Dandy Walker Malformation
Classification according to ICD-10 | |
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Q03.1 | Atresia of the median aperture (foramen magendii) or the lateral aperture (foramina Luschkae) of the fourth ventricle |
ICD-10 online (WHO version 2019) |
The Dandy-Walker syndrome , also Dandy-Walker malformation or Dandy-Walker syndrome called, is a congenital disorder of the central nervous system caused by a hypoplasia of the cerebellar vermis and cystic expansion of the fourth ventricle is characterized.
Symptoms
The affected people stand out due to delayed motor development, muscular hypotension and a coordination disorder ( ataxia ). Half of the children also have cognitive developmental delays or disabilities, and some have hydrocephalus .
genetics
Genetically there appears to be a link to deletions on chromosome 3q with two candidate genes: ZIC1 and ZIC4.
history
The American neurosurgeon Walter Edward Dandy (1886–1946) was the first doctor to describe this malformation in 1914 . The neurologist Arthur Earl Walker (1907-1995) published in 1944 a compilation of all known cases. The suggestion of a name as Dandy Walker Syndrome was made in 1954 by the German-born US neurologist and psychiatrist Clemens Ernst Benda .
Web links
- Dandy Walker Syndrome / DWS. In: Online Mendelian Inheritance in Man . (English).
See also
Individual evidence
- ↑ Dandy WE, Blackfan KD. Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child (Chicago) 1914; 8: 406-482
- ^ Walker AE. A case of congenital atresia of the foramina of Luschka and Magendie: Surgical cure. J Neuropath Exp Neurol 1944; 3: 368-373
- ^ Benda CE. The Dandy-Walker syndrome or the so-called atresia of the foramen of Magendie. J Neuropath Exp Neurol 1954; 13: 14-39