A hungarian study on Werdnig-Hoffmann disease.

The prevalence of Werdnig-Hoffmann disease was found to be 0.66 per 10,000 livebirths in Hungary from 1973 to 1980. However, the true prevalence is estimated to be 1 per 10,000 livebirths, which exceeds the level of a previously published English study. There was no higher fetal death rate in previous and subsequent pregnancies of index patients' mothers. The occurrence in sibs was 32%, probably because of greater ascertainment in families with more than one affected child.

[1]  T. Schaap.  Werdnig‐Hoffmann disease on Reunion Island: a founder effect? , 1985, Clinical genetics.

[2]  J. Feingold,et al.  Cluster of acute infantile spinal muscular atrophy (Werdnig‐Hoffmann disease) in a limited area of Reunion Island , 1984, Clinical genetics.

[3]  A. Czeizel,et al.  Cases of a Genetic Counselling Clinic. , 1980, Acta paediatrica Academiae Scientiarum Hungaricae.

[4]  K. Fried,et al.  High incidence of spinal muscular atrophy type I (Werdnig ‐ Hoffmann disease) in the Karaite community in Israel , 1977, Clinical genetics.

[5]  A. Czeizel,et al.  The occurrence of consanguineous marriages in Hungary. , 1976, Human heredity.

[6]  J. Pearn.  The Gene Frequency of Acute Werdnig-Hoffmann Disease (SMA Type 1). A Total Population Survey in North-East England , 1973, Journal of medical genetics.

[7]  A. Emery.  The nosology of the spinal muscular atrophies. , 1971, Journal of medical genetics.

[8]  A. Emery,et al.  Spinal muscular atrophy type II , 1971 .

[9]  A. Emery,et al.  Spinal muscular atrophy type II. A separate genetic and clinical entity from type I (Werdnig-Hoffmann disease) and type 3 (Kugelberg-Welander disease). , 1971, Clinical genetics.

[10]  D. F. Roberts,et al.  The Genetic Component in Child Mortality , 1970, Archives of disease in childhood.

[11]  WERDNIG‐HOFFMANN'S INFANTILE PROGRESSIVE MUSCULAR ATROPHY AND ITS RELATION TO AMYOTONIA CONGENITA , 1951 .

[12]  S. Brandt.  HEREDITARY FACTORS IN INFANTILE PROGRESSIVE MUSCULAR ATROPHY: Study of One Hundred and Twelve Cases in Seventy Families , 1949 .