Angelman Syndrome and the Potential for Management of Symptoms through Neurologic Music Therapy Techniques

Angelman Syndrome (AS) is a rare neuro-genetic disorder caused by a loss of function in the maternal UBE3A gene on chromosome 15 (Connor-Ahmad et al., 2023). Angelman syndrome causes significant impairment in developmental and behavioral areas as well as symptoms such as seizures, lack of verbal and expressive communication, sleep difficulties, poor fine and gross motor skills, and limited self-care abilities. With a prevalence of approximately 1 in 22,000 births, people with AS can live a long life, but not without continuous care. According to Sommese and Corrado (2021), around the age of six months, developmental delays are first noted, with clinical features not becoming noticeable until after the age of one. More than 80% of AS patients experience seizures with onset before the age of three. Mouthing objects, a happy demeanor, water attraction, hyperactivity, and a short attention span are all key characteristics of those diagnosed with AS.

Due to the severity of developmental delay, intellectual disability, and cognitive delay, it is highly recommended that individuals with AS seek early intervention services that target problematic symptoms (Ma et al., 2023). According to researchers, therapies such as physical therapy (PT), speech therapy (ST), occupational therapy (OT), and applied behavioral analysis (ABA) have all shown positive results in managing AS symptoms relating to development and behavior. 

Management of care and rehabilitation methods are the main focus of treatment for those with AS since there is currently no cure for this diagnosis (Sommese & Corrado, 2021). Because most who are diagnosed with AS have limited to no speech capabilities, researchers are still looking for the best communication method for these individuals. Speech therapy is one option that attempts to promote nonverbal communication methods through the use of alternative augmentative communication (AAC). In 2020, Roche and colleagues looked at three different types of AAC models and their effects on children with AS. The study found that enhanced natural gestures (ENG), picture exchange communication systems (PECS), and multi-modal ACC interventions all offered beneficial results in the development of communication methods for AS patients. It was suggested that other forms of therapy, identified by families of those with AS, have presented positive results in AS symptom management (Sommese & Corrado, 2021). These alternative therapies include hippotherapy, cranial osteopathy, aromatherapy, reflexology, hydrotherapy, and music therapy. However, current research supporting the effectiveness of these therapies with AS patients is extremely limited.

Within the field of music therapy, there are several neurologic music therapy (NMT) techniques that have explored targeting similar AS symptoms such as cognitive delay and lack of verbal speech and communication. Musical Sensory Orientation Training (MSOT), Auditory Perception Training (APT), and Musical Attention Control Training (MACT) are all NMT techniques that focus on cognitive goals (Thaut & Hoemberg, 2014). In the handbook of NMT, MSOT is used with developmental disorders to improve cognitive processing by using musical instruments to provide sensory stimulation and assistance in orienting a patient to time, place, and person (Myskja, 2014). Auditory perception training is integrated by having patients differentiate different components of sound, such as timbre, tempo, duration, pitch, and rhythmic patterns (Mertel, 2014). Musical Attention Control Training targets various kinds of attention, such as selective, divided, and sustained, by directing a patient with musical stimuli (Thaut & Hoemberg, 2014).

In one study focusing on the effectiveness of music therapy on individuals with Rett Syndrome, music therapy was shown to be beneficial in treating areas such as verbal and non-verbal communication, social skills, behavioral play, grip capabilities, eye contact, and epilepsy (Chou et al., 2019). The music therapist in this study used MSOT, APT, and MACT, as well as movement and music, in their treatment. The results of the study showed improvements in socialization and communication skills, as well as hand skills and eye gaze. Three out of the eleven participants with refractory epilepsy experienced a decrease in seizures, with two participants reducing by more than 50% and one participant being completely seizure-free during the 6-month follow-up.

While there is currently little research that tests the use of these specific neurologic music therapy interventions with AS, many individuals with a similar diagnosis have shown positive results in treating similar AS symptoms. More research is needed in this area; however, these results are a positive indication of another avenue of support for those living with Angelman syndrome.

