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muscular dystrophy rehabilitation

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https://www.readbyqxmd.com/read/28690390/integrated-care-of-muscular-dystrophies-in-italy-part-1-pharmacological-treatment-and-rehabilitative-interventions
#1
Luisa Politano, Marianna Scutifero, Melania Patalano, Alessandra Sagliocchi, Antonella Zaccaro, Federica Civati, Erika Brighina, Gianluca Vita, Sonia Messina, Maria Sframeli, Maria Elena Lombardo, Roberta Scalise, Giulia Colia, Maria Catteruccia, Angela Berardinelli, Maria Chiara Motta, Alessandra Gaiani, Claudio Semplicini, Luca Bello, Guja Astrea, Giulia Ricci, Maria Grazia D'Angelo, Giuseppe Vita, Marika Pane, Adele D'Amico, Umberto Balottin, Corrado Angelini, Roberta Battini, Lorenza Magliano
This paper describes the pharmacological therapies and rehabilitative interventions received by 502 patients with Muscular Dystrophies, evaluated in relation to patient's socio-demographic and clinical variables, and geographical areas. Data were collected by the MD-Socio-Demographic and Clinical Schedule (MD-SC-CS) and by the Family Problems Questionnaire (FPQ). The most part of the enrolled patients were in drug treatment. The number of the medications increased in relation to patient's age, disability degree and duration of illness and was higher among patients with Duchenne Muscular Dystrophy (DMD) compared with Becker (BMD) or Limb-Girdle Muscular Dystrophies (LGMD)...
March 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/28687062/evaluation-of-hand-orthoses-in-duchenne-muscular-dystrophy
#2
Johanna Weichbrodt, Britt-Marie Eriksson, Anna-Karin Kroksmark
PURPOSE: The purpose of this study was to evaluate whether treatment of boys with Duchenne muscular dystrophy using hand orthoses could benefit joint mobility, grip strength, or fine motor function. METHOD: Eight boys with Duchenne muscular dystrophy were provided with individually customised rest orthoses. The results were analysed using single-subject design. The study included a baseline and an intervention phase. A follow-up examination was also performed. RESULTS: Boys with less than 50° passive wrist extension mobility were included...
July 7, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28615996/international-society-of-sports-nutrition-position-stand-safety-and-efficacy-of-creatine-supplementation-in-exercise-sport-and-medicine
#3
REVIEW
Richard B Kreider, Douglas S Kalman, Jose Antonio, Tim N Ziegenfuss, Robert Wildman, Rick Collins, Darren G Candow, Susan M Kleiner, Anthony L Almada, Hector L Lopez
Creatine is one of the most popular nutritional ergogenic aids for athletes. Studies have consistently shown that creatine supplementation increases intramuscular creatine concentrations which may help explain the observed improvements in high intensity exercise performance leading to greater training adaptations. In addition to athletic and exercise improvement, research has shown that creatine supplementation may enhance post-exercise recovery, injury prevention, thermoregulation, rehabilitation, and concussion and/or spinal cord neuroprotection...
2017: Journal of the International Society of Sports Nutrition
https://www.readbyqxmd.com/read/28542314/toward-a-more-personalized-motor-function-rehabilitation-in-myotonic-dystrophy-type-1-the-role-of-neuroplasticity
#4
Simona Portaro, Antonino Naro, Antonino Chillura, Luana Billeri, Alessia Bramanti, Placido Bramanti, Carmelo Rodolico, Rocco Salvatore Calabrò
Myotonic dystrophy type 1 (DM1) is the most prevalent adult muscular dystrophy, often accompanied by impairments in attention, memory, visuospatial and executive functions. Given that DM1 is a multi-system disorder, it requires a multi-disciplinary approach, including effective rehabilitation programs, focusing on the central nervous system neuroplasticity, in order to develop patient-tailored rehabilitative procedures for motor function recovery. Herein, we performed a transcranial magnetic stimulation (TMS) study aimed at investigating central motor conduction time, sensory-motor plasticity, and cortical excitability in 7 genetically defined DM1 patients...
