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CCR7 signaling in pediatric opsoclonus-myoclonus: upregulated serum CCL21 expression is steroid-responsive

Michael R Pranzatelli1, Elizabeth D Tate, Nathan R McGee

  • 1National Pediatric Myoclonus Center and Neuroimmunology Research Laboratory, P.O. Box 19643, Springfield, IL 62794-9643, USA; Department of Neurology, SouthernIllinois University School of Medicine, P.O. Box 19643, Springfield, IL 62794-9643, USA.

Cytokine
|June 15, 2013
PubMed

Insights

Elevated serum CCL21 is a key biomarker in pediatric opsoclonus-myoclonus syndrome (OMS). Corticosteroids and ACTH effectively reduce CCL21 levels, offering potential therapeutic targets for this neurological disorder.

Area of Science:

  • Neuroimmunology
  • Pediatric Neurology
  • Inflammatory Biomarkers

Background:

  • Pediatric opsoclonus-myoclonus syndrome (OMS) is a rare autoimmune neurological disorder.
  • Identifying inflammatory mediators is crucial for effective treatment strategies.

Purpose of the Study:

  • To investigate the role of chemokines CCL19 and CCL21 in pediatric OMS.
  • To evaluate the impact of immunotherapies on chemokine levels.

Main Methods:

  • Prospective case-control study comparing children with OMS to controls.
  • Quantification of CCL19 in CSF and CCL21 in serum using ELISA.
  • Analysis of cross-sectional and longitudinal data, including effects of various immunotherapies.

Main Results:

  • Significantly elevated serum CCL21 levels were observed in untreated pediatric OMS patients compared to controls.
  • Corticosteroids and ACTH significantly reduced serum CCL21 to control levels.
  • CCL19 in CSF was not significantly altered by OMS or treatments; elevated serum CCL21 correlated with OMS severity.

Conclusions:

  • Serum CCL21 is a potential biomarker for pediatric OMS severity and duration.
  • Corticosteroids and ACTH are effective in down-regulating CCL21 production.
  • Further validation studies are needed to confirm CCL21's status as a treatment biomarker.
Keywords:
ACTHCorticosteroidsNeuroblastomaParaneoplastic syndromePediatric neuroinflammation

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