Dandy Walker Malformation

Classification according to ICD-10
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)

Dandy Walker variant with pons and cerebellum dysplasia in an 8-year-old child.

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

• Arnold Chiari Syndrome

Individual evidence

1. ↑ Dandy WE, Blackfan KD. Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child (Chicago) 1914; 8: 406-482
2. ^ Walker AE. A case of congenital atresia of the foramina of Luschka and Magendie: Surgical cure. J Neuropath Exp Neurol 1944; 3: 368-373
3. ^ Benda CE. The Dandy-Walker syndrome or the so-called atresia of the foramen of Magendie. J Neuropath Exp Neurol 1954; 13: 14-39

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !