Neurodegenerative conditions: Exploring the role of music to enhance speech and cognitive functioning in children with batten disease

Neurodegenerative conditions: Exploring the role of music to enhance speech and cognitive functioning in children with batten disease

Ockelford A; Atkinson R

Developmental Medicine And Child Neurology

2017

Music Therapy; neuronal ceroid lipofuscinosis; speech; Child; cohort analysis; endogenous compound; Female; Human; Language; Male; Mood; Perception; singing; Teaching

Batten disease, the most common form of the neuronal ceroid lipofuscinoses, is a genetic life-limiting neurodegenerative condition that presents as early-onset dementia in children (Mole, Williams and Goebel, 2011). Symptoms include blind-ness, epilepsy, hallucinations, memory loss, the decline of speech, language and swallowing abilities, and the catastrophic deterioration of fine and gross motor skills (Bills et al., 1998). In a recent European research initiative led by Bengt Elmerskog (National Resource Centre for VI, Norway), exploring educational and therapeutic strategies for children with Batten disease, parents reported that music plays an increasingly significant role in their children's lives as the disease progresses. Evidence investigating the effects of music on neurological conditions has highlighted important links between music perception and speech, language, cognition and movement (Zatorre, 2013, Magee, 2017). This presentation will illustrate initial findings from two studies currently in train at the Applied Music Research Centre at the University of Roehampton. The first is following a cohort of 12 children and young people with Batten disease (CLN2, CLN3, CLN5, CLN 6 and CLN8) over a period of three years, to ascertain the extent to which weekly musical interventions and music therapy may have the capacity to regulate speech, control movement, enhance mood and promote social interac-tion. The second, doctoral study, which focuses on CLN3, the juvenile form of Batten disease, explores how singing may facilitate the production of expressive language when speech is in decline through (a) encouraging patients to learn the benefits of melodic intonation and rhythmic speaking techniques, and (b) teaching patients a repertoire of 'micro-songs', that embody key functional language. It is proposed that techniques may help scaffold language and everyday speech and, subsequently, maintain functional speech and communication for longer.

2017

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