Apraxia in progressive nonfluent aphasia

The clinical and neuroanatomical correlates of specific apraxias in neurodegenerative disease are not well understood. Here we addressed this issue in progressive nonfluent aphasia (PNFA), a canonical subtype of frontotemporal lobar degeneration that has been consistently associated with apraxia of speech (AOS) and in some cases orofacial apraxia, limb apraxia and/or parkinsonism. Sixteen patients with PNFA according to current consensus criteria were studied. Three patients had a corticobasal syndrome (CBS) and two a progressive supranuclear palsy (PSP) syndrome. Speech, orofacial and limb praxis functions were assessed using the Apraxia Battery for Adults-2 and a voxel-based morphometry (VBM) analysis was conducted on brain MRI scans from the patient cohort in order to identify neuroanatomical correlates. All patients had AOS based on reduced diadochokinetic rate, 69% of cases had an abnormal orofacial apraxia score and 44% of cases (including the three CBS cases and one case with PSP) had an abnormal limb apraxia score. Severity of orofacial apraxia (but not AOS or limb apraxia) correlated with estimated clinical disease duration. The VBM analysis identified distinct neuroanatomical bases for each form of apraxia: the severity of AOS correlated with left posterior inferior frontal lobe atrophy; orofacial apraxia with left middle frontal, premotor and supplementary motor cortical atrophy; and limb apraxia with left inferior parietal lobe atrophy. Our findings show that apraxia of various kinds can be a clinical issue in PNFA and demonstrate that specific apraxias are clinically and anatomically dissociable within this population of patients

The cost-effectiveness of domiciliary non-invasive ventilation in patients with end-stage chronic obstructive pulmonary disease:a systematic review and economic evaluation

Background: Chronic obstructive pulmonary disease (COPD) is a chronic progressive lung disease characterised by non-reversible airflow obstruction. Exacerbations are a key cause of morbidity and mortality and place a considerable burden on health-care systems. While there is evidence that patients benefit from non-invasive ventilation (NIV) in hospital during an acute exacerbation, evidence supporting home use for more stable COPD patients is limited. In the UK, domiciliary NIV is considered on health economic grounds in patients after three hospital admissions for acute hypercapnic respiratory failure. Objective: To assess the clinical effectiveness and cost-effectiveness of domiciliary NIV by systematic review and economic evaluation. Data sources: Bibliographic databases, conference proceedings and ongoing trial registries up to September 2014. Methods: Standard systematic review methods were used for identifying relevant clinical effectiveness and cost-effectiveness studies assessing NIV compared with usual care or comparing different types of NIV. Risk of bias was assessed using Cochrane guidelines and relevant economic checklists. Results for primary effectiveness outcomes (mortality, hospitalisations, exacerbations and quality of life) were presented, where possible, in forest plots. A speculative Markov decision model was developed to compare the cost-effectiveness of domiciliary NIV with usual care from a UK perspective for post-hospital and more stable populations separately. Results: Thirty-one controlled effectiveness studies were identified, which report a variety of outcomes. For stable patients, a modest volume of evidence found no benefit from domiciliary NIV for survival and some non-significant beneficial trends for hospitalisations and quality of life. For post-hospital patients, no benefit from NIV could be shown in terms of survival (from randomised controlled trials) and findings for hospital admissions were inconsistent and based on limited evidence. No conclusions could be drawn regarding potential benefit from different types of NIV. No cost-effectiveness studies of domiciliary NIV were identified. Economic modelling suggested that NIV may be cost-effective in a stable population at a threshold of £30,000 per quality-adjusted life-year (QALY) gained (incremental cost-effectiveness ratio £28,162), but this is associated with uncertainty. In the case of the post-hospital population, results for three separate base cases ranged from usual care dominating to NIV being cost-effective, with an incremental cost-effectiveness ratio of less than £10,000 per QALY gained. All estimates were sensitive to effectiveness estimates, length of benefit from NIV (currently unknown) and some costs. Modelling suggested that reductions in the rate of hospital admissions per patient per year of 24% and 15% in the stable and post-hospital populations, respectively, are required for NIV to be cost-effective. Limitations: Evidence on key clinical outcomes remains limited, particularly quality-of-life and long-term (> 2 years) effects. Economic modelling should be viewed as speculative because of uncertainty around effect estimates, baseline risks, length of benefit of NIV and limited quality-of-life/utility data. Conclusions: The cost-effectiveness of domiciliary NIV remains uncertain and the findings in this report are sensitive to emergent data. Further evidence is required to identify patients most likely to benefit from domiciliary NIV and to establish optimum time points for starting NIV and equipment settings. Future work recommendations: The results from this report will need to be re-examined in the light of any new trial results, particularly in terms of reducing the uncertainty in the economic model. Any new randomised controlled trials should consider including a sham non-invasive ventilation arm and/or a higher- and lower-pressure arm. Individual participant data analyses may help to determine whether or not there are any patient characteristics or equipment settings that are predictive of a benefit of NIV and to establish optimum time points for starting (and potentially discounting) NIV. Study registration: This study is registered as PROSPERO CRD42012003286. Funding: The National Institute for Health Research Health Technology Assessment programme

