Quality of Life, Physical Disability, and Respiratory Impairment in Duchenne Muscular Dystrophy

Kohler, Malcolm; Clarenbach, Christian F.; Böni, Lukas; Brack, Thomas; Russi, Erich W.; Bloch, Konrad E.

doi:10.1164/rccm.200503-322oc

Cited by 169 publications

(174 citation statements)

References 22 publications

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“…Although further studies are needed in which a larger cohort of patients is confronted with the system and a systematic categorization of the system impact on the quality of life should take into account a range of outcomes (e.g. mood, motivation, caregiver burden; employability; satisfaction) [20][21] [22], the results obtained from this pilot study are encouraging for the establishment of a solid link between the field of human machine interaction and neurorehabilitation strategy [23].…”

Section: Discussionmentioning

confidence: 99%

Non-invasive brain–computer interface system: Towards its application as assistive technology

Cincotti

Mattia

Aloise

et al. 2008

Brain Research Bulletin

266

147

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The quality of life of people suffering from severe motor disabilities can benefit from the use of current assistive technology capable of ameliorating communication, house-environment management and mobility, according to the user's residual motor abilities. Brain Computer Interfaces (BCIs) are systems that can translate brain activity into signals that control external devices. Thus they can represent the only technology for severely paralyzed patients to increase or maintain their communication and control options.Here we report on a pilot study in which a system was implemented and validated to allow disabled persons to improve or recover their mobility (directly or by emulation) and communication within the surrounding environment. The system is based on a software controller that offers to the user a communication interface that is matched with the individual's residual motor abilities. Patients (n=14) with severe motor disabilities due to progressive neurodegenerative disorders were trained to use the system prototype under a rehabilitation program carried out in a house-like furnished space. All users utilized regular assistive control options (e.g., microswitches or head trackers). In addition, four subjects learned to operate the system by means of a non-invasive EEG-based BCI. This system was controlled by the subjects' voluntary modulations of EEG sensorimotor rhythms recorded on the scalp; this skill was learnt even though the subjects have not had control over their limbs for a long time.Correspondence should be addressed to: Febo Cincotti, PhD, Fondazione Santa Lucia, IRCCS, Via Ardeatina, 306, 00179, Rome, Italy, Phone: +39-06-51501466, Fax: +39-06-51501465, f.cincotti@hsantalucia.it. * These authors equally contributed to the paper Conflict of Interest Declaration: I had full access to all the data in the study and I take responsibility for the integrity of the data and the accuracy of the data analysis. I also state that all authors have read and approved submission of the manuscript; the manuscript contains original material that has not been published and has not being considered for publication elsewhere.Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. We conclude that such a prototype system, which integrates several different assistive technologies including a BCI system, can potentially facilitate the translation from pre-clinical demonstrations to a clinical useful BCI. NIH Public Access

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Section: Discussionmentioning

confidence: 99%

Non-invasive brain–computer interface system: Towards its application as assistive technology

Cincotti

Mattia

Aloise

et al. 2008

Brain Research Bulletin

266

147

View full text Add to dashboard Cite

show abstract

“…Some studies have shown that it is reduced among children with DMD 6,7 . Others have found no differences between the QOL of children with DMD and healthy children 4,8,9 . However, most authors agree that the QOL does not change as the child grows older and gets more dependent 7,9 , except Davis et al, who associated the reduction of the QOL of children and caregivers to the wheelchair dependence 10 .…”

mentioning

confidence: 94%

“…However, most authors agree that the QOL does not change as the child grows older and gets more dependent 7,9 , except Davis et al, who associated the reduction of the QOL of children and caregivers to the wheelchair dependence 10 . Opposite, the non-invasive ventilation dependence has not been related to the reduction of QOL in children 4 . The scores of QOL questionnaires in children are usually proportionally equivalent to the scores of their caregivers 8,11 .…”

mentioning

confidence: 94%

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Is functional dependence of Duchenne muscular dystrophy patients determinant of the quality of life and burden of their caregivers?

Moura

Wutzki

Voos

et al. 2015

Arq. Neuro-Psiquiatr.

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Objective:The relationship between functional dependence and quality of life (QOL) in Duchenne muscular dystrophy (DMD) patients and burden and QOL in caregivers is not clear. This study investigated possible relationships between functional dependence/QOL of DMD patients and QOL/burden of caregivers. Method: This study included 35 boys (6-17 years) and respective caregivers (above 21 years). Caregivers answered to World Health Organization Quality of Life and Zarit Burden Interview questionnaires. Patients were assessed with the Motor Function Measure and the Autoquestionnaire Qualité de vie Enfant Imagé. Spearman correlations and linear regressions were run to investigate relationships between the variables. Results: The occurrence of lower QOL and higher burden among the caregivers of patients with Duchenne muscular dystrophy was evidenced. The functional dependence of patients was not considered a determinant factor. Higher caregivers' burden was related to lower caregivers' QOL and to higher patients' ages.Keywords: Duchenne muscular dystrophy, behavior, quality of life, motor activity. RESUMOObjetivo: A relação entre qualidade de vida e função motora de pacientes com distrofia muscular de Duchenne (DMD) e sobrecarga e qualidade de vida (QV) dos cuidadores não está clara na literatura. Esse estudo investigou possíveis relações entre dependência funcional/ QV de pacientes e sobrecarga/QV dos cuidadores em 35 meninos (6-17 anos) com DMD e respectivos cuidadores (acima de 21 anos). Método: Cada cuidador respondeu ao questionário de QV da Organização Mundial de Saúde e de sobrecarga de Zarit e o paciente foi avaliado com a medida de função motora e o Autoquestionnaire Qualité de vie Enfant Imagé. Correlações de Spearman e regressões lineares investigaram possíveis relações entre as variáveis. Resultados: A ocorrência de menor QV e maior sobrecarga entre cuidadores foi evidenciada, enquanto a dependência funcional dos pacientes não foi considerada fator

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“…Nous nous sommes également intéressés à la fonction cardio-respiratoire puisque l'amélio-ration de ces paramètres représente une condition indispensable pour espérer un bénéfice thérapeutique chez les patients DMD. Chez ces patients en effet, les insuffisances respiratoire et cardiaque sont une des causes majeures du décès [8]. Les souris mdx ont un diaphragme particulièrement dystrophique, ce qui crée un dysfonctionnement respiratoire.…”

Section: Approche Thérapeutique Par Saut D'exonunclassified

Un nouvel outil pour le traitement de la myopathie de Duchenne : les tricyclo-ADN

et al. 2015

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Quality of Life, Physical Disability, and Respiratory Impairment in Duchenne Muscular Dystrophy

Abstract: Quality of life in DMD is not correlated with physical impairment nor the need for noninvasive positive-pressure ventilation. The surprisingly high quality of life experienced by these severely disabled patients should be taken into consideration when therapeutic decisions are made.

Cited by 169 publications

References 22 publications

Non-invasive brain–computer interface system: Towards its application as assistive technology

Non-invasive brain–computer interface system: Towards its application as assistive technology

Is functional dependence of Duchenne muscular dystrophy patients determinant of the quality of life and burden of their caregivers?

Un nouvel outil pour le traitement de la myopathie de Duchenne : les tricyclo-ADN

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