跳至主要内容

Decreased Gait and Function in Duchenne Muscular Dystrophy

Read full paper at:
http://www.scirp.org/journal/PaperInformation.aspx?PaperID=48984#.VJd5lcCAM4

Duchenne muscular dystrophy (DMD) is a genetic disorder linked to chromosome Xp21, due to absence of dystrophin production. It is clinically characterized by progressive muscle weakness, fatigue, and development of joint contractures that compromise general motor functionality, mainly the gait. Objective: To characterize the motor function and decrease gait in children with DMD using the Portuguese version of the Motor Function Measure scale (MFM-P). Methods: A review of medical records including chronological age and scores from MFM-P of children with a DMD who attended at the Neuromuscular Diseases Clinic at Campinas State University (UNICAMP), Brazil was performed in this study. A total of 36 medical records of male patients with confirmed clinical diagnosis of DMD, ambulatory or not, regardless of age; excluding those with other associated diseases or other types of muscular dystrophies were selected. Data were analyzed using Kolmogorov-Smirnov and Spearman correlation statistical tests. Results: Analysis of all data collected showed that 75% of our sample had D1 scores lower than 41.02%. There was a linear relationship between the scores of D2 and D3, but no association between D2 and D1 scores was noted. D1 score was between 40% and 80% in those patients presenting D2 scores between 80% and 100%. In all cases patients with low total score presented a greater risk for loss of gait and their functionality. Conclusion: The standing posture and the postural transfers were the worst activities observed in children with DMD, with positive correlation between proximal and distal motor function. Even with high scores according MFM-P in proximal function, the children showed strong predictors for loss of gait.
Cite this paper
Cristina Iwabe-Marchese, C. , Fávaro, A. and Luiz, L. (2014) Decreased Gait and Function in Duchenne Muscular Dystrophy. Open Journal of Therapy and Rehabilitation, 2, 120-125. doi: 10.4236/ojtr.2014.23017.
 