Results of Polysomnographies and Treatment Strategies in Elderly Patients with Symptoms of Obstructive Sleep Apnea Syndrome

OBJECTIVES: In this study, we evaluated data regarding the management of geriatric patients with symptoms of obstructive sleep apnea syndrome (age, >65 years) who were admitted to our sleep clinic. MATERIAL AND METHODS: Symptoms and sleep data of the patients were retrospectively evaluated, and the patients were reevaluated after treatment. RESULTS: A total of 85 patients with a median age of 69 years were included. Snoring and fatigue were the most common symptoms. Cardiovascular diseases were the most frequently listed comorbidity. The median Epworth sleepiness scale was 10, and based on Berlin sleep questionnaire findings, 63.5% of the participants were in the high-risk group. Eighty-seven percent were diagnosed with obstructive sleep apnea (2/3 of them were positional), and moderate-to-severe obstructive sleep apnea was observed more in women than in men. Only one patient was diagnosed with central sleep apnea. There were positive and linear correlations between increased age and the apnea-hypopnea index, arousal index, Epworth sleepiness scale, and being in a high-risk group according to the Berlin sleep scale; however, there was no correlation between increased age and the number of hypopnea and apnea events. There were also positive and linear correlations between the apnea-hypopnea index and the Epworth sleepiness scale, being in a high-risk group according to the Berlin sleep questionnaire, an increased number of known medical conditions, and increased body mass index. We were able to contact 72 of the 85 patients via phone calls. Patients who adjusted to treatment had positive feedbacks. CONCLUSION: Sleep disorders are observed more in the elderly, and an increasing age is an independent factor for sleep disorders. Besides the usual signs and symptoms of sleep disorders, it should be considered in elderly who have cognitive dysfunction and dementia

Les dysarthries acquises de l'enfant

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Effect of Intensive Voice Therapy on Vocal Tremor for Parkinson Speakers

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Altered pulmonary functions due to biomass smoke in a rural population of Turkish women: a descriptive study

Introduction: Wood or other organic sources of fuel are used as source of energy for heating or cooking particularly in developing countries. The aim of the current study was to evaluate the association between biomass exposure time and parameters of pulmonary function tests. Materials and Methods: Four hundred twenty-four consecutive women who lived and exposed to biomass smoke in a small province in Eastern Turkey were involved. This study was performed with women who had come to pulmonology out-patient clinic with symptom of dyspnea. Results: The independent variables assessed in the study patients were age, BMI, starting age of cooking, hours per day and weeks per month spent cooking, and cooking years; the dependent variables were PFT parameters. Ninety-two (21.6%) patients had an obstructive PFT pattern. Sixty-seven (73%) of these patients were classified as GOLD 2 and 25 (27%) patients were classified as GOLD 3. Seventy-five (17.6 %) of the patients had restrictive lung disease; 54 (72%) of these patients were found to have a mild and 21 (27%) had a moderate restrictive pattern. Increased number of years in cooking and to start cooking at younger ages were a risk factors for the development of obstructive and restrictive disease. There was a statistically significant and negative correlation between increased number of years and the value of FEV1 (r=-0.917; p=<0.001), FEV1/FVC (r=-0.739; p<0.001), and FVC (r=-0.906; p<0.001). The median time of cooking required was 23 years for the development of obstruction, and 25 years for restriction, respectively. Conclusion: Cumulative biomass exposure time is associated with impairment in PFT parameters; results in both obstructive and restrictive lung disease. Biomass exposure is a public health problem and pre-cautions should be taken in order to prevent impaired pulmonary functions

Analysis of vocal tremor and application to Parkinson speech

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