Prader- Willi Syndrome

Language & Learning Implications

Commonly Found Speech and Language Difficulties

A variety of characteristics could contribute to the high incidence of speech and language deficits, however, HYPOTONIA and COGNITIVE IMPAIRMENTS are regarded as the main cause of difficulties.

**Hypotonia may result in slower speech rates and imprecise articulation.

** High incidence of intellectual disability may result in significant impairments to expressive and receptive language due to deficits associated specifically with; auditory short term memory, temporal order processing, and auditory verbal processing.

Other factors include:

  • Structural characteristics of the mouth and jaw = impaired articulation/ compromised feeding as an infant
  • Inadequate velopharyngeal movement/ closure = hyponasality /hypernasality
  • Altered growth of the larynx due to endocrine dysfunction =poor pitch/ voice quality
  • Reduced saliva output = xerostomia (dry mouth)
  • Behavioral disturbances ie. verbal aggression = poor pragmatic language skills

(Lewis et al, 2002)

Poor articulation skills impact the normal development of language, the result is commonly a Co-occuring articulation and language deficits

Language Implications

  • Early signs of language delay: reduced babbling, and emergence of vocabulary as late as 18 months- 6 years of age
  • Expressive and receptive language difficulties
  • Difficulties with sequencing story events in narrative retell
  • Pragmatic language difficulties associated with common negative behaviors such as: verbal aggression, pre-occupation with food, and skin picking.
  • It was found in a 2012 study conducted by Anastasia Dimitropulos et al. that the causal genetic sub-type may impact language deficits differently. For all participants in her study who had Pradar-Willi Syndrome as a result of the maternal uniparental disomy (uMPD) their expressive language skills were better than their receptive language skills- this is opposite for most with Pradar-Willi Syndrome.

(Dimitropoulos et al., 2012)