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On the Epidemiology of Juvenile Amaurotic Idiocy in Denmark

Published online by Cambridge University Press:  01 August 2014

Kirsten Kristensen  and
Hans Christensen Lou
Kirsten Kristensen*
Affiliation:
National Boarding School for Blind, and Partially Sighted Children, Kalundborg, Denmark
Hans Christensen Lou
Affiliation:
National Boarding School for Blind, and Partially Sighted Children, Kalundborg, Denmark
*
Refssnaeskolen, National Boarding School for Blind, and Partially Sighted Children, Kalundborg, Denmark

Abstract

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Juvenile amaurotic idiocy is an autosomal recessive disease with onset around 6 years of age. Principal symptoms are: blindness; dementia; focal and generalized seizures; cerebellar, striatic, and pyramidal deficits; and psychosis. The course is progressive, common duration 10-20 years. Epidemiological studies are very few, but important work has been done by Sjöstrand and Rayner in Sweden.

The preliminary results of the present study indicate a rather higher incidence of the disease in Denmark. It is furthermore demonstrated that there is no evidence of consanguinity between parents, and that the disease is geographically evenly distributed. These findings are in contrast with earlier epidemiological investigations.

Type
8. Free Contributions: Fourth Group
Information
Acta geneticae medicae et gemellologiae: twin research , Volume 23 , supplement S1 , January 1974 , pp. 325 - 327
Copyright
Copyright © The International Society for Twin Studies 1974

References

REFERENCES

Christensen Lou, H.O., Kristensen, K. 1973. A clinical and psychological investigation into juvenile amaurotic idiocy in Denmark. Dev. Med. Child Neurol., 15: 313–323.Google Scholar
Kristensen, K. 1974. (Thesis, in preparation). Rayner, S. 1962. Juvenile amaurotic idiocy in Sweden. Hereditas (Lund), p. 109.Google Scholar
Sjögren, T. 1931. Die Juvenile Amaurotische Idiotie. Hereditas (Lund), 14: 197–425.Google Scholar
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