Cytokine Production by Mononuclear Cells from Patients with Familial Infantile Bilateral Striatal Necrosis

Neuroimmunomodulation. 2017;24(4-5):276-281. doi: 10.1159/000486861. Epub 2018 Mar 6.

Abstract

Prompted by findings suggesting immune instability in infantile bilateral striatal necrosis (IBSN), we evaluated levels of proinflammatory (tumor necrosis factor α, interleukin [IL]-1β, IL-6, interferon [IFN]γ) and anti-inflammatory (IL-10 and IL-1ra) cytokines produced by peripheral blood mononuclear cells (PBMC) from 6 children with IBSN and 11 age-matched controls. Compared to controls, non-stimulated PBMC from the IBSN group produced a significantly lower level of IL-1ra (by 38%; p < 0.001) and significantly lower levels of TNFα, IL-1β, and IFNγ (by 36% [p < 0.001], 25% [p = 0.06], and 32% [p < 0.02]) under PBMC stimulation. The severe cachexia manifesting shortly after IBSN onset may impair the immunological state, placing patients at risk of death from hyperpyrexia and sepsis.

Keywords: Cytokines; Familial infantile bilateral striatal necrosis; Immunology; Mononuclear cells; Striatal necrosis.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cytokines / blood*
  • Cytokines / immunology*
  • Female
  • Humans
  • Infant
  • Leukocytes, Mononuclear / immunology*
  • Leukocytes, Mononuclear / metabolism*
  • Male
  • Striatonigral Degeneration / blood
  • Striatonigral Degeneration / congenital*
  • Striatonigral Degeneration / diagnosis
  • Striatonigral Degeneration / immunology

Substances

  • Cytokines

Supplementary concepts

  • Striatonigral degeneration infantile