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  • 1 Department of Pediatric Rehabilitation, Szent János Hospital, Budapest, , Hungary
  • | 2 Department of Morphology and Physiology, Semmelweis University, Budapest, , Hungary
  • | 3 Department of Biomechanics, University of Physical Education, Budapest, , Hungary
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Clinical Aspects of Rehabilitation

Neuromuscular dystrophy diseases (NMDs) exhibit high degree of heterogeneity, which is generally characterized by decreased muscle strength and muscle fiber integrity. Muscle fibers of different sizes were detected in the dystrophic muscle, as well as the presence of connective tissue elements and fat tissue infiltration could be observed. Twenty-nine different genes have been identified in muscular dystrophy diseases [1]. The flaws of these and unidentified genes cause 34 different genetic disorders that differ both in the severity and onset of the symptoms and in the extent of inheritance. One of the most devastating genetic NMDs is the Duchenne muscular dystrophy (DMD), which is a multilevel/multisystem X-linked disease, that affects 1 in 3600–6000 live male births. The pathological characteristics of DMD include the lack of dystrophin or its isoforms and the subsequent secondary effects on brain development and functioning including social and emotional behaviours as well [2]. The chronic treatment factors (such as glucocorticoid treatment) can also play a part in psychosocial health. Improving the neuromuscular condition of the diseased children requires specific needs that constantly change, sometimes rapidly as the disease progresses.

The basic clinical institution presented the review here is the Neuromusculoskeletal Rehabilitation Center in Budapest at the Szent János Hospital, which also hosts the Neuromuscular Expertise Center for Rare Diseases in Hungary. This institution can be professionally considered as a physical medicine and rehabilitation department for children dealing with neurohabilitation and neurorehabilitation in childhood. It works as a multidisciplinary rehabilitation team as presented in Figure 1. Further preferable professionals may be also included, such as occupational and speech therapists, and special education teachers.

Figure 1.
Figure 1.

The multidisciplinary rehabilitation team consists of the listed professionals supported by social and hospitalization care and the availability of proper medical devices. The “coordinating neurorehabilitator” physician (at the center of the figure) is a specialist for physical medicine, rehabilitation, and neurology

Citation: Developments in Health Sciences 1, 1; 10.1556/2066.1.2018.04

This hospital unit is a patient- and family-centered complex, applying physical, cognitive, and psychosocial elements of therapeutic interventions, i.e., early, preventive neurorehabilitation with movement, sensory, precognitive, and cognitive trainings, furthermore, communication- and learning-centered trainings. From the points of social health care, the profile of the institution is to ensure: (a) from early childhood, the practice of preventive neurorehabilitation for a better quality of life, (b) the prevention of learning disorders, (c) the support in psychosocial integration, and (d) the orientation to a family-centered therapy.

Clinical assessment in DMD is based on standard physical examinations, with the focus on the impairments of the musculoskeletal system and related functional consequences. The regular assessment of the clinical condition is based on measuring muscular strength and range of motion, evaluating posture gait, timed testing of movements, monitoring the ability to scope with activities of daily living, and application of motor function scales. These regular assessments are used to conclude decisions about therapeutic interventions. In the clinical practice, the available objective monitoring methods for rehabilitation needs further development and additional methodological procedures also based on instrumental and technical development. At present, subjective functional scales are dominating functional assessments.

Experimental Research Line

Based on priority selection, DMD is in the frontline of this presentation especially because of our present aim that is to survey complex biomechanical research methods helping early preventive rehabilitation of this disease. These research methods were developed earlier by our team [3].

To evaluate the functional status of patients with progressive muscular atrophy, first of all in the case of DMD, we need non-invasive instrumental assays, such as stabilometry for posture analysis and to measure coordination abilities, muscle strength assessment, detailed analysis of walking, neuromuscular reaction time, etc., providing objective data for the monitoring of the complex rehabilitation process [4].

Methods

In our experimental studies using complex biomechanical research methods, the determination of equilibrium and upper-body coordination parameters through registration of pressure-centered trajectories was aimed to carry out for Duchenne (DMD) and Becker muscular dystrophic (BMD) wheelchair-using children. In the presence of parents, 10 muscular dystrophic boys participated in the measurements. Two control groups were created, which were collected from healthy schoolboys of the same age. One control group was examined while sitting in wheelchair and the other without the wheelchair. The average age of examined healthy children was 10.90 ± 3.93 years, whereas the average age of wheelchair-using sick children was 13.67 ± 4.62 years.

