DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY
COMMENTARY
Duchenne muscular dystrophy and the stomatognathic system MARCELO COELHO GOIATO Department of Dental Materials and Prosthodontics, Aracßatuba Dental School, UNESP, Aracßatuba, S~ao Paulo, Brazil. doi: 10.1111/dmcn.12755 This commentary is on the original article by Ferreira et al. To view this paper visit http:/dx.doi.org/10.1111/dmcn.12707
Duchenne muscular dystrophy (DMD) is a chronic, progressive disease that only affects male children, due to the glycoprotein complex mutation that is related to the dystrophin gene.1 The lack of dystrophin promotes serious changes in the muscular system that begins in the lower limbs, causing gradual loss of functional capacity and decreased ambulation, usually the child as wheelchair dependent before 12 years of age. In the literature, few studies use electromyography to analyze the activity of skeletal striated muscle in individual carriers of DMD, especially in relation to the function of the stomatognathic system. A surface electromyographic analysis can be considered a valuable tool to understand the kinematics and kinetic changes that the disease causes throughout the human body affected by the condition. The article by Ferreira et al.2 focuses on the functional analysis of the stomatognathic system of patients with the disease, since the symptoms can possibly and eventually affect muscles in the masticatory system and also the neck.3 The results showed that even at an early age, children recently diagnosed demonstrated severe muscle changes, proving that DMD affects the function of the masticatory system even at an early stage of the disease. Interestingly,
in the article by Ferreira et al. the results found that the muscles in the mastication region are consistent with the characteristics of DMD, as observed in other regions of the human body in early and late stages of the disease.4 According to Kunert-Keil et al.5 DMD promoted oro-maxillary system changes, with its main clinical findings as pain in the ear region, a decline and lack of symmetry in mandibular movements, and excessive muscle tension that supports the onset of dental malocclusion. Ferreira et al.2 also shows alterations of the stomatognathic system regarding muscle thickness and bite force, as well as changes in masticatory efficiency, demonstrating that the disease can gradually affect kinematics and kinetics chewing, and can lead to serious problems in the chewing and swallowing process, affecting the quality of life of individuals and facilitating pulmonary diseases that can lead to death. The results obtained by Ferreira et al.2 show that patients with DMD need to re-establish the pattern of masticatory muscle activation as soon as possible, particularly during chewing. The changes throughout the stomatognathic system of these patients may be associated with oral problems already identified by other authors, such as increasing biofilm and dental calculus, gingival inflammation, anterior and posterior open bite, incompetent lips, mouth breathing, and macroglossy,6 with consequent impairment of the stomatognathic system. The dentist should be aware of all these signs and symptoms in patients with DMD, intervening early in an attempt to solve the imbalance between the lingual and facial muscles, promoting proper function of the stomatognathic system.
REFERENCES 1. Kawazoe Y, Kobayashi M, Tasaka T, Tamamoto M.
3. Matsuyuki T, Kitahara T, Nakashima A. Developmental
genes involved in the calcium homeostasis in mastica-
Effects of therapeutic exercise on masticatory function in
changes in craniofacial morphology in subjects with Duch-
tory muscles of MDX mice. J Physiol Pharmacol 2014;
patients with progressive muscular dystrophy. J Neurol
enne muscular dystrophy. Eur J Orthod 2006; 28: 42–50.
Neurosurg Psychiatry 1982; 45: 343–7. 2. Ferreira B, Da Silva GP, Goncßalves CR, et al. The effects of Duchenne muscular dystrophy on the performance of the stomatognathic system: case–control study. Dev Med Child Neurol; doi: 10.1111/dmcn.12707.
© 2015 Mac Keith Press
65: 317–24.
4. Theadom A, Rodrigues M, Roxburgh R, et al. Prevalence
6. Symons AL, Townsend GC, Hughes TE. Dental charac-
of muscular dystrophies: a systematic literature review.
teristics of patients with Duchenne muscular dystrophy.
Neuroepidemiology 2014; 43: 259–68.
ASDC J Dent Child 2002; 69: 277–83.
