Long-term effects of bilateral subthalamic nucleus deep brain stimulation on gait disorders in Parkinson's disease: a clinical-instrumental study.

OBJECTIVE: To assess the long-term effects of bilateral subthalamic nucleus deep brain stimulation (STN-DBS) on gait in a cohort of advanced Parkinson's Disease (PD) patients. METHODS: This observational study included consecutive PD patients treated with bilateral STN-DBS. Different stimulation and drug treatment conditions were assessed: on-stimulation/off-medication, off-stimulation/off-medication, and on-stimulation/on-medication. Each patient performed the instrumented Timed Up and Go test (iTUG). The instrumental evaluation of walking ability was carried out with a wearable inertial sensor containing a three-dimensional (3D) accelerometer, gyroscope, and magnetometer. This device could provide 3D linear acceleration, angular velocity, and magnetic field vector. Disease motor severity was evaluated with the total score and subscores of the Unified Parkinson Disease Rating Scale part III. RESULTS: Twenty-five PD patients with a 5-years median follow-up after surgery (range 3-7) were included (18 men; mean disease duration at surgery 10.44 ± 4.62 years; mean age at surgery 58.40 ± 5.73 years). Both stimulation and medication reduced the total duration of the iTUG and most of its different phases, suggesting a long-term beneficial effect on gait after surgery. However, comparing the two treatments, dopaminergic therapy had a more marked effect in all test phases. STN-DBS alone reduced total iTUG duration, sit-to-stand, and second turn phases duration, while it had a lower effect on stand-to-sit, first turn, forward walking, and walking backward phases duration. CONCLUSIONS: This study highlighted that in the long-term after surgery, STN-DBS may contribute to gait and postural control improvement when used together with dopamine replacement therapy, which still shows a substantial beneficial effect.

Self-managed physical activity in breast cancer survivors: A scoping review.

OBJECTIVE: Breast cancer survivors (BCS) experience many issues of rehabilitative concern due to the treatments they have undergone. Given the chronicity of these outcomes, the increasing number of survivors, and the positive results obtained by supervised exercise, professionals should consider offering self-managed physical activity (PA) programs to this population. Our aim was to map the currently available evidence about self-care rehabilitation for BCS. METHODS: Medline, CINAHL, and Cochrane databases were searched for primary literature. Scoping review methodological frameworks were used to tackle the heterogeneity of the topic. Studies investigating self-managed PA interventions prescribed to adult BCS were included. RESULTS: One hundred-eight studies were included, with sample sizes ranging from 6 to 692 patients. Information was systematically collected in tables displaying study design, type of PA, duration and recommended frequency, professional leading the study, type of supervision, initial training, strategies used to help patients integrate self-care into their daily lives, and self-managed PA efficacy. Tables were produced for every oncological side effect that BCS might experience: lymphedema, arthralgia, cancer-related fatigue, a decline in physical parameters, treatment-related cardiotoxicity, peripheral neurotoxicity, and a possible decline in the quality of life. CONCLUSIONS: Self-managed PA has the potential to improve BCS oncological issues. Professionals can adopt many strategies to support patients and empower them with long-lasting self-care competencies. This scoping review provided a comprehensive and easy-to-consult overview of self-managed PA interventions for BCS. We also provided recommendations for future primary studies and secondary synthesis.

Interplay between speech and gait variables in Parkinson's disease patients treated with subthalamic nucleus deep brain stimulation: A long-term instrumental assessment.

