Browsing by Subject "Amyotrophic lateral sclerosis"
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- PublicationOpen AccessAdenosine deaminase, not immune to a mechanistic rethink in central nervous system disorders?(Universidad de Murcia, Departamento de Biologia Celular e Histiologia, 2022) Hall, Benjamin; George, Jonathan G.; Allen, Scott P.Adenosine deaminase (ADA) is a purine metabolism enzyme that catalyses the breakdown of adenosine and deoxyadenosine. The enzyme is important in several cellular processes, including the innate immune response and cellular differentiation, and it is also an important enzyme for the maintenance of brain homeostasis, in part due to its regulation of adenosine. Aberrant regulation of ADA enzyme activity has been linked to several neurodegenerative diseases and diseases that can result in neurological impairment. However, the mechanisms behind altered ADA regulation and how this leads to the development of neurological dysfunction are poorly characterised. This review summarises the current research on ADA and its role and regulation in disease pathology, with a focus on the central nervous system (CNS) and the neurodegenerative disease, amyotrophic lateral sclerosis (ALS)
- PublicationOpen AccessNew insights into the pathophysiology of fasciculations in amyotrophic lateral sclerosis: an ultrasound study(Elsevier, 2018) Vázquez Costa, J. F.; Campins Romeu, M.; Martínez Payá, Jacinto Javier; Tembl, J. I.; Baño Aledo, María Elena del; Ríos Díaz, J.; Fornés Ferrer, V.; Chumillas, M. J.; Sevilla, T.; FisioterapiaObjective: To describe the fasciculation pattern in ALS and to analyse its clinical and pathophysiological significance. Methods: Ultrasound of 19 muscles was performed in 44 patients with a recent diagnosis (<90 days) of ALS. The numberof fasciculationswas recorded in each muscle and the muscle thickness and strengthwere additionally measured in limb muscles. A subgroup of patients were electromyographically assessed. Results: US was performed in 835 muscles and EMG was available in 263 muscles. US detected fasciculations more frequently than EMG. Fasciculations were widespread, especially in upper limbs onset patients and in the cervical region. Fasciculations’ number inversely associated with ALSFR-R and body mass index (BMI) and directly with BMI loss and upper motor neuron (UMN) impairment. Our statistical model suggest that fasciculations increasewith the initial lower motor neuron (LMN) degeneration, reach their peak when the muscle became mildly to moderately weak, decreasing afterwards with increasing muscle weakness and atrophy.
- PublicationOpen AccessQuantitative neuromuscular ultrasound analysis as biomarkers in amyotrophic lateral sclerosis(2019-08) Martínez Payá, Jacinto Javier; Vázquez-Costa, Juan F.; Chumillas, Maria J.; Tembl Ferrairó, José I.; Baño Aledo, María Elena del; Ríos Díaz, José; FisioterapiaObjectives: To assess the differences in morphological and texture parameters of median nerve (MN) and abductor pollicis brevis (APB) between amyotrophic lateral sclerosis (ALS) patients and controls. Methods: The cross-sectional area (CSA) of the MN and the muscle thickness (MTh) of APB were measured bilaterally in 59 recently diagnosed ALS patients and 20 matched healthy controls. Echointensity (EI), echovariation (EV) and grey-level co-occurrence matrix (GLCM) texture features of both structures were also analysed. Correlations between these parameters and clinical variables (muscle strength and disability) were analysed. Results: The CSA of MN was significantly lower in ALS patients (MD = - 1.83 mm2 [95% CI = 2.89; - 0.77 mm2]; p = 0.01). ALS patients showed significantly lower MTh (- 2.23 mm [3.16; - 1.30 mm]; p < 0.001) and EV (- 7.40 [11.5; - 3.33]; p = 0.004) and higher EI (21.2 [11.9; 30.6]; p < 0.001) in the APB muscle. No relevant differences were detected in GLCM features for this muscle. The model including all parameters (CSA for MN and MTh, EI and EV for APB) showed an AUC of 82% (sensitivity 87%; specificity 42%). Muscle strength and disability correlated with APB muscle ultrasound parameters but not with those of the MN. Conclusions: APB muscle ultrasound biomarkers (especially MTh and EI) showed better discrimination capacity and correlation with clinical variables than MN biomarkers. However, the combination of both biomarkers increased their ability to detect LMN impairment, suggesting that both biomarkers could be used in a complementary manner for the diagnosis and progression monitoring in ALS.
- PublicationOpen AccessSonoelastography for the Assessment of Muscle Changes in Amyotrophic Lateral Sclerosis: Results of a Pilot Study(Elsevier, 2018) Martínez Payá, Jacinto Javier; Baño Aledo, María Elena del; Ríos Díaz, José; Fornés Ferrer, Victoria; Vázquez Costa, Juan F.; FisioterapiaThe purpose of this study was to assess the sonoelastographic features of four different muscles in patients with amyotrophic lateral sclerosis compared with healthy controls and to evaluate the relationship of these features to muscle strength and other ultrasonographic variables. Fourteen patients with amyotrophic lateral sclerosis and 20 controls were examined using strain sonoelastography scanning. The RGB channel fraction ratio was analyzed with ImageJ software (Version 1.48). Two main sonoelastographic patterns could be distinguished in the controls: a clear predominance of the blue channel (hard areas) and a more heterogeneous pattern with predominance of the green channel (intermediate stiffness). These patterns were also observed in patients, although a higher green channel score was observed in mildly impaired muscles, whereas a higher blue channel score was observed in the most severely impaired muscle. Sonoelastography may be a good complementary biomarker in the detection and monitoring of muscle changes in amyotrophic lateral sclerosis.