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Morphological Manifestations of the Dandy-Walker Syndrome in Female Members of a Family
Marina Titlić
; University of Split, School of Medicine, Split, Croatia and University of Split, University Hospital Center Split, Department of Neurology, Split, Croatia
Stanko Alfirević
orcid.org/0000-0001-5083-9013
; University of Split, School of Medicine, Split, Croatia
Krešimir Kolić
; University of Split, University Hospital Center Split, Department of Radiology, Split, Croatia
Anamarija Soklo
; »J. J. Strossmayer« University, School of Medicine, Osijek, Croatia
Ana Batoš Tripalo
; University of Zagreb, »Sestre Milosrdnice« University Hospital Center, Department of Radiology, Zagreb, Croatia
Abstract
The Dandy-Walker syndrome (DWS) is a hereditary disorder, appearing somewhat more frequently in women. The
most important characteristics of the DWS are the lack of the cerebellar vermis, varying from a partial lack to a complete
agenesis, and enlargement of the cerebrospinal spaces, especially in the fourth ventricle. The above mentioned morphological
changes clinically manifest in ataxia, increased intracranial pressure and hydrocephalus. Here is presented a
family with DWS, where the disease is contracted only by female members, in two generations, whereas no signs of DWS
have been noticed in male family members. DWS is clinically manifested from early childhood to middle age, with the
morphological changes varying from hypoplastic cerebellar vermis to widening of the brain ventricles and hydrocephalus
and arachnoid cyst in the occipital part.
Keywords
Dandy-Walker; malformation; brain; women
Hrčak ID:
147813
URI
Publication date:
30.3.2015.
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