title: Patterns of cognitive and fine motor deficits in a case of Dandy-Walker continuum.
abstract: Cerebellar vermian hypoplasia in the context of Dandy-Walker complex is a relatively common disorder associated with a variety of cognitive and behavioral deficits in addition to impairment in motor control. Few studies, however, have examined the neuropsychological profiles of children with isolated hypoplasias of the cerebellum. Herein, we report a 6-year-old girl with Dandy-Walker continuum presenting with mild mental retardation and an inability to produce intelligible speech, despite adequate comprehension of single items and simple instructions. She was able to articulate vowels but not consonants, and fine motor function was deficient. Visual memory was intact for single items but not for multiple items, and visuospatial perception was impaired. An inability to form intelligible speech is not typically reported in cases of isolated vermian hypoplasia. The case extends our knowledge of the phenotypes associated with cerebellar hypoplasia and its relation to fine motor and articulatory control.
authors.affiliation: Department of Psychology, University of Athens, Athens, Greece.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Economou
authors.forename: Christos D
authors.initials: CD
authors.lastname: Katsetos
mesh_terms: D002531:Cerebellum / Q000473:pathology; D002648:Child; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D003616:Dandy-Walker Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008568:Memory / Q000502:physiology; D009069:Movement Disorders / Q000175:diagnosis / Q000209:etiology*; D009483:Neuropsychological Tests; D011597:Psychomotor Performance / Q000502:physiology*; D014796:Visual Perception / Q000502:physiology