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Neuromuscular Laboratory
Published in Maher Kurdi, Neuromuscular Pathology Made Easy, 2021
Neuromuscular pathology service is passionately committed to providing a specialized assessment for the most comprehensive and contemporary diagnosis of neuromuscular diseases in adult and pediatric patients. This service should be presented in a functional, precise lab unit. The laboratory handling of muscle and nerve biopsies is specific and requires specialist knowledge beyond that of routine anatomical pathology services. Without a specialized NML, clinicians would have difficulties diagnosing challenging neuromuscular cases. Indeed, a fully equipped lab dealing with muscle and nerve tissue specimens is essential.
Chronic respiratory failure – pathophysiology
Published in Claudio F. Donner, Nicolino Ambrosino, Roger S. Goldstein, Pulmonary Rehabilitation, 2020
Mafalda Vanzeller, Marta Drummond, João Carlos Winck
Interpretation of results may be complicated in patients with airway obstruction (such as COPD or asthma) because the associated hyperinflation of the lungs itself impairs inspiratory muscle function simply because of the distorted thoracic mechanics, so PIM values in patients with COPD may be as low as one-third to one-half those of age-matched normal subjects (14). Moreover, aging- and malnutrition-associated changes in the diaphragm may further impair PIM in subjects with COPD (14). Nevertheless, an apparently impaired PIM in such patients may not necessarily reflect true muscle weakness. Maximum expiratory pressure is not significantly affected by hyperinflation, however, and can be used as a guide to the presence of true muscle weakness in this situation. In neuromuscular diseases these parameters are frequently affected, and that impairment can vary from mild to very severe.
The respiratory system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Pulmonary lymphatics are present around pulmonary blood vessels at the alveolar level and in the septa and pleura. These drain directly into the mediastinal nodes, particularly in the upper lobes. The lymphatics can be traced to the respiratory bronchioles and continue around small bronchi and bronchioles, forming a plexus outside muscle. The lungs expand and contract by movement of either the diaphragm, supplied by the phrenic nerve (C3–5), or the ribs. The diaphragm lengthens or shortens the thoracic cavity. Elevation or depression of the ribs alters the anterior/posterior diameter of the chest wall. These muscles may be affected in neuromuscular disease. The lungs lie in the pleural cavities, the parietal and visceral layers of which are lined by mesothelial cells; these produce a thin film of fluid.
Is there value in using randomized placebo controlled trials in neuromuscular disease?
Published in Expert Review of Neurotherapeutics, 2021
John R. Bach, Giulio Chiarello, William Weiss, Nizar Souayah
References to placebos and the placebo effect have come down to us from ancient Egypt. The placebo effect on outcomes is considerable and increasing, even in neuromuscular disease (NMD) studies. A transient placebo effect was observed in the CHERISH study of Nusinersen for SMA types 2 and 3. In one of our previous placebo-controlled SMA studies the placebo controls’ muscles also strengthened [1]. As a result of positive placebo effects, there is lower separation between the outcomes of randomized double-blinded placebo-controlled trials (RDBPCTs), especially on subjective parameters. Up to 90% of new antidepressants, antipsychotics, pain medications, and even statins are demonstrating less and less benefits over placebos [2]. Further, unlike for infectious and some oncological diseases, there have been no medical cures for the relatively rare neuromuscular diseases (NMD), for which adequate recruitment to establish subtle differences is especially daunting and problems with placebos and randomization take on additional dimensions.
Iatrogenic dys-appearance: first-person accounts of chronic neuromuscular disease reveal unintended harms of treatment
Published in European Journal of Physiotherapy, 2020
Karen Synne Groven, Jean Braithwaite, Tone Dahl-Michelsen
The vast majority of research on physiotherapy for chronic neuromuscular disease draws on studies with quantitative research designs, and the predominant research questions relate to finding effective interventions. The focus is on measurable effects of various interventions such as endurance training, strength training, stretching and walking. Clinical physiotherapists who specialise in chronic neuromuscular disease emphasise the significance of regular follow-ups, and the need to give priority to physical function as an important effect goal [8]. In a case report published in the Norwegian journal Fysioterapeuten [Physiotherapist], Holtebekk et al. emphasise that in treating chronic neuromuscular disease, the physiotherapist [i.e. themselves] should be regarded as
Is it accurate to classify ALS as a neuromuscular disorder?
Published in Expert Review of Neurotherapeutics, 2020
Michael A. van Es, H. Stephan Goedee, Henk-Jan Westeneng, Tanja C.W. Nijboer, Leonard H. van den Berg
Neuromuscular diseases are a spectrum of disorders that predominantly/exclusively affect the peripheral nervous system. Current insights from clinical, imaging, neuropathological and genetic studies show that ALS exhibits the cardinal features of a neurodegenerative disease. Therefore, classifying ALS as a neuromuscular disease in the strict sense has become untenable. Diagnosing ALS however does require significant neuromuscular expertise and therefore neuromuscular specialists remain best equipped to evaluate this category of patients. Designating motor neuron diseases as a separate category in the ICD-11 is justified and adequately deals with this issue. However, to drive effective therapy development the fields of motor neuron disease and neurodegenerative disorders must come together.