The stabilometric parameters were measured in one of the control groups in the standing position, while in case of the DMD and BMD groups and the control group sitting in the wheelchair over a force platform with 1000 × 1000 mm measuring surface as shown in Figure 2. In addition, a three-channel amplifier, a measuring system containing ADDON microcomputer and laptops, was applied. The Feed 103C software operated the machine (Ing. Büro, Bretz). “Psycho 8” type reaction time measurement system and Dyna 10 universal dynamometer were used to complete the coordination tests.

Figure 2.
Figure 2.

(a) Technical drawing of a wheelchair with standard sizes; (b) stabilometric analysis with a DMD child sitting in a wheelchair positioned on a force platform with 1000 × 1000 mm measuring surface

Citation: Developments in Health Sciences 1, 1; 10.1556/2066.1.2018.04

Ethics

All procedures were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards (IKEB 555-A/2014).

Results and Conclusions

The applied biomechanical functional tests provided informative data on [1] habilitation and rehabilitation of the musculoskeletal condition of DMD children for seat positioning and body posture, on coordinating body posture while sitting in wheelchair, on prevention of asymmetric body posture, preservation of hand function such as choice reaction time, including preservation of combined regulation of neuromuscular and cognitive functions [2]. These novel lines of research are providing important data for rehabilitation in the advanced stage of DMD [3]. The objective data contribute to the selection of more efficient, custom designed orthoses, positioners, and appropriate mobility devices [4]. In addition, gradually achievable postural support may be further emphasized especially for the future studies in helping to reduce the impact of gravity and discourage developing spine statics disturbances leading to asymmetric posture, kyphosis, and scoliosis.

References

  • 1.

    Dalkilic I , Kunkel LM . Muscular dystrophies: genes to pathogenesis. Curr Opin Genet Dev. 2003;13(3):2318.

  • 2.

    Bushby K , Finkel R , Birnkrant DJ , et al. Diagnosis and management of Duchenne muscular dystrophy, Part 2: implementation of multidisciplinary care. Lancet Neurol. 2010;9(2):17789.

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  • 3.

    Bretz É , Kóbor-Nyakas , Bretz KJ , Hrehuss N , Radák Zs , Nyakas CS . Correlations of psycho-physiological parameters influencing the physical fitness of aged women. Acta Physiol Hung. 2014;101(4):4718.

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  • 4.

    Medveczky E , Heintz B , Bretz KJ , Nyakas CS , Bretz KJ . Izomerő, koordináció, kardiorespiratorikus paraméterek és a választásos reakcióidő méréstechnikája gyermekkori neurológiai kórképekben [Measurement technology of muscle force, coordination, cardio-respiratory parameters and choice reaction time in childhood neurological disorders]. Rehabilitáció [Rehabilitation]. 2016;26(1):20710.

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    • Export Citation
  • 1.

    Dalkilic I , Kunkel LM . Muscular dystrophies: genes to pathogenesis. Curr Opin Genet Dev. 2003;13(3):2318.

  • 2.

    Bushby K , Finkel R , Birnkrant DJ , et al. Diagnosis and management of Duchenne muscular dystrophy, Part 2: implementation of multidisciplinary care. Lancet Neurol. 2010;9(2):17789.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 3.

    Bretz É , Kóbor-Nyakas , Bretz KJ , Hrehuss N , Radák Zs , Nyakas CS . Correlations of psycho-physiological parameters influencing the physical fitness of aged women. Acta Physiol Hung. 2014;101(4):4718.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 4.

    Medveczky E , Heintz B , Bretz KJ , Nyakas CS , Bretz KJ . Izomerő, koordináció, kardiorespiratorikus paraméterek és a választásos reakcióidő méréstechnikája gyermekkori neurológiai kórképekben [Measurement technology of muscle force, coordination, cardio-respiratory parameters and choice reaction time in childhood neurological disorders]. Rehabilitáció [Rehabilitation]. 2016;26(1):20710.

