Intrathecal synthesis of interferon-alpha in infants with progressive familial encephalopathy
P Lebon, J Badoual, G Ponsot, F Goutières… - Journal of the …, 1988 - Elsevier
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 …
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 …