast, success rates were 79% and 36% in patients with abrupt occlusion morphology of < and > 3 months duration (p = 0.001).

[Stable angina: long-term results]. / Angina stabile: risultati a lungo termine.

We report on 345 patients who underwent percutaneous transluminal coronary angioplasty (PTCA) for stable angina in a 5-year period, with an average follow-up of 34 months (range 12-72). The primary success rate was 94.5%. The late mortality and incidence of myocardial infarction were 1.5% and 2.1% respectively. The rate of recurrence of angina was 34.5% and required PTCA (28.5%) and/or coronary artery bypass grafting (7%). At the end of follow-up 79.5% of patients were free from angina and a further 10% had improved. Our data confirm the importance of PTCA in stable angina.

[Role of cervical infections in the genesis of premature rupture of membranes]. / Ruolo delle infezioni cervicali nella genesi della rottura prematura delle membrane.

In order to assess the importance of cervical infections due to CT, Neisseria Gonorrheae and group B Streptococci in the genesis of PMR, the authors examined 80 pregnant women suffering from this pathology who had been admitted to hospital between the 27th and 36th week of gestation. The only statistically significant causal link (p < 0.01) was cervical CT infection found in 40% of patients with PMR. In these cases we also observed a marked rise in neonatal mortality (10%) and neonatal morbidity (51%). For these reasons careful screening should be carried out during the course of pregnancy in order to identify the infection and treat it appropriately so as to reduce correlated perinatal morbidity and mortality rates.

[Prognosis in patients with myocardial infarction and angiographically normal coronaries]. / La prognosi nei pazienti con infarto miocardico e coronarie angiograficamente normali.

From a survey on 8,000 coronary angiographic studies performed in our Institute between January 1980 and June 1990, 105 patients were identified as having angiographically normal coronary arteries and myocardial infarction (MI). Coronary arteries considered as normal were subdivided in completely normal (Group I), or slightly abnormal, with minimal lesions resulting in less than 30% narrowing of a major artery, defined as mild coronarosclerosis (Group II). Thirty-five patients were excluded from the study, because of the lack of complete follow-up data; the remaining 70 patients represent the study group we examined. The following parameters were examined: sex, age at the time of acute MI, family of ischemic heart disease, hypertension, dyslipidemia, diabetes, smoking, stable or unstable angina before MI, location of the MI, ejection fraction (EF), presence of completely normal coronary arteries or mild coronarosclerosis. Follow-up was obtained by contacting the patients or their families; post infarction angina, reinfarction, sudden death or cardiac death were noted. The subjects were divided in 2 groups, according to the coronary anatomy; Group I consisted of 41 patients with completely normal coronary arteries and Group II consisted of 29 patients with mild coronarosclerosis. No significant statistical difference was noted between the 2 groups regarding age, sex and coronary risk factors (except for dyslipidemia which was significantly more prevalent in the subjects with mild coronarosclerosis). Prevalence of left ventricular impairment (EF < 45%) and coronary events (angina, reinfarction and death) were significantly higher in Group II. There was no significant difference in age or clinical risk factor prevalence between patients with complicated and non complicated clinical course.(ABSTRACT TRUNCATED AT 250 WORDS)