In both patients there was normal motor and intellectual development during childhood, but as adults they had gradual evolution of brain stem and cerebellar signs and obstructive hydrocephalus. Following resection of the fourth ventricular cyst, both patients recovered. A review of the literature disclosed seven additional patients in whom the Dandy-Walker syndrome was first diagnosed in adult life. These cases illustrate that this congenital brain malformation may not only first become symptomatic later in life, but that it is possible for patients to remain asymptomatic. Adult Onset of the Dandy-Walker Syndrome.
Dandy—Walker syndrome DWS is a rare group of congenital human brain malformations. There are three subtypes which affect multiple organs to varying degrees, but the fundamental abnormalities involve the cerebellum which controls muscle coordination. The degree of disability varies but is typically lifelong. In the majority of individuals with Dandy—Walker malformation, signs and symptoms are present at birth or develop within the first year of life. Some children have a buildup of fluid in the brain hydrocephalus that may cause increased head size macrocephaly. Up to half of affected individuals have intellectual disability that ranges from mild to severe, and those with normal intelligence may have learning disabilities.
This 34 years old man presented with moderate and persistent headache occurring 48 hours following Benin head injury. There were no symptoms of increased intracranial pressure. Neurological examination was unremarkable without cerebellar ataxia or psychomotor retardation. He was managed conservatively and he is doing well following 2 years ago.
Dandy Walker Syndrome. Dandy Walker syndrome is a rare type of abnormality in. There are two main. Syndrome, which are described below.