2017: PloS One
https://www.readbyqxmd.com/read/28503465/correlation-of-serum-creatine-kinase-level-with-pulmonary-function-in-duchenne-muscular-dystrophy
#5
Eun Young Kim, Jang Woo Lee, Mi Ri Suh, Won Ah Choi, Seong Woong Kang, Hyeon Jun Oh
OBJECTIVE: To investigate the relationship between serum creatine kinase (CK) level and pulmonary function in Duchenne muscular dystrophy (DMD). METHODS: A total of 202 patients with DMD admitted to the Department of Rehabilitation Medicine, Gangnam Severance Hospital were enrolled from January 1, 1999 to March 31, 2015. Seventeen patients were excluded. Data collected from the 185 patients included age, height, weight, body mass index, pulmonary function tests including forced vital capacity (FVC), peak cough flow, maximal expiratory pressure (MEP), and maximal inspiratory pressure (MIP), and laboratory measurements (serum level of CK, CK-MB, troponin-T, and B-type natriuretic peptide)...
April 2017: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/28470591/high-intensity-interval-training-in-facioscapulohumeral-muscular-dystrophy-type-1-a-randomized-clinical-trial
#6
Grete Andersen, Karen Heje, Astrid Emile Buch, John Vissing
Increasing evidence suggests that high-intensity training (HIT) is a time-efficient exercise strategy to improve fitness. HIT has never been explored in neuromuscular diseases, likely because it may seem counterintuitive. A single session of high-intensity exercise has been studied without signs of muscle damage in facioscapulohumeral muscular dystrophy type 1 (FSHD1). We aimed to determine whether HIT is safe and effective in FSHD1 in a randomized, controlled parallel study. Untrained adults with genetically verified FSHD1 (n = 13) able to perform cycle-ergometer exercise were randomized to 8 weeks of supervised HIT (n = 6) (3 × 10-min cycle-ergometer-HIT/week) or 8 weeks of usual care (n = 7)...
June 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28395534/application-of-the-international-classification-of-functioning-disability-and-health-system-to-symptoms-of-the-duchenne-and-becker-muscular-dystrophies
#7
Kristin M Conway, Emma Ciafaloni, Dennis Matthews, Chris Westfield, Kathy James, Pangaja Paramsothy, Paul A Romitti
PURPOSE: Duchenne and Becker muscular dystrophies, collectively referred to as dystrophinopathies, are X-linked recessive diseases that affect dystrophin production resulting in compromised muscle function across multiple systems. The International Classification of Functioning, Disability and Health provides a systematic classification scheme from which body functions affected by a dystrophinopathy can be identified and used to examine functional health. MATERIALS AND METHODS: The infrastructure of the Muscular Dystrophy Surveillance, Tracking, and Research Network was used to identify commonly affected body functions and link selected functions to clinical surveillance data collected through medical record abstraction...
April 11, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28338349/clinical-features-of-children-and-adults-with-a-muscular-dystrophy-using-powered-indoor-outdoor-wheelchairs-disease-features-comorbidities-and-complications-of-disability
#8
Andrew Oliver Frank, Lorraine H De Souza
PURPOSE: To describe the clinical features of electric powered indoor/outdoor wheelchair users with a muscular dystrophy, likely to influence optimal prescription; reflecting features of muscular dystrophies, conditions secondary to disability, and comorbidities impacting on equipment provision. METHODS: Cross-sectional retrospective case note review of recipients of electric powered indoor/outdoor wheelchairs provided by a specialist regional wheelchair service...
February 25, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28272972/a-feasibility-study-using-solution-focused-coaching-for-health-promotion-in-children-and-young-people-with-duchenne-muscular-dystrophy
#9
Amy C McPherson, Laura McAdam, Sarah Keenan, Heidi Schwellnus, Elaine Biddiss, Andrea DeFinney, Kirsten English
PURPOSE: To evaluate the feasibility and acceptability of a coaching intervention (Solution-Focused Coaching in pediatric rehabilitation [SFC-Peds]) related to physical activity and diet in males with Duchenne muscular dystrophy. METHODS: A pre-post design was employed. Participants had five coaching sessions over 8 weeks. The first session was face-to-face, followed by four virtual sessions. Feasibility criteria included recruitment rates, attrition, and intervention fidelity...