-Beatriz Leal, Music Therapy Intern

References

Chou, M.-Y., Chang, N.-W., Chen, C., Lee, W.-T., Hsin, Y.-J., Siu, K.-K., Chen, C.-J., Wang, L.-J., & Hung, P.-L. (2019). The effectiveness of music therapy for individuals with Rett syndrome and their families. Journal of the Formosan Medical Association, 118(12), 1633–1643. https://doi.org/10.1016/j.jfma.2019.01.001

Connor-Ahmad, S., Tjeertes, J., Chladek, M., Newton, L., Symonds, T., Clinch, S., Vincenzi, B., & McDougall, F. (2023). Developing Angelman syndrome-specific clinician-reported and caregiver-reported measures to support holistic, patient-centered drug development. Orphanet Journal of Rare Diseases, 18(1), 156. https://doi.org/10.1186/s13023-023-02729-y

Ma, V. K., Mao, R., Toth, J. N., Fulmer, M. L., Egense, A. S., & Shankar, S. P. (2023). Prader-Willi and Angelman Syndromes: Mechanisms and Management. The Application of Clinical Genetics, 16, 41–52. https://doi.org/10.2147/TACG.S372708

Mertel, K. (2014). Auditory Perception Training (APT). In M. H. Thaut & V. Hoemberg (Eds.), Handbook of Neurologic Music Therapy (pp. 227–256). Oxford University Press.

Myskja, A. (2014). Musical Sensory Orientation Training (MSOT). In M. H. Thaut & V. Hoemberg (Eds.), Handbook of Neurologic Music Therapy (pp. 221-226). Oxford University Press.

Roche, L., Sigafoos, J., & Trembath, D. (2020). Augmentative and Alternative Communication Intervention for People With Angelman Syndrome: A Systematic Review. Current Developmental Disorders Reports, 7(1), 28–34. https://doi.org/10.1007/s40474-020-00187-w

Sommese, M., & Corrado, B. (2021). A Comprehensive Approach to Rehabilitation Interventions in Patients with Angelman Syndrome: A Systematic Review of the Literature. Neurology International, 13(3), Article 3. https://doi.org/10.3390/neurolint13030036

Thaut, M. H., & Hoemberg, V. (Eds.). (2014). Handbook of Neurologic Music Therapy. Oxford University Press.

Article Review: Music Therapy Supports Children with Neurological Diseases during Physical Therapy Interventions

Music and physical therapy are two professional disciplines that both play a vital role in the growth, rehabilitation, and enhancement of many patients' lives. In their own respects, they offer specific techniques and skills to better help their patients. Music therapy uses music as its primary tool to reach the non-musical goals of its patients. These goals can include, but are not limited to, motor movement, communication, emotional identification, social skills, and cognition. Physical therapy accomplishes goals through positioning and exercises that target assisting patients in their ability to move. These goals usually look to improve range of motion, balance, gait training, sitting and standing, pain management, and the strengthening of muscles. When these two successful disciplines come together, there is opportunity for great results.

One recent study looked to test the ability of live music therapy to improve the vital signs of child patients during their physical therapy treatments (Kobus et al.). This study took seventeen children between the ages of 1 to 11 years old, with hospital admission due to severe neurologic diseases, to attend two sessions per week, receiving both music therapy and physical therapy simultaneously. This was compared to two other physical therapy sessions without live music therapy. The data that was taken during each session looked at each patient's heart rate, respiratory rate, and oxygen saturation, taken 15 minutes before and 15 minutes after treatment. The purpose of the study was to see how these vital signs would be affected by having music therapy present versus not.

The primary goals of the physical therapy sessions were to obtain functional skills and improve the mobility of the patient. Exercises during treatment utilized positionings such as prone, back, and side positions. Other areas addressed were grasping and handling, turning around, getting up, and walking or running. According to the article, “the aim is to strengthen the child’s independence.” The physical therapist looks to create a genuine relationship with the patient during this process to better understand and influence positive attitudes toward treatment.