OBJECTIVE: To evaluate correlations between speech and gait parameters in the long term and under different medication and subthalamic nucleus deep brain stimulation (STN-DBS) conditions in a cohort of advanced Parkinson's disease (PD) patients. METHODS: This observational study included consecutive PD patients treated with bilateral STN-DBS. Axial symptoms were evaluated using a standardized clinical-instrumental approach. Speech and gait were assessed by perceptual and acoustic analyses and by the instrumented Timed Up and Go (iTUG) test, respectively. Disease motor severity was evaluated with the total score and subscores of the Unified Parkinson's Disease Rating Scale (UPDRS) Part III. Different stimulation and drug treatment conditions were assessed: on-stimulation/off-medication, off-stimulation/off-medication, and on-stimulation/on-medication. RESULTS: Twenty-five PD patients with a 5-year median follow-up after surgery (range 3-7 years) were included (18 males; disease duration at surgery: 10.44 [SD 4.62] years; age at surgery: 58.40 [SD 5.73] years). In the off-stimulation/off-medication and on-stimulation/on-medication conditions, patients who spoke louder had also the greater acceleration of the trunk during gait; whereas in the on-stimulation/on-medication condition only, patients with the poorer voice quality were also the worst to perform the sit to stand and gait phases of the iTUG. Conversely, patients with the higher speech rate performed well in the turning and walking phases of the iTUG. CONCLUSIONS: This study underlines the presence of different correlations between treatment effects of speech and gait parameters in PD patients treated with bilateral STN-DBS. This may allow us to better understand the common pathophysiological basis of these alterations and to develop a more specific and tailored rehabilitation approach for axial signs after surgery.

Self-managed physical activity in cancer survivors for the management of cancer-related fatigue: A scoping review.

OBJECTIVE: Cancer-related fatigue (CRF) is a disabling chronic condition that cancer survivors could experience during and after recovery and that might benefit from self-managed physical activity (PA) programs. This scoping review aimed to map self-managed PA interventions found in literature for the management of CRF. METHODS: Given the heterogeneity of the topic, scoping review methodological frameworks were used. Pubmed, Cinahl and Cochrane databases were searched for primary literature. Inclusion criteria: self-managed PA meant as any exercise program prescribed by a professional either with or without initial supervision and training which then continued independently for a given time frame; patient-reported fatigue assessment included in the outcome measures. Articles dealing with entirely supervised interventions, dietary or psychological-only therapies, and with palliative care were excluded. RESULTS: Of the 543 experimental or observational studies screened, 63 were included. Of these forty-three studies were randomized controlled trials. Data were summarized in tables describing self-managed interventions according to: type of self-managed activity, frequency and duration, strategies to promote adherence, professionals supervising the treatment, outcome measures, and efficacy. A narrative synthesis was also added to further explain findings. CONCLUSIONS: We collected the available evidence on PA when this was self-managed by patients after prescription by a healthcare provider. Clinicians and researchers should consider incorporating self-care programs in CRF patients' recovery journey gradually, identifying the best strategies to integrate them into daily life. Researchers should specify the characteristics of PA programs when designing new studies. This review highlighted the areas to be investigated for future studies pertaining to self-managed PA.

Freezing of Gait in Parkinson's Disease Patients Treated with Bilateral Subthalamic Nucleus Deep Brain Stimulation: A Long-Term Overview.

Bilateral subthalamic nucleus deep brain stimulation (STN-DBS) is an effective treatment in advanced Parkinson's Disease (PD). However, the effects of STN-DBS on freezing of gait (FOG) are still debated, particularly in the long-term follow-up (≥5-years). The main aim of the current study is to evaluate the long-term effects of STN-DBS on FOG. Twenty STN-DBS treated PD patients were included. Each patient was assessed before surgery through a detailed neurological evaluation, including FOG score, and revaluated in the long-term (median follow-up: 5-years) in different stimulation and drug conditions. In the long term follow-up, FOG score significantly worsened in the off-stimulation/off-medication condition compared with the pre-operative off-medication assessment (z = -1.930; p = 0.05) but not in the on-stimulation/off-medication (z = -0.357; p = 0.721). There was also a significant improvement of FOG at long-term assessment by comparing on-stimulation/off-medication and off-stimulation/off-medication conditions (z = -2.944; p = 0.003). These results highlight the possible beneficial long-term effects of STN-DBS on FOG.

Acute respiratory failure as presentation of late-onset Pompe disease complicating the diagnostic process as a labyrinth: a case report.

