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Isolation and Activation of Murine Lymphocytes
08:08

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Published on: October 30, 2016

T-lymphocyte production, function, and death in children who recovered from opsoclonus-myoclonus syndrome

Alessandra Sottini1, Roberto Micheli, Claudia Ghidini

  • 1Laboratorio di Biotecnologie, Spedali Civili of Brescia, Brescia, Italy.

Pediatric Hematology and Oncology
|November 30, 2006
PubMed

Insights

Children with opsoclonus-myoclonus syndrome may have persistent low CD4+ T-cells even after recovery. This reduction in CD4+ lymphocytes is likely not due to intrinsic T-cell defects.

Area of Science:

  • Immunology
  • Pediatric Neurology

Background:

  • Opsoclonus-myoclonus syndrome (OMS) is a rare autoimmune disorder often affecting children.
  • Recent studies suggest a link between reduced CD4+ lymphocytes and active OMS.

Purpose of the Study:

  • To investigate the persistence of CD4+ lymphocyte reduction in children after recovery from opsoclonus-myoclonus syndrome.
  • To evaluate T-cell homeostasis and function in recovered OMS patients.

Main Methods:

  • Case study of two children who recovered from opsoclonus-myoclonus syndrome.
  • Analysis of peripheral blood CD4+ lymphocyte counts and T-cell populations (CD45RA+).
  • Assessment of T-cell heterogeneity and function.

Main Results:

  • One child exhibited reduced CD4+ lymphocytes two years post-disease onset.
  • A decrease in "naive" CD45RA+ CD4+ T-cells and mild restriction of T-cell heterogeneity were observed in one patient, possibly due to viral infection response.
  • No intrinsic T-cell defects were identified in either child.

Conclusions:

  • CD4+ lymphocyte reduction can persist in children following clinical recovery from opsoclonus-myoclonus syndrome.
  • The observed CD4+ lymphopenia is unlikely caused by inherent T-cell functional defects.

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