Intrathecal synthesis of interferon-alpha in infants with progressive familial encephalopathy.
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.
Lebon
P
P
INSERM Unité 43 de Recherche sur les Infections Virales, Hôpital St Vincent de Paul, France.
Badoual
J
J
Ponsot
G
G
Goutières
F
F
Hémeury-Cukier
F
F
Aicardi
J
J
D000914:Antibodies, Viral / Q000032:analysis; D001480:Basal Ganglia Diseases / Q000134:cerebrospinal fluid / Q000235:genetics / Q000378:metabolism; D001927:Brain Diseases / Q000134:cerebrospinal fluid / Q000235:genetics* / Q000378:metabolism; D002675:Child, Preschool; D005260:Female; D004854:Herpesvirus 4, Human / Q000276:immunology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007370:Interferon Type I / Q000096:biosynthesis* / Q000134:cerebrospinal fluid; D008297:Male
D000914:Antibodies, Viral; D007370:Interferon Type I