This study contains the largest body of epidemiological data on Werdni
g-Hoffmann disease (acute infantile spinal muscular atrophy; ASMA) in
West-Thuringen in Germany. The incidence of ASMA was calculated to be
1 in 10 202 live births. The prevalence was 1 in 595 362 of the genera
l population (as of 31 December 1987). The study gives an unexpectedly
high incidence rate confirming the suggestion that ASMA in Central an
d Eastern Europe might be more frequent than in Western Europe. Howeve
r, we consider that this high incidence rate in West-Thuringen is a re
sult of the almost complete ascertainment made possible because of the
well-organised and centralised health system existing in Thuringen ov
er the last few decades.