Is Boston questionnaire an alternative to electromyography for evaluation of the surgical outcome for carpal tunnel syndrome?

Objectives: This study aims to identify the optimal follow-up method for evaluation of the surgical outcome for carpal tunnel syndrome (CTS). Patients and methods: Between January 2006 and December 2010, 61 hands of a total of 46 patients (7 males, 39 females; mean age 56.0 +/- 10.4 years; range, 20 to 71 years) with a diagnosis of CTS were retrospectively analyzed. All operations were performed by a single surgeon with a mini-incision distal to the transverse carpal ligament. At a mean follow-up of seven years after surgery, electromyography (EMG) was repeated for all patients. The Boston Carpal Tunnel Questionnaire (BCTQ), Boston Symptom Severity Scale, Boston Functional Status Scale, palmar pinch strength, grip strength, and EMG of the patients were compared before and after surgery. Results: The mean follow-up was 84410 (range, 72 to 104) months. There were significant improvements in the Boston Symptom and Functional Scale scores postoperatively, as well as in the grip and pinch strength. After surgery, EMG findings improved in 83.6% of the patients. However, there was no significant correlation between pre- and postoperative Boston Symptom Severity Scale scores, functional status, pinch and grip strengths, and pre- and postoperative EMG results. Conclusion: Our study results demonstrate that the symptom severity and functional status scores of the BCTQ are favorable, and this tool is reliable and easy-to-apply for the diagnosis and follow-up of CTS surgeries

Peripheral neuropathy in ALS: phenotype association

© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.Amyotrophic lateral sclerosis (ALS) is a rare and progressive neurodegenerative disease mainly affecting upper and lower motor neurons but also causing multisystem involvement, in particular, associated with cognitive changes. Minor sensory fibre dysfunction has been described in the past and confirmed in recent studies. In a multicentre study investigating a population of 88 patients with ALS, the ESTEEM group (a European Telematic Project for quality assurance within Clinical Neurophysiology) reported sensory polyneuropathy (PNP) in 12.5% of the patients, not influenced by age, disease duration and onset region. In this study, we aimed to readdress prevalence of and risk factors for PNP in a larger population of patients with ALS. A large number of variables, including gene mutations, were assessed.info:eu-repo/semantics/publishedVersio

Churg-strauss syndrome presented with acute painful peripheral neuropathy

Churg-Strauss syndrome (CSS) characterised by asthma, sinusitis, hypereosinophilia and peripheral neuropathy is a systemic necrotising vasculitis affecting small and medium sized vessels. Peripheral neuropathy is the most common neurological feature. A 64-year-old man applied to the neurology department with the complaint of pain and numbness in his both legs. He did not define a history of infection. He had been followed by a pulmonologist for a long time because of asthma bronchiale. 75% eosinoplilia was determined in his hemogramme. IgE level was higher also. Thorax CT showed ground-glass attenuation, atelectatic band formation in bilateral lung parenchyma. Axonal asymmetric sensorimotor polyneuropathy was determined in his electrophysiological evaluation. His sural nevre biopsy was consistent with vasculitic neuropathy. We wanted to present the clinical and laboratory findings of a patient with CSS who had acute painful neuropathy because of its rarity

Spreading in ALS: The relative impact of upper and lower motor neuron involvement

© 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.Objective: To investigate disease spread in amyotrophic lateral sclerosis (ALS), and determine the influence of lower (LMN) and upper motor neuron (UMN) involvement. Methods: We assessed disease spread in ALS in 1376 consecutively studied patients, from five European centers, applying an agreed proforma to assess LMN and UMN signs. We defined the pattern of disease onset and progression from predominant UMN or lower motor neuron (LMN) dysfunction in bulbar, upper limbs, lower limbs, and thoracic regions Non-linear regression analysis was applied to fit the data to a model that described the relation between two random variables, graphically represented by an inverse exponential curve. We analyzed the probability, rate of spread, and both combined (area under the curve). Results: We found that progression was more likely and quicker to or from the region of onset to close spinal regions. When the disease had a limb onset, bulbar motor neurons were more resistant. Furthermore, in the same time frame more patients progressed from bulbar to lower limbs than vice-versa, whether predominantly UMN or LMN involvement. Patients with initial thoracic involvement had a higher probability for rapid change. The presence of predominant UMN signs was associated with a faster caudal progression. Interpretation: Contiguous progression was leading pattern, and predominant UMN involvement is important in shortening the time for cranial-caudal spread. Our results can best be fitted to a model of independent LMN and UMN degeneration, with regional progression of LMN degeneration mostly by contiguity. UMN lesion causes an acceleration of rostral-caudal LMN loss.This is an EU Joint Programme - Neurodegenerative Disease Research (JPND) project. The project is supported through national funding organizations under the aegis of JPND - www.jpnd.eu. This project was also partially supported by FCT funding to Neuroclinomics2 (PTDC/EEI-SII/1937/2014).info:eu-repo/semantics/publishedVersio