5. Kunert-Keil CH, Gredes T, Lucke S, Botzenhart U, Dominiak M, Gedrange T. Differential expression of
1
COMMENTARY
Duchenne muscular dystrophy and the stomatognathic system MARCELO COELHO GOIATO Department of Dental Materials and Prosthodontics, Aracßatuba Dental School, UNESP, Aracßatuba, S~ao Paulo, Brazil. doi: 10.1111/dmcn.12755 This commentary is on the original article by Ferreira et al. To view this paper visit http:/dx.doi.org/10.1111/dmcn.12707
Duchenne muscular dystrophy (DMD) is a chronic, progressive disease that only affects male children, due to the glycoprotein complex mutation that is related to the dystrophin gene.1 The lack of dystrophin promotes serious changes in the muscular system that begins in the lower limbs, causing gradual loss of functional capacity and decreased ambulation, usually the child as wheelchair dependent before 12 years of age. In the literature, few studies use electromyography to analyze the activity of skeletal striated muscle in individual carriers of DMD, especially in relation to the function of the stomatognathic system. A surface electromyographic analysis can be considered a valuable tool to understand the kinematics and kinetic changes that the disease causes throughout the human body affected by the condition. The article by Ferreira et al.2 focuses on the functional analysis of the stomatognathic system of patients with the disease, since the symptoms can possibly and eventually affect muscles in the masticatory system and also the neck.3 The results showed that even at an early age, children recently diagnosed demonstrated severe muscle changes, proving that DMD affects the function of the masticatory system even at an early stage of the disease. Interestingly,
in the article by Ferreira et al. the results found that the muscles in the mastication region are consistent with the characteristics of DMD, as observed in other regions of the human body in early and late stages of the disease.4 According to Kunert-Keil et al.5 DMD promoted oro-maxillary system changes, with its main clinical findings as pain in the ear region, a decline and lack of symmetry in mandibular movements, and excessive muscle tension that supports the onset of dental malocclusion. Ferreira et al.2 also shows alterations of the stomatognathic system regarding muscle thickness and bite force, as well as changes in masticatory efficiency, demonstrating that the disease can gradually affect kinematics and kinetics chewing, and can lead to serious problems in the chewing and swallowing process, affecting the quality of life of individuals and facilitating pulmonary diseases that can lead to death. The results obtained by Ferreira et al.2 show that patients with DMD need to re-establish the pattern of masticatory muscle activation as soon as possible, particularly during chewing. The changes throughout the stomatognathic system of these patients may be associated with oral problems already identified by other authors, such as increasing biofilm and dental calculus, gingival inflammation, anterior and posterior open bite, incompetent lips, mouth breathing, and macroglossy,6 with consequent impairment of the stomatognathic system. The dentist should be aware of all these signs and symptoms in patients with DMD, intervening early in an attempt to solve the imbalance between the lingual and facial muscles, promoting proper function of the stomatognathic system.
REFERENCES 1. Kawazoe Y, Kobayashi M, Tasaka T, Tamamoto M.
3. Matsuyuki T, Kitahara T, Nakashima A. Developmental
genes involved in the calcium homeostasis in mastica-
Effects of therapeutic exercise on masticatory function in
changes in craniofacial morphology in subjects with Duch-
tory muscles of MDX mice. J Physiol Pharmacol 2014;
patients with progressive muscular dystrophy. J Neurol
enne muscular dystrophy. Eur J Orthod 2006; 28: 42–50.
Neurosurg Psychiatry 1982; 45: 343–7. 2. Ferreira B, Da Silva GP, Goncßalves CR, et al. The effects of Duchenne muscular dystrophy on the performance of the stomatognathic system: case–control study. Dev Med Child Neurol; doi: 10.1111/dmcn.12707.
© 2015 Mac Keith Press
65: 317–24.
4. Theadom A, Rodrigues M, Roxburgh R, et al. Prevalence
6. Symons AL, Townsend GC, Hughes TE. Dental charac-
of muscular dystrophies: a systematic literature review.
teristics of patients with Duchenne muscular dystrophy.
Neuroepidemiology 2014; 43: 259–68.
ASDC J Dent Child 2002; 69: 277–83.
5. Kunert-Keil CH, Gredes T, Lucke S, Botzenhart U, Dominiak M, Gedrange T. Differential expression of
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