March 8, 2017: Developmental Neurorehabilitation
https://www.readbyqxmd.com/read/28040606/steinert-syndrome-and-repercussions-in-dental-medicine
#10
REVIEW
Helena Baptista, Inês Lopes Cardoso
Steinert syndrome, also called myotonic dystrophy type 1, is a genetic disorder with autosomal dominant transmission characterized by myotonia and a multisystemic clinical picture that affects several tissues of the human body. The most common systemic phenotypes are: muscular, cardiac, respiratory, CNS, ocular, gynecological, digestive, orthopedical, as well as cognitive and psychological symptoms (cognitive decline). Muscles involved in voluntary movement are highly affected by myotonia especially distal muscles of upper limbs...
March 2017: Archives of Oral Biology
https://www.readbyqxmd.com/read/27866827/nucleoprotein-supplementation-enhances-the-recovery-of-rat-soleus-mass-with-reloading-after-hindlimb-unloading-induced-atrophy-via-myonuclei-accretion-and-increased-protein-synthesis
#11
Ryosuke Nakanishi, Yusuke Hirayama, Minoru Tanaka, Noriaki Maeshige, Hiroyo Kondo, Akihiko Ishihara, Roland R Roy, Hidemi Fujino
Hindlimb unloading results in muscle atrophy and a period of reloading has been shown to partially recover the lost muscle mass. Two of the mechanisms involved in this recovery of muscle mass are the activation of protein synthesis pathways and an increase in myonuclei number. The additional myonuclei are provided by satellite cells that are activated by the mechanical stress associated with the reloading of the muscles and eventually incorporated into the muscle fibers. Amino acid supplementation with exercise also can increase skeletal muscle mass through enhancement of protein synthesis and nucleotide supplements can promote cell cycle activity...
December 2016: Nutrition Research
https://www.readbyqxmd.com/read/27854227/do-psychosocial-interventions-improve-quality-of-life-and-wellbeing-in-adults-with-neuromuscular-disorders-a-systematic-review-and-narrative-synthesis
#12
Elaine Walklet, Kate Muse, Jane Meyrick, Tim Moss
Quality of life and well-being are frequently restricted in adults with neuromuscular disorders. As such, identification of appropriate interventions is imperative. The objective of this paper was to systematically review and critically appraise quantitative studies (RCTs, controlled trials and cohort studies) of psychosocial interventions designed to improve quality of life and well-being in adults with neuromuscular disorders. A systematic review of the published and unpublished literature was conducted. Studies meeting inclusion criteria were appraised using a validated quality assessment tool and results presented in a narrative synthesis...
August 30, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27762634/fatigue-in-facioscapulohumeral-muscular-dystrophy-a-qualitative-study-of-people-s-experiences
#13
Karen Schipper, Minne Bakker, Tineke Abma
PURPOSE: The aim of this article is to describe how fatigue affects the lives of people with facioscapulohumeral dystrophy (FSHD), how they experience fatigue, and how they deal with it in order to attune rehabilitation care to patients' needs. METHOD: A qualitative study, consisting of 25 semistructured interviews with patients with FSHD and severe fatigue (as measured with the checklist individual strength (CIS) fatigue questionnaire), was conducted to gain insight into the experiences of patients with fatigue...
October 20, 2016: Disability and Rehabilitation
https://www.readbyqxmd.com/read/27718629/retracted-article-early-use-of-power-wheelchair-for-children-with-duchenne-muscular-dystrophy-a-narrative-review
#14
Michal Elboim-Gabyzon, Osnat Atun-Einy, Insaf Khoury-Assaf
No abstract text is available yet for this article.
October 9, 2016: Disability and Rehabilitation
https://www.readbyqxmd.com/read/27677016/muscle-activations-during-gait-in-children-with-duchenne-muscular-dystrophy
#15
Juliette Ropars, Mathieu Lempereur, Sylvain Brochard, Carole Vuillerot, Vincent Tiffreau, Jean-Marie Cuisset, Yann Péréon, Fabien Leboeuf, Raphaël Gross, Ludovic Delporte, Yannick Delpierre
OBJECTIVE: The aim of this prospective study was to investigate changes in muscle activity during gait in children with Duchenne muscular Dystrophy (DMD). MATERIALS/PATIENTS AND METHODS: Dynamic surface electromyography recordings (EMGs) of 16 children with DMD and pathological gait were compared with those of 15 control children. The activity of the rectus femoris (RF), vastus lateralis (VL), medial hamstrings (HS), tibialis anterior (TA) and gastrocnemius soleus (GAS) muscles was recorded and analyzed quantitatively and qualitatively...
