Alberto Romano1,2,3, Elena Ippolito4, Camilla Risoli5, Edoardo Malerba6, Martina Favetta2, Andrea Sancesario2, Meir Lotan7,8, Daniel Sender Moran1. 1. Department of Health System Management, Ariel University, Ariel 4070000, Israel. 2. Movement Analysis and Robotics Laboratory, Intensive Neurorehabilitation and Robotics Department, Bambino Gesù Children's Hospital, 00165 Rome, Italy. 3. Centro AIRETT Ricerca e Innovazione (CARI), Research and Innovation AIRETT Center, 37122 Verona, Italy. 4. SMART Learning Center, 20133 Milan, Italy. 5. Department of Radiological Functions, Radiology Unit, Guglielmo da Saliceto Hospital, 29121 Piacenza, Italy. 6. Poliambulatorio Health Medical, 29122 Piacenza, Italy. 7. Department of Physiotherapy, Ariel University, Ariel 4070000, Israel. 8. Israeli Rett Syndrome National Evaluation Team, Ramat Gan 5200100, Israel.
Abstract
BACKGROUND: A scoliosis prevalence of 94% was reported in the population with Rett syndrome (RTT), with an annual progression rate of 14 to 21° Cobb which may result in pain, loss of sitting balance, deterioration of motor skills, and lung disfunction. This paper describes the efficacy of an intensive conservative individualized physical and postural activity program in preventing scoliosis curvature progression in patients with RTT. METHODS: Twenty subjects diagnosed with RTT and scoliosis were recruited, and an individualized intensive daily physical activity program was developed for each participant. Each program was conducted for six months by participants' primary caregivers in their daily living environment. Fortnightly remote supervision of the program implementation was provided by an expert therapist. Pre- and post-intervention radiographs and motor functioning were analyzed. RESULTS: An averaged progression of +1.7° ± 8.7° Cobb, over one year (12.3 ± 3.5 months) was observed in our group, together with motor function improvements. A relation between curve progression and motor skill improvement was observed. CONCLUSIONS: The intervention prevented scoliosis progression in our group. The achievement of functional motor improvements could enable better body segment control and muscle balancing, with a protective effect on scoliosis progression. The intervention was effective for individuals with RTT across various ages and severity levels. Individual characteristics of each participant and the details of their activity program are described.
BACKGROUND: A scoliosis prevalence of 94% was reported in the population with Rett syndrome (RTT), with an annual progression rate of 14 to 21° Cobb which may result in pain, loss of sitting balance, deterioration of motor skills, and lung disfunction. This paper describes the efficacy of an intensive conservative individualized physical and postural activity program in preventing scoliosis curvature progression in patients with RTT. METHODS: Twenty subjects diagnosed with RTT and scoliosis were recruited, and an individualized intensive daily physical activity program was developed for each participant. Each program was conducted for six months by participants' primary caregivers in their daily living environment. Fortnightly remote supervision of the program implementation was provided by an expert therapist. Pre- and post-intervention radiographs and motor functioning were analyzed. RESULTS: An averaged progression of +1.7° ± 8.7° Cobb, over one year (12.3 ± 3.5 months) was observed in our group, together with motor function improvements. A relation between curve progression and motor skill improvement was observed. CONCLUSIONS: The intervention prevented scoliosis progression in our group. The achievement of functional motor improvements could enable better body segment control and muscle balancing, with a protective effect on scoliosis progression. The intervention was effective for individuals with RTT across various ages and severity levels. Individual characteristics of each participant and the details of their activity program are described.
Entities:
Keywords:
Rett syndrome; home exercise program; motor skills; physical therapy modalities; scoliosis; telerehabilitation
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