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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Association Between Periodic Limb Movements in Sleep and Cerebrovascular Changes in Children With Sickle Cell Disease

Jenny Lin1, Kerry Morrone2, Deepa Manwani2

  • 1Division of Pediatric Respiratory and Sleep Medicine, The Children's Hospital at Montefiore, Albert Einstein College of Medicine, New York, New York.

Journal of Clinical Sleep Medicine : JCSM : Official Publication of the American Academy of Sleep Medicine
|August 7, 2019
PubMed

Insights

Periodic limb movements (PLMs) in children with sickle cell disease (SCD) are linked to a higher risk of cerebrovascular disease. This study found elevated PLMI in 42% of children with SCD, associated with increased stroke risks.

Area of Science:

  • Neurology
  • Hematology
  • Sleep Medicine

Background:

  • Periodic limb movements (PLMs) are linked to stroke risk, but their association with cerebrovascular disease in pediatric sickle cell disease (SCD) is understudied.
  • Sickle cell disease (SCD) is a known risk factor for cerebrovascular complications in children.

Purpose of the Study:

  • To investigate the association between elevated periodic limb movement index (PLMI) and cerebrovascular disease in children with SCD.
  • To identify clinical and laboratory differences between children with SCD with normal versus elevated PLMI.

Main Methods:

  • Retrospective review of medical records for 129 children (≤18 years) with SCD who underwent polysomnography.
  • Analysis of polysomnography data to determine periodic limb movement index (PLMI).
  • Correlation of elevated PLMI with cerebrovascular disease markers identified via MRI and transcranial Doppler.

Main Results:

  • Elevated PLMI (PLMI > 5 events/h) was observed in 42% of children with SCD.
  • Children with elevated PLMI showed higher hemoglobin S percentage, lower total iron, and higher arousal index.
  • A significant association was found between elevated PLMI and cerebrovascular stenosis, including Moyamoya disease, detected by MRI.

Conclusions:

  • The prevalence of elevated PLMI in children with SCD is higher than previously reported.
  • Elevated PLMI is significantly associated with increased rates of cerebrovascular disease in children with SCD, highlighting a potential risk factor.
Abstract

Keywords:
cerebrovascular diseasechildrenmagnetic resonance imagingperiodic limb movements in sleeppolysomnographysickle cell diseasetranscranial doppler

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