Emotional lability at disease onset is an independent prognostic factor of faster disease progression in Amyotrophic Lateral Sclerosis

Copyright: © 2019 Barc K et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.Amyotrophic lateral sclerosis (ALS) is a fast progressing neurodegenerative disease leading to quadriplegia, anarthria and respiratory insufficiency. A large variety of phenotypes and disability progression requires individually tailored management. Identification of predictors of poor prognosis may not only improve management, but also allow for more precise patients' stratification for clinical trials or research studies. The aim of the study was to investigate the influence of emotional lability present at disease onset on ALS progression by exploring its direct impact on the decay of the ALS Functional Rating Scale-Revised (ALSFRS-R). The study was performed in a group of 1145 patients from Germany, Poland, Portugal and Turkey between 2014 and 2018. The analysis showed that the presence of emotional lability at ALS onset was linked to a faster decline of ALSFRS-R (0.70 vs 0.50, p<0.0001), in case of either bulbar (0.80 vs 0.65, p<0.05) or limb disease onset (0.59 vs 0.46, p <0.01). It was most prominent in the bulbar subscore of ALSFRS-R. A multiple regression analysis showed a direct influence of emotional lability at ALS onset on disease progression, regardless of age, gender, site of onset, weight loss, cognitive impairment and diagnosis delay (β=0.071; p=0.019). It can therefore be concluded that the presence of emotional lability at the disease onset is an independent factor of faster disease progression in ALS.This study was supported by OnWebDuals project (JNPD 01ED1511B; DZP/2/JPND-III/2015). This is an EU Joint Programme - Neurodegenerative Disease Research (JPND) project. The project is supported through the following funding organization under the aegis of JPND - www.jpnd.eu: Germany, Bundes-ministerium für Bildung und Forschung (BMBF); Poland, Narodowe Centrum Badań i Rozwoju (NCBiR); Portugal, Fundação a Ciência e a Tecnologia (FCT); Sweden, Vetenskapsrådet (VR).info:eu-repo/semantics/publishedVersio

Case report: Revealing the rare—a Brody Disease patient from Turkey expanding the phenotype

Brody Disease is an exceptionally rare, autosomal recessive myopathy attributed to the pathogenic variants in the ATP2A1, which encodes the sarcoplasmic/endoplasmic reticulum Ca (2+) ATPase type 1 protein SERCA1. It was first described by Brody IA in 1969. To date, only thirty-three Brody families with forty-seven patients have been reported in the literature, and the disease prevalence is considered as 1 in 10 million, demonstrating the peculiarity of the disease. Clinical characteristics of Brody Disease include muscle stiffness after exercise, myalgia, and muscle cramps. Brody Disease patients generally have disease onset in the first decade, and genetic diagnosis is delayed as a consequence of both the rareness and the mild course of the disease. Here, we report a Turkish Brody Disease patient with a homozygous c.428G&gt;A p.Arg143Gln (NM_004320.4) missense mutation in the ATP2A1. The male patient, whose symptoms started at the age of 14–15, is now 36 years old. His clinical manifestations are athletic appearance, exotropia, slightly elevated creatine kinase (CK), mild progressive proximal muscle weakness in the lower extremities, muscle cramps, pain and stiffness. The patient described here has a very mild progression with an onset in the second decade, expanding the Brody Disease phenotype. The study also implies that in the era of emerging genetic therapies, the routine testing of patients with myopathies is a prerequisite since not only future therapies will be designed on molecular findings, but also currently available symptomatic and palliative treatment options will be more precisely applied

Utilización del documento de consentimiento informado en la práctica odontológica pública del partido de La Plata (Argentina) - 2003

