Vol. 1, Issue 1, Part A (2019)

Background: Dandy-Walker syndrome is a rare heterogeneous congenital disorder characterized by a triad of cystic dilatation of the fourth ventricle, complete or partial agenesis of the cerebellar vermis and an enlarged posterior fossa. Most patients develop hydrocephalus with increased head size and variable degree of mental retardation, and my develop spasticity and seizures. The syndrome has not been well-documented in Iraq. The aim of this paper is to report the unusual presentation of the syndrome with a presentation like ataxic cerebral palsy with mental retardation without increase in head size because of hydrocephalus. The patient also had bilateral pachygyria more on the left side on brain CT-scan which is also unusual radiologic sign in this condition.Results: The boy was hypotonic since early infancy and experienced delayed development, unsteadiness and uncoordinated movements, and poor feeding. At the age of five years, he had ataxic movements, and was unable to sit unsupported, unable to feed self, and had no bowel control. He was not saying any word. He had poor cognitive abilities and did not understand simple commands. He was hypotonic and had normal tendon reflexes. Family history was negative for simillar illness. Brain CT-scan showed bilateral Pachygyria more on the left side in addition to the radiologic signs of Dandy-Walker syndrome.Patients and methods: A five year boy was referred with a clinical diagnosis of ataxic cerebral palsy and mental retardation was studied at the pediatric neuropsychiatry clinic of the Children Teaching Hospital of Baghdad Medical City during the year 2019. Conclusion: The of Dandy-Walker syndrome in Iraqi boy with unusual presentation, a presentation like ataxic cerebral palsy with mental retardation without increase in head size because of hydrocephalus is reported. In addition, the patient also had bilateral pachygyria more on the left side on brain CT-scan which is also unusual radiologic sign in this condition.