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Mucopolysaccharidosis Type I & Motor Development

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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 Idaho (ID)

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

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