[1] Fernandes, L.A.Y., Caromano, F.A., Hukuda, M.E., Escorcio, R. and Carvalho, E.V. (2010) Development and Reliability of the Functional Scale Going up and down Stairs for Muscular Dystrophy. Revista Brasileira de Fisioterapia, 14, 518-526. http://dx.doi.org/10.1590/S1413-35552010000600011
[2] Araújo, A.P.Q.C., Deco, M.C., Kloh, B.S., Costa, M.R., Gois, F.V. and Guimarães, F.H.H. (2004) Delay in Diagnosis of Duchenne Muscular Dystrophy. Revista Saúde Materno Infantil, 4, 179-183.
[3] Gomes, A.L.O., Pinto, A.N., Goes, E.R., Hirosue, L.N., Peixoto, B.O., Caromano, F.A. and Blascovi Assis, S.M. (2011) Motor and Functional Performance in Duchenne Muscular Dystrophy: A Case Report. Journal of the Health Sciences Institute, 29, 131-135.
[4] Escorcio, R., Caromano, F.A., Hukuda, M.E. and Fernandes, L.A.Y. (2011) Development of an Evaluation Scale for Sitting and Standing from the Ground for Children with Duchenne Muscular Dystrophy. Journal of Motor Behavior, 43, 37-41.
[5] D’Angelo, M.G., Berti, M., Piccinini, L., Romei, M., Guglieri, M., Bonato, S., Degrate, A., Turconi, A.C. and Bresolin, N. (2009) Gait Pattern in Duchenne Muscular Dystrophy. Gait & Posture, 29, 36-41.
http://dx.doi.org/10.1016/j.gaitpost.2008.06.002
[6] Melanda, A.G., Pauleto, A.C., Knaut, L.A., Ferreira, A.E.K., Iucksch, D.D., Silva, R.M. and Cunha, R.F.M. (2011) Evaluation of Patients with Duchenne Muscular Dystrophy Laboratory Computerized Index March through the Amendment of the March. Revista Brasileira de Ortopedia, 46, 10-13.
[7] Beenakker, E.A.C., Maurits, N.M., Fock, J.M., Brouwer, O.F. and Hoeven, J.H.V.D. (2005) Functional Ability and Muscle Force in Healthy Children and Ambulant Duchenne Muscular Dystrophy Patients. European Journal of Paediatric Neurology, 9, 387-393. http://dx.doi.org/10.1016/j.ejpn.2005.06.004
[8] Bérard, C., Payan, C. and Hodgkinson, I.J. (2005) The Fermanian Motor Function Measure Scale for Neuromuscular Diseases. Construction and Validation Study. Neuromuscular Disorders, 15, 463-470.
http://dx.doi.org/10.1016/j.nmd.2005.03.004
[9] Iwabe, C., Miranda-Pfeilsticker, B.H. and Nucci, A. (2008) Motor Function Measure Scale: English Version and Reliability Analysis. Revista Brasileira de Fisioterapia, 12, 417-424.
http://dx.doi.org/10.1590/S1413-35552008000500012
[10] Jeannet, P.Y., Aminian, K., Bloetzer, C., Najafi, B. and Paraschiv-Ionescu, A. (2011) Continuous Monitoring and Quantification of Multiple Parameters of Daily Physical Activity in Ambulatory Duchenne Muscular Dystrophy Patients. European Journal of Paediatric Neurology, 15, 40-47.
[11] Bushby, K., Finkel, R., Birnkrant, D.J., Case, L.E., Clemens, P.R., Cripe, L., et al. (2010) Diagnosis and Management of Duchenne Muscular Dystrophy, Part 1: Diagnosis, and Pharmacological and Psychosocial Management. Lancet Neurology, 9, 77-93. http://dx.doi.org/10.1016/S1474-4422(09)70271-6
[12] Conover, J.W. (1971) Practical Nonparametric Statistics. John Wiley & Sons, New York, 295-301,309-314.
[13] Vuillerot, C., Girardot, F., Payan, C., Iwaz, J., De Lattre, C. and Berard, C. (2009) Monitoring Changes and Predicting Loss of Ambulation in Duchenne Muscular Dystrophy with the Motor Function Measure. Developmental Medicine & Child Neurology, 52, 60-65.
http://dx.doi.org/10.1111/j.1469-8749.2009.03316.x
[14] Chaustre, D.M. and Willington, C.S. (2011) Duchenne Muscular Dystrophy. Prospects of Rehabilitation. Review Article. Rev. Fac Med, 19, 37-44.
[15] Ganea, R., Jeannet, P.Y., Ionescu, A.P., Goemans, N.M., Piot, C., Den Hauwe, M.V. and Aminian, K. (2012) Gait Assessment in Children with Duchenne Muscular Dystrophy during Long-Distance Walking. Journal of Child Neurology, 27, 30-38. http://dx.doi.org/10.1177/0883073811413581
[16] Fischmann, A., Hafner, P., Gloor, M., Schmid, M., Klein, A., Pohlman, U., et al. (2013) Quantitative MRI and Loss of Free Ambulation in Duchenne Muscular Dystrophy. Journal of Neurology, 260, 969-974.
http://dx.doi.org/10.1007/s00415-012-6733-x
[17] Da Silva, E.C., Machado, D.L., Resende, M.B.D., Silva, R.F., Zanoteli, E. and Reed, U.C. (2012) Scale Measure of Motor Function, Steroid Therapy, and Patients with Duchenne Muscular Dystrophy. Arquivos de Neuro-Psiquiatria, 70, 191-195.
[18] Santos, N.M., Rao, M.M., Terni, A., Hayashi, M.C.B., Fávero, F.M., Tables, A.A.J., et al. (2006) Clinical and Functional Profile of Patients with Duchenne Muscular Dystrophy Assisted in Brazilian Muscular Dystrophy Association (ABDIM). Reviews in the Neurosciences, 14, 15-22.
[19] McDonald, C.M., Henricson, E.K., Han, J.J., Abresch, R.T., Nicorici, A., Elfring, G.L., Atkinson, L., Reha, A., Hirawat, S. and Miller, L.L. (2009) The 6-Minute Walk Test as a New Outcome Measure in Duchenne Muscular Dystrophy. PTC Therapeutics, 100 Corporate Court, South Plainfield, New Jersey. www.interscience.wiley.com
[20] Bakker, J.P.J., de Groot, I.J.M., Beelen, A. and Lankhorst, G.J. (2002) Predictive Factors of Cessation of Ambulation in Patients with Duchenne Muscular Dystrophy. American Journal of Physical Medicine & Rehabilitation, 81, 906-912. http://dx.doi.org/10.1097/00002060-200212000-00004
[21] Zanoteli, E. (2014) Predicting the Loss of Ambulation in Duchenne Muscular Dystrophy. Arquivos de Neuro-Psiquiatria, 72, 1-2. http://dx.doi.org/10.1590/0004-282X20130243
[22] Martini, J., Voos, M.C., Hukuda, M.E., Resende, M.B.D. and Caromano, F.A. (2014) Compensatory Movements during Functional Activities in Ambulatory Children with Duchenne Muscular Dystrophy. Arquivos de Neuro-Psiquiatria, 72, 5-11. http://dx.doi.org/10.1590/0004-282X20130196         eww141222lx

