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Mucopolysaccharidosis Type I & Motor Development

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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)

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* 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

Initial publication: September 2008; last update/revision: July 2019
Current Authors and Reviewers:
Author: Merlin G. Butler, MD, PhD
Funding: This page was developed in partnership with the Heartland Genetic Services Network and was funded in part by a Health Resources Services Administration (HRSA) cooperative agreement (U22MC03962). We appreciate the Prader-Willi Syndrome Association (USA) for their outstanding support of individuals with PWS and their families and for the information they provide on their website – www.pwsausa.org – to which we have provided several links within the Diagnosis Module.
Authoring history
2014: revision: Merlin G. Butler, MD, PhDA
2008: update: Mary Riske, RN, MSA
2008: first version: Kyna Byerly, MS, CGCCA
AAuthor; CAContributing Author; SASenior Author; RReviewer

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.