La odontología como ciencia biológica difiere del pensamiento jurídico en numerosos puntos. La biología es a menudo variante. Por lo tanto el razonamiento biológico lleva implícito una parte de reserva, de incertidumbre y de duda. Por el contrario el pensamiento jurídico tiene que responder a los textos que lo instituyen como los códigos y las leyes, que son de elaboración humana, por lo tanto discernibles y asimilables. Desde la época que el hombre realizaba investigaciones empíricas, hasta nuestros días, han concurrido ciencias y disciplinas de investigación que han generado el crecimiento de diferentes especialidades, aportando elementos que definen y caracterizan su campo de acción. La Odontología Legal, como cualquier rama de la ciencia, es un producto de la evolución del hombre. Por lo tanto varía con el tiempo, es distinta según los estados o los países. El Derecho moderno establece una colaboración entre la ciencia y la ley. En estos casos el grado de responsabilidad del odontólogo es alto, porque tiene la función de auxiliar con sus conocimientos, donde sus dichos pueden influir con fuerza sobre el fallo del magistrado. Es preciso, actualmente que el perito odontólogo, adquiera un cúmulo de conocimientos sobre el espíritu jurídico y los procedimientos a seguir en los distintos fueros.Facultad de Odontología (FOLP

Analysis of routine blood parameters in patients with amyotrophic lateral sclerosis and evaluation of a possible correlation with disease progression—a multicenter study

ObjectiveAmyotrophic lateral sclerosis (ALS) pathogenesis is still unclear, its course is considerably variable, and prognosis is hard to determine. Despite much research, there is still a lack of easily accessible markers predicting prognosis. We investigated routine blood parameters in ALS patients regarding correlations with disease severity, progression rate, and survival. Additionally, we analyzed disease and patients' characteristics relating to baseline blood parameter levels.MethodsWe analyzed creatine kinase (CK), albumin (ALB), creatinine (CREA), total cholesterol (TC), high-density lipoprotein cholesterol (HDL), low-density lipoprotein cholesterol (LDL), and triglycerides (TG) levels around time of diagnosis in 1,084 ALS patients. We carried out linear regression analyses including disease and patients' characteristics with each blood parameter to detect correlations with them. Linear regression models were performed for ALSFRS-R at study entry, its retrospectively defined rate of decay and prospectively collected progression rate. Different survival analysis methods were used to examine associations between blood parameters and survival.ResultsWe found higher CK (p-value 0.001), ALB (p-value &lt;0.001), CREA (p-value &lt;0.001), and HDL levels (p-value 0.044) at time of diagnosis being associated with better functional status according to ALSFRS-R scores at study entry. Additionally, higher CREA levels were associated with lower risk of death (p-value 0.003).ConclusionsOur results indicate potential of CK, ALB, CREA, and HDL as disease severity or progression markers, and may also provide clues to ALS pathogenesis. However, these values are highly dependent on other variables, and further careful, longitudinal analyses will be necessary to prove the relevance of our findings

Trends in the diagnostic delay and pathway for amyotrophic lateral sclerosis patients across different countries

BackgroundAmyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease with a median survival of 2–5 years. An early diagnosis is essential for providing ALS patients the finest management possible. Studies from different countries report a similar median diagnostic delay of around 12 months, which is still far from desirable. We analyzed the diagnostic pathway in different countries in order to identify the major challenges.MethodsWe studied a cohort of 1,405 ALS patients from five different centers, in four different countries (Turkey, Germany, Poland, and Portugal), which collaborated in a common database. Demographic, disease and sociocultural factors were collected. Time from first symptom onset to first medical evaluation and to diagnosis, the specialist assessment and investigations requested were analyzed. Factors contributing to diagnostic delay were evaluated by multivariate linear regression.ResultsThe median diagnostic delay from first symptom onset was 11 months and was similar between centers. Major differences were seen in the time from symptom onset to first medical evaluation. An earlier first medical evaluation was associated with a longer time to diagnosis, highlighting that ALS diagnosis is not straightforward in the early stages of the disease. The odds for ALS diagnosis were superior when evaluated by a neurologist and increased over time. Electromyography was decisive in establishing the diagnosis.ConclusionsWe suggest that a specific diagnostic test for ALS—a specific biomarker—will be needed to achieve early diagnosis. Early referral to a neurologist and to electromyography is important for early ALS diagnosis

The Usability of E-learning Platforms in Higher Education: A Systematic Mapping Study

The use of e-learning in higher education has increased significantly in recent years, which has led to several studies being conducted to investigate the usability of the platforms that support it. A variety of different usability evaluation methods and attributes have been used, and it has therefore become important to start reviewing this work in a systematic way to determine how the field has developed in the last 15 years. This paper describes a systematic mapping study that performed searches on five electronic libraries to identify usability issues and methods that have been used to evaluate e-learning platforms. Sixty-one papers were selected and analysed, with the majority of studies using a simple research design reliant on questionnaires. The usability attributes measured were mostly related to effectiveness, satisfaction, efficiency, and perceived ease of use. Furthermore, several research gaps have been identified and recommendations have been made for further work in the area of the usability of online learning