title: Morphological manifestations of the Dandy-Walker syndrom in female members of a family.
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.
authors.forename: Marina
authors.initials: M
authors.lastname: Titlić
authors.forename: Stanko
authors.initials: S
authors.lastname: Alfirević
authors.forename: Krešimir
authors.initials: K
authors.lastname: Kolić
authors.forename: Anamarija
authors.initials: A
authors.lastname: Soldo
authors.forename: Ana Batoš
authors.initials: AB
authors.lastname: Tripalol
mesh_terms: D000328:Adult; D016080:Arachnoid Cysts; D002552:Cerebral Ventricles / Q000473:pathology*; D002675:Child, Preschool; D003616:Dandy-Walker Syndrome / Q000175:diagnosis*; D005192:Family Health; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000150:complications / Q000175:diagnosis*; D007223:Infant; D007427:Intracranial Pressure; D008279:Magnetic Resonance Imaging; D008297:Male; D010375:Pedigree; D012737:Sex Factors; D055815:Young Adult