Analysis of the Autonomic Regulation in a Case of Facioscapulohumeral Muscular Dystrophy after Ken Ware Treatment

Abstract This is a study on autonomic neuroscience. In a previous paper in [1], we studied a subject affected from facioscapulohumeral muscular dystrophy before and after Ken Ware treatment (NPT). Using the non linear methodology of the Generalized Mutual Information (GMI) analysis of Sensory Motor Rhythm, we produced detailed results evidencing that the mentioned NPT treatment involved a net improvement of the patient under his subjective psychological condition, and in particular, under the neurological and sensory motor profile. We quantified with accuracy the improvement that the subject realized during such treatment. Of course, previous studies of several authors have evidenced that muscular dystrophies are strongly linked to a profound ANS disfunction. Therefore, the aim of the present study was to analyze the ANS of the subject before and after the treatment. We performed analysis in time as well as in frequency domain and by using non linear methods. The basic result of the paper was that, according to our analysis, the subjects started with a serious ANS disfunction before the NPT treatment and that a net improvement was obtained after this therapy. All the examined parameters resulted strongly altered before the treatment and all they returned in the normal range after the NPT

Muscle Tissue Damage Induced by the Venom of Bothrops asper: Identification of Early and Late Pathological Events through Proteomic Analysis

Citation: Herrera C, Macêdo JKA, Feoli A, Escalante T, Rucavado A, Gutiérrez JM, et al. (2016) Muscle Tissue Damage Induced by the Venom of Bothrops asper: Identification of Early and Late Pathological Events through Proteomic Analysis. PLoS Negl Trop Dis 10(4): e0004599. doi:10.1371/journal. pntd.0004599The time-course of the pathological effects induced by the venom of the snake Bothrops asper in muscle tissue was investigated by a combination of histology, proteomic analysis of exudates collected in the vicinity of damaged muscle, and immunodetection of extracellular matrix proteins in exudates. Proteomic assay of exudates has become an excellent new methodological tool to detect key biomarkers of tissue alterations for a more integrative perspective of snake venom-induced pathology. The time-course analysis of the intracellular proteins showed an early presence of cytosolic and mitochondrial proteins in exudates, while cytoskeletal proteins increased later on. This underscores the rapid cytotoxic effect of venom, especially in muscle fibers, due to the action of myotoxic phospholipases A2, followed by the action of proteinases in the cytoskeleton of damaged muscle fibers. Similarly, the early presence of basement membrane (BM) and other extracellular matrix (ECM) proteins in exudates reflects the rapid microvascular damage and hemorrhage induced by snake venom metalloproteinases. The presence of fragments of type IV collagen and perlecan one hour after envenoming suggests that hydrolysis of these mechanically/structurally-relevant BM components plays a key role in the genesis of hemorrhage. On the other hand, the increment of some ECM proteins in the exudate at later time intervals is likely a consequence of the action of endogenous matrix metalloproteinases (MMPs) or of de novo synthesis of ECM proteins during tissue remodeling as part of the inflammatory reaction. Our results offer relevant insights for a more integrative and systematic understanding of the time-course dynamics of muscle tissue damage induced by B. asper venom and possibly other viperid venoms.Universidad de Costa Rica/[741-B4-660]/UCR/Costa RicaUniversidad de Costa Rica/[741-B6-125]/UCR/Costa RicaUCR::Vicerrectoría de Investigación::Unidades de Investigación::Ciencias de la Salud::Instituto Clodomiro Picado (ICP

Monitoring of the endangered Pinna nobilis (Linneus 1758) in the Mar Grande of Taranto (Ionian Sea, Italy)

The present study aimed to improve the knowledge of the bivalve Pinna nobilis L. population distribution in Mar Grande of Taranto (Ionian Sea). Although historical references report the local abundant presence of this endangered species, there is a lack of updated information about its exact distribution. For this purpose, a visual census of P. nobilis was performed by SCUBA diving in the Mar Grande basin from September 2004 to March 2005. Pinnids were found at depths from 3 to 16 m, with a density ranging from 0.1 to 0.7 ind ha(-1). The survey method employed in this study was non-destructive, relatively simple to perform and easily applicable for monitoring studies. Field data were stored in a database and linked with the study area by means of the GIS technology. The results of the present study indicate a tentative of recovery of P. nobilis population in Mar Grande in spite of all the difficulties of a degraded and heavily polluted environment and the damages of illegal fishing methods

Botulinum toxin type a and physiotherapy in spasticity of the lower limbs due to amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease (unknown pathogenesis) of the central nervous system that causes death within 1–5 years. Clinically, flabby paralysis, areflexia, muscular atrophy, and muscle fasciculations, signs of II motor neuron damage, appear. Sometimes, clinical manifestations of damage of the I motor neuron come out in lower limbs; spastic paralysis, iperflexia, and clonus emerge, and they impair deambulation and management of activities of daily living, such as personal hygiene or dressing. Thus, the first therapeutic approach in these patients involves antispasmodic drugs orally followed by botulinum toxin type A injection (BTX-A). In this study, we study the efficacy of BTX-A and physiotherapy in lower limb spasticity due to ALS and no response to treatment with oral antispastic drugs. We evaluated 15 patients (10 male and five female), with a mean age of 48.06 ± 5.2 with spasticity of adductor magnus (AM), at baseline (T0, before BTX-A treatment) and in the following three follow-up visits (T1 30 days, T2 60 days, and T3 90 days after infiltration). We evaluated myometric measure of muscle tone, the Modified Ashworth Scale of AM, Barthel Index, Adductor Tone Rating Scale, and Hygiene Score. The study was conducted between November 2018 and April 2019. We treated AM with incobotulinum toxin type A (Xeomin®, Merz). Spasticity (myometric measurement, Adductor Tone Rating Scale, and Modified Ashworth Scale) and clinical (Barthel Index and Hygiene Score) improvements were obtained for 90 days after injection (p < 0.05). Our study shows the possibility of using BTX-A in the treatment of spasticity in patients with ALS and no response to oral antispastic drugs, with no side effects. The limitation of the study is the small number of patients and the limited time of observation; therefore, it is important to increase both the number of patients and the observation time in future studies