评论

此博客中的热门博文

A Comparison of Methods Used to Determine the Oleic/Linoleic Acid Ratio in Cultivated Peanut (Arachis hypogaea L.)

Cultivated peanut ( Arachis hypogaea L.) is an important oil and food crop. It is also a cheap source of protein, a good source of essential vitamins and minerals, and a component of many food products. The fatty acid composition of peanuts has become increasingly important with the realization that oleic acid content significantly affects the development of rancidity. And oil content of peanuts significantly affects flavor and shelf-life. Early generation screening of breeding lines for high oleic acid content greatly increases the efficiency of developing new peanut varieties. The objective of this study was to compare the accuracy of methods used to classify individual peanut seed as high oleic or not high oleic. Three hundred and seventy-four (374) seeds, spanning twenty-three (23) genotypes varying in oil composition (i.e. high oleic (H) or normal/not high oleic (NH) inclusive of all four peanut market-types (runner, Spanish, Valencia and Virginia), were individually tested ...

Location Optimization of a Coal Power Plant to Balance Costs against Plant’s Emission Exposure

Fuel and its delivery cost comprise the biggest expense in coal power plant operations. Delivery of electricity from generation to consumers requires investment in power lines and transmission grids. Placing a coal power plant or multiple power plants near dense population centers can lower transmission costs. If a coalmine is nearby, transportation costs can also be reduced. However, emissions from coal plants play a key role in worsening health crises in many countries. And coal upon combustion produces CO 2 , SO 2 , NO x , CO, Metallic and Particle Matter (PM10 & PM2.5). The presence of these chemical compounds in the atmosphere in close vicinity to humans, livestock, and agriculture carries detrimental health consequences. The goal of the research was to develop a methodology to minimize the public’s exposure to harmful emissions from coal power plants while maintaining minimal operational costs related to electric distribution losses and coal logistics. The objective was...

Evaluation of the Safety and Efficacy of Continuous Use of a Home-Use High-Frequency Facial Treatment Appliance

At present, many home-use beauty devices are available in the market. In particular, many products developed for facial treatment use light, e.g., a flash lamp or a light-emitting diode (LED). In this study, the safety of 4 weeks’ continuous use of NEWA TM , a high-frequency facial treatment appliance, every alternate day at home was verified, and its efficacy was evaluated in Japanese individuals with healthy skin aged 30 years or older who complained of sagging of the facial skin.  Transepidermal water loss (TEWL), melanin levels, erythema levels, sebum secretion levels, skin color changes and wrinkle improvement in the facial skin were measured before the appliance began to be used (study baseline), at 2 and 4 weeks after it had begun to be used, and at 2 weeks after completion of the 4-week treatment period (6 weeks from the study baseline). In addition, data obtained by subjective evaluation by the subjects themselves on a visual analog scale (VAS) were also analyzed. Fur...