BACKGROUND: Acute respiratory failure can be triggered by several causes, either of pulmonary or extra-pulmonary origin. Pompe disease, or type II glycogen storage disease, is a serious and often fatal disorder, due to a pathological accumulation of glycogen caused by a defective activiy of acid α-glucosidase (acid maltase), a lysosomal enzyme involved in glycogen degradation. The prevalence of the disease is estimated between 1 in 40,000 to 1 in 300,000 subjects. CASE PRESENTATION: This case report describes a difficult diagnosis of late-onset Pompe disease (LOPD) in a 52 year old Caucasian woman with acute respiratory failure requiring orotracheal intubation and subsequent tracheostomy for long-term mechanical ventilation 24 h/day. Despite a complex diagnostic process including several blood tests, bronchoscopy with BAL, chest CT, brain NMR, electromyographies, only a muscle biopsy allowed to reach the correct diagnosis. DISCUSSION: The most frequent presentation of myopathies, including LOPD, is proximal limb muscle weakness. Respiratory related symptoms (dyspnea on effort, reduced physical capacity, recurrent infections, etc.) and respiratory failure are often evident in the later stages of the diseases, but they have been rarely described as the onset symptoms in LOPD. In our case, a third stage LOPD, the cooperation between pulmonologists and neurologists was crucial in reaching a correct diagnosis despite a very complex clinical scenario due to different confounding co-morbidities as potential causes of respiratory failure and an atypical presentation. In this patient, enzyme replacement therapy with infusion of alglucosidase alfa was associated with progressive reduction of ventilatory support to night hours, and recovery of autonomous walking.

Feasibility and predictive performance of the Hendrich Fall Risk Model II in a rehabilitation department: a prospective study.

BACKGROUND: Falls are a common adverse event in both elderly inpatients and patients admitted to rehabilitation units. The Hendrich Fall Risk Model II (HIIFRM) has been already tested in all hospital wards with high fall rates, with the exception of the rehabilitation setting. This study's aim is to address the feasibility and predictive performances of HIIFRM in a hospital rehabilitation department. METHODS: A 6 months prospective study in a Italian rehabilitation department with patients from orthopaedic, pulmonary, and neurological rehabilitation wards. All admitted patients were enrolled and assessed within 24 h of admission by means of the HIIFRM. The occurrence of falls was checked and recorded daily. HIIFRM feasibility was assessed as the percentage of successful administrations at admission. HIIFRM predictive performance was determined in terms of area under the Receiver Operating Characteristic (ROC) curve (AUC), best cutoff, sensitivity, specificity, positive and negative predictive values, along with their asymptotic 95% confidence intervals (95% CI). RESULTS: One hundred ninety-one patents were admitted. HIIFRM was feasible in 147 cases (77%), 11 of which suffered a fall (7.5%). Failures in administration were mainly due to bedridden patients (e.g. minimally conscious state, vegetative state). AUC was 0.779(0.685-0.873). The original HIIFRM cutoff of 5 led to a sensitivity of 100% with a mere specificity of 49%(40-57%), thus suggesting using higher cutoffs. Moreover, the median score for non-fallers at rehabilitation units was higher than that reported in literature for geriatric non fallers. The best trade-off between sensitivity and specificity was obtained by using a cutoff of 8. This lead to sensitivity = 73%(46-99%), specificity = 72%(65-80%), positive predictive value = 17% and negative predictive value = 97%. These results support the use of the HIIFRM as a predictive tool. CONCLUSIONS: The HIIFRM showed satisfactory feasibility and predictive performances in rehabilitation wards. Based on both available literature and these results, the prediction of falls among all hospital wards, with high risk of falling, could be achieved by means of a unique tool and two different cutoffs: a standard cutoff of 5 in geriatric wards and an adjusted higher cutoff in rehabilitation units, with predictive performances similar to those of the best-preforming pathology specific tools for fall-risk assessment.

Endobronchial ultrasound-guided transbronchial needle aspiration under conscious sedation with meperidine and midazolam.