When looking at the music therapy side, the music therapist would come alongside the physical therapist by providing improvised playing for infants on softer-sounding instruments such as the sansula. The music therapist would use soft humming tones followed by adjusted tempos based on the breathing and heart rate of the child. In premature infants, it was shown that their vital signs would improve during these interventions. In contrast, during difficult physical therapy exercises, the music therapist would play faster and more upbeat music to match the energy needed by the patient to accomplish their task or exercise.

After a total of 256 sessions, 128 with live music therapy, and 128 without, the results of this study showed that the incorporation of live music therapy during physical therapy treatment was a beneficial option for improving the vital signs of children going through neurological rehabilitation. Significantly lower heart and respiratory rates with a mean difference of -8.0 beats per minute and -0.8 breaths per minute were a result of this study. In the area of oxygen saturation, improvement was seen by a higher level mean of +0.6%.

Both music therapy and physical therapy are great rehabilitative occupations that work toward similar goals. In the case of working together, especially on the rehabilitation side of patients, it is evident that coming together creates beautiful work that benefits their patients greatly and proves to show positive results.

-Beatriz Leal, Music Therapy Intern

References

Kobus, S., Bologna, F., Maucher, I., Gruenen, D., Brandt, R., Dercks, M., Debus, O., & Jouini, E. (2022). Music Therapy Supports Children with Neurological Diseases during Physical Therapy Interventions. International Journal of Environmental Research and Public Health, 19(3), 1492. https://doi.org/10.3390/ijerph19031492

Music Therapy within Assisted Living Facilities and Memory Care Groups and the Use of Songwriting Methods to Promote Quality of Life.

Quality of life, defined by the National Library of Medicine, refers to a concept that looks at the well-being of a person’s life in which both positive and negative aspects are measured during one period of time (Teoli and Bhardwaj, 2024). A few notable examples of these areas measured include health, relationships, autonomy in decision-making, independence or freedom, wealth, and physical surroundings. A common population in which quality of life is often measured is among older adults, especially those residing in assisted living or memory care centers. At this stage of life, a person’s independence begins to be taken away, and their health often declines, sometimes at a rapid pace. Health difficulties that commonly appear within this population are in the form of Alzheimer’s disease and dementia, arthritis, hearing loss, depression, and anxiety.

During a Pew Research survey on what areas influence a person’s quality of life as they age, areas such as the ability to feed oneself, ability to talk or communicate, maintenance of short- and long-term memory, being able to dress oneself, live without pain, life enjoyment and feelings of accomplishment were all rated. Results of the survey showed that all age groups rated the ability to talk or communicate as being the most important in maintaining quality of life throughout aging (van Leeuwen, et al., 2019).

Music therapy is a great avenue for enhancing an individual’s quality of life by providing them with opportunities for their voices to be heard through expressive communication. One intervention that can be used to accomplish this is songwriting. Songwriting can be an exciting yet intimidating concept to observers, preventing them from ever getting the chance to experience its unique and creative properties. A music therapist is able to break down these barriers and make them accessible and achievable by simplifying the process. For example, for a group of individuals within an assisted living facility, a music therapist might use visuals such as scenic photographs or physical objects to spark both lyrical and musical inspiration. The presented picture or objects may be passed around the group, prompting each resident to share what they think of when they see them. Common contributions will be from reminiscent memories or aspects of the picture or object that stands out to them as significant or beautiful. The music therapist can then take those words or phrases and create a personalized song based on the group's responses. This is a collaborative process in which control is placed in the hands of the residents. It can go further by giving different musical options to choose from, such as rhythm, tempo, and chord progressions.