September 2016: Annals of Physical and Rehabilitation Medicine
https://www.readbyqxmd.com/read/27677014/2005-2015-ten-years-clinical-experience-in-treating-dmd-patients-by-corticosteroids-in-lyon
#16
Stéphanie Fontaine Carbonnel, Pascal Rippert, Isabelle Poirot, Dominique Gachet, Capucine de Lattre, Carole Vuillerot
OBJECTIVE: Since 2005, in France, corticosteroid therapy is now widely used in Duchenne muscular dystrophy (DMD). This treatment has changed our practice of pediatric rehabilitation teams. We describe here our 10-year clinical experience in treating DMD patients by CS according to international guidelines i.e. prednisone 0.75mg/kg/day started from the plateau of motor function. MATERIALS/PATIENTS AND METHODS: We conducted a prospective study. Information was given on the expected effects and side effects...
September 2016: Annals of Physical and Rehabilitation Medicine
https://www.readbyqxmd.com/read/27622734/muscle-activation-during-gait-in-children-with-duchenne-muscular-dystrophy
#17
Juliette Ropars, Mathieu Lempereur, Carole Vuillerot, Vincent Tiffreau, Sylviane Peudenier, Jean-Marie Cuisset, Yann Pereon, Fabien Leboeuf, Ludovic Delporte, Yannick Delpierre, Raphaël Gross, Sylvain Brochard
The aim of this prospective study was to investigate changes in muscle activity during gait in children with Duchenne muscular Dystrophy (DMD). Dynamic surface electromyography recordings (EMGs) of 16 children with DMD and pathological gait were compared with those of 15 control children. The activity of the rectus femoris (RF), vastus lateralis (VL), medial hamstrings (HS), tibialis anterior (TA) and gastrocnemius soleus (GAS) muscles was recorded and analysed quantitatively and qualitatively. The overall muscle activity in the children with DMD was significantly different from that of the control group...
2016: PloS One
https://www.readbyqxmd.com/read/27478558/extensive-functional-evaluations-to-monitor-aerobic-training-in-becker-muscular-dystrophy-a-case-report
#18
Caterina Tramonti, Bruno Rossi, Carmelo Chisari
Low-intensity aerobic training seems to have positive effects on muscle strength, endurance and fatigue in Becker Muscular Dystrophy (BMD) patients. We describe the case of a 33-year old BMD man, who performed a four-week aerobic training. Extensive functional evaluations were executed to monitor the efficacy of the rehabilitative treatment. Results evidenced an increased force exertion and an improvement in muscle contraction during sustained exercise. An improvement of walk velocity, together with agility, endurance capacity and oxygen consumption during exercise was observed...
June 13, 2016: European Journal of Translational Myology
https://www.readbyqxmd.com/read/27438864/vocational-rehabilitation-supporting-ill-or-disabled-individuals-in-to-work-a-uk-perspective
#19
REVIEW
Andrew Frank
Work is important for one's self-esteem, social standing and ability to participate in the community as well as for the material advantages it brings to individuals and their families. The evidence suggests that the benefits of employment outweigh the risks of work and are greater than the risks of long-term unemployment or sickness absence. Individuals may be born with physical or intellectual disadvantages (e.g., cerebral palsy), or they may be acquired during childhood or adult life. Some progressive conditions may present in childhood or adolescence (e...
July 16, 2016: Healthcare (Basel, Switzerland)
https://www.readbyqxmd.com/read/27427990/respiratory-failure-because-of-neuromuscular-disease
#20
Robin S Howard
PURPOSE OF REVIEW: Understanding the mechanisms and abnormalities of respiratory function in neuromuscular disease is critical to supporting the patient and maintaining ventilation in the face of acute or chronic progressive impairment. RECENT FINDINGS: Retrospective clinical studies reviewing the care of patients with Guillain-Barré syndrome and myasthenia have shown a disturbingly high mortality following step-down from intensive care. This implies high dependency and rehabilitation management is failing despite evidence that delayed improvement can occur with long-term care...
October 2016: Current Opinion in Neurology
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