To determine the prevalence of clinically significant nervous system d
isease attributable to transplacental transmission of Borrelia burgdor
feri, we surveyed neurologists in areas of the United States in which
Lyme disease is endemic (i.e., Massachusetts, Rhode Island, Connecticu
t, New York, New Jersey, Wisconsin, and Minnesota). Overall, 162 of th
e 176 (92%) pediatric neurologists contacted responded to the survey w
ith a range of 90-100% in the different geographic areas. One pediatri
c neurologist was following 3 children who were labeled as having ''co
ngenital Lyme disease,'' but none of the 3 met our case definition. No
ne of the other pediatric neurologists surveyed had ever seen a child
whose mother had been diagnosed as having Lyme disease during pregnanc
y. Similarly, none of the 37 adult neurologists in Connecticut surveye
d had ever seen a child whose mother had been diagnosed as having had
Lyme disease during pregnancy. We conclude that congenital neuroborrel
iosis is either not occurring or is occurring at an extremely low rate
in areas endemic for Lyme disease.