For others that might require more support, such as a memory care group, allowing room for expressive communication can be in the form of lyric substitution in familiar songs. Some examples might be singing well-known classics such as “She’ll Be Coming Round the Mountain” or “Here Comes the Sun,” followed by the music therapist prompting the group with a question. Using a familiar tune can provide a sense of comfort, while the play on words allows for preferences and opinions to be acknowledged and heard. A question prompt might be “What is your favorite part of this season?”  by rewriting to the familiar tune:

“She’ll be Coming Round the Mountain”

• “There will be lots of beautiful flowers in the spring”

• “We’ll all have fun at picnics in the spring”

“Here Comes the Sun”

• “Here comes the rain”

• “Here come the flowers”

Overall, whether it’s creating a completely unique song from scratch or rewriting existing ones, songwriting is a positive way to improve the quality of life for older adults. It has the potential to meet an individual’s preferences and promote creativity, all while giving them back some form of control throughout a decline in independence.

-Beatriz Leal, Music Therapy Intern

References

Author, N. (2013, November 21). Chapter 6: Aging and Quality of Life. Pew Research Center. https://www.pewresearch.org/religion/2013/11/21/chapter-6-aging-and-quality-of-life/

Clark, I. N., Stretton-Smith, P. A., Baker, F. A., Lee, Y.-E. C., & Tamplin, J. (2020). “It’s Feasible to Write a Song”: A Feasibility Study Examining Group Therapeutic Songwriting for People Living With Dementia and Their Family Caregivers. Frontiers in Psychology, 11. https://doi.org/10.3389/fpsyg.2020.01951

Teoli, D., & Bhardwaj, A. (2024). Quality Of Life. In StatPearls. StatPearls Publishing. http://www.ncbi.nlm.nih.gov/books/NBK536962/

van Leeuwen, K. M., van Loon, M. S., van Nes, F. A., Bosmans, J. E., de Vet, H. C. W., Ket, J. C. F., Widdershoven, G. A. M., & Ostelo, R. W. J. G. (2019). What does quality of life mean to older adults? A thematic synthesis. PLoS ONE, 14(3), e0213263. https://doi.org/10.1371/journal.pone.0213263

The Importance of Incorporating Movement in Music Therapy to Increase Self-Regulation in Children with All Abilities

Movement and music is an essential part of a music therapy session. It is an intervention used to increase self-regulation skills with individuals of all ages and abilities. According to Kate Williams, an early childhood professor and music therapist, “self-regulation is used as an umbrella term to refer to a range of processes related to the regulation of attention, behavior, and emotions.” The ability to regulate one’s attention, behavior, and emotions is a complex process that begins to develop within the first five years of a child’s life. Many of the clients receiving music therapy services are working towards developing or growing in these areas of regulation. One of the many ways that this is addressed in a music therapy session is through the incorporation of movement or dance interventions.

Through the incorporation of movement and dancing, a client is given the opportunity to not only have a creative outlet but also work out any pent-up energy that might prevent them from focusing on more challenging tasks throughout the session. For this same reason, a music therapist might specifically place their movement intervention at the beginning of a session to assist the client in self-regulating through the movement of their body. However, if a client is experiencing overstimulation, or is observed to be struggling to focus throughout the session, the music therapist might switch gears in the middle of a session and move straight into a movement intervention to help the client work towards that self-regulation. 

When targeting attention, a uniquely created or already established dance routine might challenge the individual to follow the specific sequences and work towards maintaining sustained attention. To focus on the behavioral side, a music therapist might incorporate drums that allow a client to redirect negative behaviors on an instrument that allows alternating movements between both hands and arms while also providing an extra sensory input of deep pressure against the drum head. When looking to target emotional regulation, a client might follow along to the movements of “If You’re Happy and You Know It” while creating their own dance moves that imitate different emotions they might be feeling or could feel later. Even something as simple as spontaneously dancing along to a client’s favorite song can improve attention, mood, and the ability to express emotions.

