Dandy-Walker syndrome

Abstract

А mini-review and portfolio. Every year, the number of patients with congenital defects of the nervous system increases, which, on the one hand, may be related to the improvement of postnatal neuroimaging methods, and on the other hand, to a significant increase in the influence of adverse factors on brain development in the antenatal period of a child's life. Dandy-Walker syndrome (SDU) is an abnormality in the development of the cerebellum and its surrounding cerebrospinal fluid spaces; genetically determined disease. The syndrome was first described by Americans: neurosurgeon Walter Dandy (1886 – 1946) in 1921 and neurosurgeon, neurobiologist Earl Walker (1907 – 1995) in 1944. When establishing a diagnosis, it is necessary to differentiate SDU with cerebellar hypoplasia of other etiology, retrocerebral cysts and expansion of the large cerebral cistern. A defect of the cerebellar worm, which occurs in other cases of malformations of the central nervous system, is considered a pathognomonic sign of SDU. Differential diagnosis with arachnoid cysts is carried out, which requires additional instrumental research methods. SDU usually manifests itself in childhood, but rare cases have been reported in adulthood. This is illustrated by the portfolio data. Many patients remain clinically asymptomatic for many years, while others may have various associated developmental abnormalities leading to earlier diagnosis.