Mucopolysaccharidosis Type I & Motor Development
Children with Prader-Willi syndrome (PWS) have developmental, postural, and movement disabilities from birth through adulthood.
Newborns:
- Hypotonia presents before birth with decreased fetal movements, abnormal fetal position at delivery, and an increased incidence of assisted delivery or cesarean section.
- Neonatal hypotonia is a nearly universal finding. Hypotonia is of central origin and results in decreased movement and lethargy, decreased spontaneous arousals, weak cry, and poor reflexes, including a poor suck. Special feeding and arousal techniques may be necessary, including nasogastrointestinal feeding. Nutritional service and assessments are required from infancy throughout adulthood.
Infants and toddlers:
- Infants with PWS demonstrate hypotonia leading to a delay in the achievement of gross motor milestones.
- Sitting is usually around 12 months, walking at 24 months.
- Developmental skills should be closely monitored and Early Intervention should begin as soon as possible.
Preschool and childhood:
- Overeating and obesity may become a problem in the preschool years.
- Low muscle tone often improves.
- Children with PWS continue to have problems with strength, coordination, balance, and motor planning, and they have delays in activities such as jumping, climbing, and catching.
- Fine motor skills are also delayed, with difficulties in activities, such as drawing and writing.
- Motor problems often compound learning and behavior problems.
- Interventions with growth hormone therapy and rehabilitation techniques, such as orthotics and therapies, help maximize function.
- Motor problems continue.
- Orthopedic complications, particularly scoliosis, are often observed. [Kroonen: 2006]
- In addition to therapies, adaptive and modified physical education programs are recommended to maintain strength, muscle tone, and function.
Adolescence and adulthood:
- Motor problems continue.
- Orthopedic complications, particularly scoliosis, are often observed. [Kroonen: 2006]
- In addition to therapies, adaptive and modified physical education programs are recommended to maintain strength, muscle tone, and function.
Resources
Services for Patients & Families in New Mexico (NM)
Service Categories | # of providers* in: | NM | NW | Other states (3) (show) | | NV | RI | UT |
---|---|---|---|---|---|---|---|---|
Adaptive Sports | 22 | 9 | 17 | 27 | 52 | |||
Dieticians and Nutritionists | 1 | 1 | 4 | 3 | 6 | |||
Early Intervention for Children with Disabilities/Delays | 34 | 3 | 31 | 13 | 51 | |||
Pediatric Orthopedics | 7 | 4 | 8 | 16 | 11 | |||
Physical Therapy | 12 | 9 | 5 | 40 |
For services not listed above, browse our Services categories or search our database.
* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.
Authors & Reviewers
Author: | Merlin G. Butler, MD, PhD |
2014: revision: Merlin G. Butler, MD, PhDA |
2008: update: Mary Riske, RN, MSA |
2008: first version: Kyna Byerly, MS, CGCCA |
Page Bibliography
Kroonen LT, Herman M, Pizzutillo PD, Macewen GD.
Prader-Willi Syndrome: clinical concerns for the orthopaedic surgeon.
J Pediatr Orthop.
2006;26(5):673-9.
PubMed abstract
Cites osteopenia, poor impulse control and defiant behaviors, and diminished pain sensitivity as aspects of PWS that may complicate
all facets of orthopaedic nonsurgical and surgical management in this patient population.