Intrathecal synthesis of interferon-alpha in infants with progressive familial encephalopathy

J Neurol Sci. 1988 Apr;84(2-3):201-8. doi: 10.1016/0022-510x(88)90125-6.


IFN-alpha was detected in cerebrospinal fluid and/or sera from 7 of 8 patients with a progressive familial encephalopathy associated with calcifications of the basal ganglia and white matter alterations. The secretion of IFN-alpha was prolonged, as shown by its presence at different times between birth and 5 years, and was not associated with IFN-gamma. Virological investigations excluded various congenital infections. In only 2 patients, high levels of Epstein-Barr virus antibodies were observed, indicating the possibility of an abnormal response to viral infection rather than a congenital infection. Further investigations are required for characterization of the recessive autosomal trait of this syndrome and its relation to the IFN system.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibodies, Viral / analysis
  • Basal Ganglia Diseases / cerebrospinal fluid
  • Basal Ganglia Diseases / genetics
  • Basal Ganglia Diseases / metabolism
  • Brain Diseases / cerebrospinal fluid
  • Brain Diseases / genetics*
  • Brain Diseases / metabolism
  • Child, Preschool
  • Female
  • Herpesvirus 4, Human / immunology
  • Humans
  • Infant
  • Infant, Newborn
  • Interferon Type I / biosynthesis*
  • Interferon Type I / cerebrospinal fluid
  • Male


  • Antibodies, Viral
  • Interferon Type I