Other ideas for movement interventions:

• Body percussion

• Jumping on a trampoline to their favorite song

• Freeze dance or “stop and go” to increase impulse control within a sequence

• Creating unique dance moves that match a themed song

• Incorporating instruments to play throughout a routine

• Drumming on a big floor drum at different tempos to prompt gross and fine motor movements

-Beatriz Leal, Music Therapy Intern

References

Williams, K. E. (2018). Moving to the Beat: Using Music, Rhythm, and Movement to Enhance Self-Regulation in Early Childhood Classrooms. International Journal of Early Childhood, 50(1), 85–100. https://doi.org/10.1007/s13158-018-0215-y

Williams, K. E., Savage, S., & Eager, R. (2020). Rhythm and Movement for Self-Regulation (RAMSR) intervention for preschool self-regulation development in disadvantaged communities: A clustered randomized controlled trial study protocol. BMJ Open, 10(9), e036392. https://doi.org/10.1136/bmjopen-2019-036392

Neurologic Music Therapy Techniques Targeting Speech and Communication for Individuals with Down Syndrome.

Neurologic Music Therapy (NMT) is an evidence-based system of music therapy that uses neuroscientific research targeting music’s effect on the brain. Neurologic music therapy techniques focus on areas such as cognition, speech and language, and motor dysfunctions caused by neurologic impairments or neurodevelopmental disorders. A music therapist with training in NMT techniques can help individuals accomplish their non-musical goals by using specific music interventions to reroute neuropathways.

Individuals diagnosed with Down Syndrome, also known as Trisomy 21, can have delayed speech and language development or display difficulties in their articulation and pronunciation. This can be an area where NMT techniques for speech or language could provide beneficial results in treatment. One case study conducted by Blythe LaGasse found that using NMT techniques with a child with Down Syndrome can show beneficial results in expressive communication and oral motor movements after 12 months of treatment.

Three specific NMT techniques that are useful in this population are Oral Motor Respiratory Exercises (OMREX), Developmental Speech and Language Training through Music (DSLM), and Therapeutic Singing (TS).

1. Oral Motor Respiratory Exercises (OMREX) are techniques that focus on the strengthening of the individual's facial muscles. These muscles, essential in the production of speech, can be strengthened by the vocalization of sounds through the use of wind instruments. Another aspect of OMREX looks to improve respiratory control, which can be accomplished through inhalation and sustained exhalation through the same wind instruments. To accomplish these, a music therapist might use  harmonicas, recorders, melodicas, or kazoos to create fun exercises for their client to engage in. 

2. Developmental Speech and Language Training through Music (DSLM) is another NMT technique that focuses on the improvement of functional communication and is especially useful with children. Singing, chanting, and instrument play are key areas used in treatment but come alongside engaging materials and experiences to promote the production of speech. A music therapist might use an engaging book and sing through its contents, providing musical stimuli while also prompting the repetition of sounds or words.

3. Therapeutic Singing (TS) is another useful NMT technique in promoting speech production in individuals with Down Syndrome. Singing through client-preferred songs is an essential part of most music therapy sessions; however, in the case of individuals with Down Syndrome, it can be useful in promoting the use of specific words or phrases through song. A music therapist can focus on areas such as breath control, pacing of speech, and even visual scanning as a client follows along to the presented lyrics.

Overall, there are several beneficial ways a music therapist can use NMT techniques to achieve great results when working with an individual with Down Syndrome when targeting speech and communication goals. The music therapist can take these techniques and add fun components such as visuals, props, or games to create a setting in which the client is working hard on these neurologic tasks while still having fun in the process.

-Beatriz Leal, Music Therapy Intern

References

Bruscia, K. E. (2012). Case examples of music therapy for developmental problems in learning and communication. Barcelona Publishers.  http://ebookcentral.proquest.com/lib/uiwtx/detail.action?docID=3117658

Key elements of the NMT evidence-based system. (2018, May 12). The Academy of Neurologic Music Therapy. https://nmtacademy.co/key-elements-of-nmt/

Thaut, M. H., & Hoemberg, V. (Eds.). (2014). Handbook of neurologic music therapy. Oxford University Press.

WHAT IS NEUROLOGIC MUSIC THERAPY. (n.d.). NMTSA. Retrieved February 17, 2024, from https://www.nmtsa.org/what-is-nmt