Dandy–Walker syndrome

The Dandy-Walker malformation or malformation is a congenital disorder of the central nervous system that is characterized by hypoplasia of the cerebellar vermis and cystic enlargement of the fourth ventricle. It is also referred to as Dandy-Walker syndrome.

Symptoms

The people affected by falling on delayed motor development, hypotonia and incoordination (ataxia). Half of the children has an additional cognitive developmental delay or disability - some hydrocephalus.

Genetics

Genetically seems to be a connection to deletions on chromosome 3q to exist, with two candidate genes: ZIC1 and ZIC4.

History

The American neurosurgeon Walter Dandy Edward (1886-1946), described in 1914 as the first physician to this malformation. The neurologist Arthur Earl Walker (1907-1995) published in 1942 a compilation of all previously known cases.