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    • Export Citation

 

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Senior Editors

Editor-in-Chief: Zoltán Zsolt NAGY
Vice Editors-in-Chief: Gabriella Bednárikné DÖRNYEI, Ákos KOLLER
Managing Editor: Johanna TAKÁCS

Editorial Board

  • Zoltán BALOGH (Department of Nursing, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Klára GADÓ (Department of Clinical Studies, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • István VINGENDER (Department of Social Sciences, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Attila DOROS (Department of Imaging and Medical Instrumentation, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Judit Helga FEITH (Department of Social Sciences, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Mónika HORVÁTH (Department of Physiotherapy, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Illés KOVÁCS (Department of Clinical Ophthalmology, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Ildikó NAGYNÉ BAJI (Department of Applied Psychology, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Tamás PÁNDICS (Department for Epidemiology, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • József RÁCZ (Department of Addictology, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Attila Lajos RÉTHY (Department of Family Care Methodology, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • János RIGÓ (Department of Clinical Studies in Obstetrics and Gynaecology, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Andrea SZÉKELY (Department of Oxyology and Emergency Care, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Márta VERESNÉ BÁLINT (Department of Dietetics and Nutritional Sicences, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Gyula DOMJÁN (Department of Clinical Studies, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Péter KRAJCSI (Department of Morphology and Physiology, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • György LÉVAY (Department of Morphology and Physiology, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Csaba NYAKAS (Department of Morphology and Physiology, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Vera POLGÁR (Department of Morphology and Physiology, InFaculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • László SZABÓ (Department of Family Care Methodology, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Katalin TÁTRAI-NÉMETH (Department of Dietetics and Nutrition Sciences, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Katalin KOVÁCS ZÖLDI (Department of Social Sciences, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • Gizella ÁNCSÁN (Library, Faculty of Health Sciences, Semmelweis University, Budapest, Hungary)
  • András FALUS (Department of Genetics, Cell- and Immunbiology, Faculty of Medicine, Semmelweis University, Budapest, Hungary)
  • Romána ZELKÓ (Faculty of Pharmacy, Semmelweis University, Budapest, Hungary)
  • Mária BARNAI (Faculty of Health Sciences and Social Studies, University of Szeged, Szeged, Hungary)
  • László Péter KANIZSAI (Department of Emergency Medicine, Medical School, University of Pécs, Pécs, Hungary)
  • Bettina FŰZNÉ PIKÓ (Department of Behavioral Sciences, Faculty of Medicine, University of Szeged, Szeged, Hungary)
  • Imre SEMSEI (Faculty of Health, University of Debrecen, Debrecen, Hungary)
  • Teija-Kaisa AHOLAAKKO (Laurea Universities of Applied Sciences, Vantaa, Finland)
  • Ornella CORAZZA (University of Hertfordshire, Hatfield, Hertfordshire, United Kingdom)
  • Oliver FINDL (Department of Ophthalmology, Hanusch Hospital, Vienna, Austria)
  • Tamás HACKI (University Hospital Regensburg, Phoniatrics and Pediatric Audiology, Regensburg, Germany)
  • Xu JIANGUANG (Shanghai University of Traditional Chinese Medicine, Shanghai, China)
  • Paul GM LUITEN (Department of Molecular Neurobiology, University of Groningen, Groningen, Netherlands)
  • Marie O'TOOLE (Rutgers School of Nursing, Camden, United States)
  • Evridiki PAPASTAVROU (School of Health Sciences, Cyprus University of Technology, Lemesos, Cyprus)
  • Pedro PARREIRA (The Nursing School of Coimbra, Coimbra, Portugal)
  • Jennifer LEWIS SMITH (Collage of Health and Social Care, University of Derby, Cohehre President, United Kingdom)
  • Yao SUYUAN (Heilongjiang University of Traditional Chinese Medicine, Heilongjiang, China)
  • Valérie TÓTHOVÁ (Faculty of Health and Social Sciences, University of South Bohemia, České Budějovice, Czech Republic)
  • Tibor VALYI-NAGY (Department of Pathology, University of Illonois of Chicago, Chicago, IL, United States)
  • Chen ZHEN (Central European TCM Association, European Chamber of Commerce for Traditional Chinese Medicine)

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Developments in Health Sciences
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Developments in Health Sciences
Language English
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ISSN 2630-9378 (Print)
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