EPIDEMIOLOGIC DATA ON WERDNIG-HOFFMANN DISEASE IN GERMANY (WEST-THURINGEN)

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.