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Periodic limb movement in sleep in children with Williams syndrome

R Arens1, B Wright, J Elliott

  • 1Division of Pulmonary Medicine, Children's Hospital of Philadelphia, PA 19104-4399, USA.

The Journal of Pediatrics
|November 20, 1998
PubMed

Insights

Children with Williams syndrome (WS) often experience sleep disturbances due to periodic limb movement in sleep (PLMS). This study found a significant association between WS and PLMS, with clonazepam showing potential benefits.

Area of Science:

  • Neuroscience
  • Sleep Medicine
  • Genetics

Background:

  • Williams syndrome (WS) is a genetic disorder associated with neurodevelopmental and behavioral issues, including sleep problems.
  • Parents of children with WS frequently report difficulties with sleep initiation and maintenance, often linked to restlessness and frequent arousals.

Purpose of the Study:

  • To investigate the prevalence of movement arousal sleep disorders in children with Williams syndrome.
  • To evaluate the specific association between Williams syndrome and periodic limb movement in sleep (PLMS).

Main Methods:

  • A telephone survey was conducted with 28 families of children with WS to screen for movement arousal disorders.
  • Polysomnography was performed on 7 children with WS who screened positive for a movement disorder, and their results were compared to 10 age-matched controls.

Main Results:

  • Children with WS showed a significantly higher Periodic Limb Movement in Sleep (PLMS) index (14.9 vs 2.8) compared to controls.
  • Arousal and awakening events in children with WS were strongly correlated with PLMS, and they spent more time awake during sleep periods.
  • Treatment with clonazepam resulted in a significant clinical response in 4 out of 5 children with WS and PLMS.

Conclusions:

  • This study establishes a significant association between Williams syndrome and periodic limb movement in sleep (PLMS).
  • Clonazepam may be an effective treatment option for reducing clinical symptoms associated with PLMS in children with Williams syndrome.
Abstract

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