The Ljungan virus (LV) has been shown to cause central nervous system malformations in laboratory mouse models. The LV has also been associated with intrauterine fetal death in humans. We investigated the presence of LV in a series of human hydrocephaly and anencephaly cases from elective abortions.


A series of elective abortions owing to hydrocephaly, anencephaly, and similarly aged trisomy 21 elective abortions as controls were examined for LV by immunohistochemistry and real time RT-PCR. A second experiment involved newborn mice exposed to LV.


LV was diagnosed in 9 of 10 cases with hydrocephalus and in 1 of 18 trisomy 21 controls by immunohistochemistry. Five of nine cases with anencephaly had a positive PCR result, whereas none of the 12 trisomy 21 available for PCR testing had a positive result. The 47 newborn mice exposed to LV all developed encephalitis, with eight having hydrocephalus. None of the 52 control animals had encephalitis or hydrocephalus.


The association between LV and both hydrocephaly and anencephaly suggests that LV may be playing an important role in central nervous system malformations in humans. Birth Defects Research (Part A), 2009. © 2009 Wiley-Liss, Inc.