Endobronchial Ultrasound guided transbronchial needle aspiration (EBUS-TBNA) is usually performed under general anesthesia or deep sedation with drugs such as Propofol that, at least in Italy, can be administered only by an anesthesiologist. Aim of the study was to assess conscious sedation feasibility, safety and tolerability using Meperidine and Midazolam as administered by Pulmonologist and relevant impact on the efficiency of the sampling procedures.All patients undergoing EBUS-TBNA from February 2013 to July 2014 were examined retrospectively. Efficiency using Meperidine and Midazolam during EBUS-TBNA has been assessed: completion of lymph-nodal sampling, sampling adequacy, diagnostic yield, cough during endoscopic procedure complications and need for procedure repetition with Anesthesiology assistance. Patient satisfaction and cost/effectiveness were also evaluated. One hundred and thirty-four consecutive patients were considered; 97.7% completed the procedure. In 96.9% of cases the prefixed program of lymph-nodal sampling was accomplished. Sampling adequacy was 92,4%. Diagnostic yield was 55%. In 94.7% of cases cough was absent or did not interfere with EBUS-TBNA. The need to repeat the endoscopic procedure occurred in 6 cases but only in 2 the presence of an Anesthesiologist was required. Patient satisfaction was very high, with 95.9% of subjects reporting they would "definitely return". A 27% cost reduction was calculated. EBUS-TBNA under conscious sedation using Meperidine and Midazolam prescribed and administered by pulmonologist without the Anesthesiologist assistance, revealed to be a safe, well tolerated and cost saving procedure. The efficiency of sampling was good, apart from a relatively low diagnostic yield due to different expertise of operators.

Profile of anti-IL-5 mAb mepolizumab in the treatment of severe refractory asthma and hypereosinophilic diseases.

Asthma is a complex disorder frequently associated with a poor symptom control, concomitant morbidity, mortality, and significant health care costs due to lack of compliance or inadequate therapeutic options. Interleukin-5 (IL-5) plays a key role in the pathogenesis of eosinophilic disorders, and in the latest years has become a definite target for treatment. Besides asthma, other hypereosinophilic disorders include the hypereosinophilic syndrome, eosinophilic granulomatosis with polyangiitis, sinonasal polyposis, COPD with eosinophilic airway inflammation, allergic rhinitis, atopic dermatitis, eosinophilic esophagitis. The introduction of mepolizumab, a fully humanized monoclonal antibody that binds to IL-5, may represent a useful therapeutic option to control exacerbations and improve asthma-related quality of life in a subgroup of patients with persistent airway eosinophilia and moderate to severe asthma. Several studies carried out in recent years allow, at present, a careful patient selection for appropriate individualized treatment in severe asthma. Further research is anyway needed in order to better understand the pathogenetic mechanisms of asthma and to find new biomarkers. The high costs of biological agents as compared with standard drugs may be largely offset by increased clinical efficacy and good safety profile in selected patients.

Pharmacoeconomics in COPD and inappropriateness of diagnostics, management and treatment.

Chronic respiratory disorders (CRDs) concern many people and generate important health and social costs. Their global impact (particularly that of COPD) has been the subject of numerous pharmacoeconomic studies published in recent years. These studies confirm the growing impact of COPD in all countries, in terms of the patient and patient's family, and that of society as a whole. The attitude towards COPD management is still largely inadequate, from prevention to diagnosis to drug and non-pharmacologic long-term treatment. The pulmonary specialist, in cooperation with other health professionals, can play a fundamental role in detecting inappropriateness in the clinical course of COPD and provide the basis for a correct assessment of pharmacoeconomic issues. Given the increasing social impact of COPD, the meeting inspiring this review, "COPD a social disease: inappropriateness and pharmacoeconomics. The role of the specialist: present and future", Venice, Italy, 21-22 April 2010, fits in perfectly with the goals and recommendations of GARD (Global Alliance against chronic Respiratory Diseases) of the WHO. GARD has formulated working recommendations: (i) to develop national programs of prevention and control of CRDs, starting from health education campaigns and better knowledge of epidemiology, impact, and relative risk factors; (ii) to provide training and continuing education on prevention and treatment of CRDs, disseminating the existing guidelines; and (iii) to facilitate access to essential treatments and favour adherence to long-term treatment, including drug treatment and pulmonary rehabilitation, particularly amongst disadvantaged sectors of the population.