Movement, Singing, and Instrument Playing Strategies for a Child with Myotonic Dystrophy

ABSTRACT: Myotonic dystrophy, a type of muscular dystrophy, is defined as a genetic disorder with progressive muscle deterioration and weakness. The subject in this study, a 10-year-old boy, was diagnosed with congenital myotonic dystrophy with gross and fine motor deficits and articulation disorders. The purpose of this case study was to investigate the transfer of gross motor, articulation, and instrument playing skills during small group music therapy sessions to regular physical education classes and regular music education classes. Results indicated that the frequency of "accurate" task performances were significantly correlated in music therapy and physical education classes and music therapy and music education classes. Although the results of this study may not substantiate the transfer of skills from the music therapy group sessions to regular physical education and music education classes, the findings may suggest the efficacy of practicing gross motor, articulation, and instrument playing skills in small group music therapy settings as supplemental strategies that may enhance regular classroom skills. According to Emery (2000), there are at least 40,000 individuals affected with a severe form of muscular dystrophy in the United States of America. Muscular dystrophy is defined in various literatures as a hereditary and progressive condition that results in increasing degeneration of muscle cells (AhIstrom & Natterlund, 1999), increasing loss of muscle power (Willard & Spackman, 1971), and muscle weakness and wasting (Emery, 2000; Porr & Rainville, 1999). Different types of muscular disorders involve different groups of muscles (AhIstrom & Natterlund, 1999). Myotonic dystrophy is a distinct type of muscular dystrophy in which the congenital onset type is considered the most severe diagnosis (Harper, 2001; Moxley, 1997; Ruben & Macciomei, 1986). Harper (2001 (defined myotonic dystrophy as a genetically determined disorder where a characteristic pattern of dystrophic muscle disease is associated with myotonia and with specific abnormalities of a variety of other systems. All myotonic disorders appear to result 1) from either specific mutations in genes that encode muscle chloride or sodium channels or 2) from mutations, such as the protein kinase in myotonic dystrophy (Moxley, 1997). The affected muscles enlarge due to fat and connective tissue deposit, but muscle fibers degenerate and atrophy. The various affected areas associated with myotonic dystrophy include the face, fingers, hands, forearms, legs, and feet (Marieb, 2002). Weakness in the muscles of the face is a prominent feature of myotonic dystrophy (Porr & Rainville, 1999), which is especially severe in children with congenital myotonic dystrophy (Emery, 2000; Harper, 2001; Moxley, 1997; Ruben & Macciomei, 1986). Harper (2001) further pointed out that the jaw may hang open in children with congenital myotonic dystrophy that results in mouth breathing and speech disturbance and difficulties. Delayed motor development is another striking characteristic in myotonic dystrophy (Harper, 2001). Children with congenital myotonic dystrophy exhibit muscle wasting and weakness of lower leg and forearm muscles as well as the small muscles of the hands (Emery, 2000; Moxley, 1997; Ruben & Macciomei, 1986) and imbalance during gross motor skills (Harper, 2001). In addition to various physical deficits, mental retardation, usually moderate to mild in degree, is associated with congenital myotonic dystrophy (Harper, 2001 ; Jamero & Dundore, 1982; Moxley, 1997). Children with congenital myotonic dystrophy exhibit an increased frequency of hearing and speech disorders (Moxley, 1997), attention deficiencies (Ambrosini & Nurnberg, 1979; Moxley, 1997), mental deterioration (Ambrosini & Nurnberg, 1979; Jamero & Dundore, 1982), and memory disturbance (Ambrosini & Nurnberg, 1979) as they grow older. Other associated psychological aspects include attention deficit hyperactivity disorder and problem behaviors (Harper, 2001), somnolence, and apathy (Ambrosini & Nurnberg, 1979; Harper, 2001), and depression (Harper, 2001). …