Diaphragm ultrasonography in amyotrophic lateral sclerosis: a diagnostic tool to assess ventilatory dysfunction and disease severity
AbstractBackgroundRespiratory failure represents an unavoidable step in patients with amyotrophic lateral sclerosis (ALS) and other motor neuron diseases (MND). The development of diaphragm ultrasonography (DUS) provides an alternative useful and risk-free tool to supply clinical, functional, and neurophysiological assessment of respiratory muscle weakness. Our aim was to evaluate if sonographic changes (thickness and echogenicity in the costal portion of the diaphragm, at rest and during respiratory movements) may be used in ALS patients to assess disease severity over time, to rule out any risk or discomfort due to traditional neurophysiological investigations.MethodsTwenty ALS patients (mean age, 64.6 ± 10.5 years) were enrolled and data were compared with age-matched healthy volunteers; DUS data were correlated with respiratory function and disease severity scale. Examinations were performed using Telemed Echo-wave II or Esaote MyLabGamma devices in conventional B-Mode.ResultsMean resting thickness was reduced in all cases; changes in thickness during inspiration and expiration were also reduced (p
Conclusions This study demonstrates that cerebral degeneration in ALS is more pronounced in the motor than prefrontal cortex, that multicenter MRS studies are feasible, and that motor tNAA/Ino shows promise as a potential biomarker.
Authors: Valori CF, Guidotti G, Brambilla L, Rossi D Abstract Motor neuron disorders are highly debilitating and mostly fatal conditions for which only limited therapeutic options are available. To overcome this limitation and develop more effective therapeutic strategies, it is critical to discover the pathogenic mechanisms that trigger and sustain motor neuron degeneration with the greatest accuracy and detail. In the case of Amyotrophic Lateral Sclerosis (ALS), several genes have been associated with familial forms of the disease, whilst the vast majority of cases develop sporadically and no defined cause can be...
People living with amyotrophic lateral sclerosis (ALS) and their families were provided with equitable open access to a new dedicated psychology service. An integrative psychotherapeutic model of support was offered, incorporating existential therapy. The therapeutic potential of existential therapy, which was evident here, has not been explored previously in ALS. Cognitive behavioural interventions were used in a minority of cases. One third of all patients attending the Bristol motor neurone (MND) centre elected to use this service.
Amyotrophic Lateral Sclerosis (ALS) is an incurable motor neuron (MN) disorder, characterized by degeneration of MNs leading to progressive paralysis and death within two to five years after diagnosis. Gene therapy is emerging as promising treatment option for patients affected by familial forms of ALS (fALS), representing 10% of all ALS cases. Taking advantage of viral vectors derived from Adeno-Associated Virus serotype 10 (AAV10) and the small nuclear RNA U7, we developed a gene therapy for SOD1-linked ALS (20% of fALS).
Authors: Trostchansky A Abstract Amyotrophic lateral sclerosis (ALS) is a multifactorial neurodegenerative disease involving motor neuron (MN) degeneration in the spinal cord, brain stem and primary motor cortex. The existence of inflammatory processes around MN and axonal degeneration in ALS has been shown. Unfortunately, none of the successful therapies in ALS animal models has improved clinical outcomes in patients with ALS. Therefore, the detection of blood biomarkers to be used as screening tools for disease onset and progression has been an expanding research area with few advances in the development of drugs...
Cell Death &Differentiation, Published online: 30 September 2019; doi:10.1038/s41418-019-0422-6LanCL1 promotes motor neuron survival and extends the lifespan of amyotrophic lateral sclerosis mice
Discussion Benign fasciculations are very common and occur in up to 70% of the general population. They occur at different points in people’s lives. They can be brought out by stress, poor sleep hygiene and caffeine. Caffeine has numerous uses especially for regulating sleep and attention. However too much can cause restlessness, jitteriness and sleep deprivation too. The recommended amount of caffeine for a teenager is
Authors: Ralli M, Lambiase A, Artico M, de Vincentiis M, Greco A Abstract BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the progressive death of motor neurons leading to fatal paralysis. The causes of ALS remain unknown; however, evidence supports the presence of autoimmune mechanisms contributing to pathogenesis. Although several environmental factors have been proposed, the only established risk factors are older age, male gender, and a family history of ALS. To date, there are no diagnostic test for ALS, and clinicians rely on the combination of upper motor neur...
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CONCLUSION The Turkish versions of the ALSAQ short and long forms are valid and reliable tools to be used in the assessment of the quality of life in ALS patients.