title: Bobble-head doll syndrome associated with Dandy-Walker syndrome. Case report.
abstract: Bobble-head doll syndrome (BHDS) presents in childhood and is usually associated with lesions of the third ventricle. This disorder is characterized by stereotypical head movements of the type "yes-yes" (up and down) at a frequency of 2 to 3 Hz. Rarely, movements of the type "no-no" (side-to-side) are described. There are a few hypotheses to explain the mechanism responsible for BHDS, but its real pathophysiological characteristics are still unknown. The authors describe the case of a child born with hydrocephalus and Dandy-Walker syndrome. A ventriculoperitoneal shunt was implanted in the child because of progressive head enlargement. One year after shunt placement, she began making frequent horizontal head movements of the type "no-no". There were no other signs or symptoms. Imaging studies demonstrated small ventricles and a posterior fossa cyst with no signs of hypertension. The child's growth, development, and head circumference (within the 5th percentile) remained satisfactory. Three aspects of this case were of interest: the association of BHDS with Dandy-Walker syndrome, the rare occurrence of BHDS of the "no-no" type, and the absence of third ventricle dilation. The authors' findings support the hypothesis that cerebellar malformations themselves can
authors.affiliation: Hospital das Clinicas, Federal University of Minas Gerais, Brazil. henriques_jgb@hotmail.com
authors.forename: José Gilberto
authors.initials: JG
authors.lastname: de Brito Henriques
authors.forename: Karina Santos Wandeck
authors.initials: KS
authors.lastname: Henriques
authors.forename: Geraldo Pianetti
authors.initials: GP
authors.lastname: Filho
authors.forename: Luiz Fernando
authors.initials: LF
authors.lastname: Fonseca
authors.forename: Francisco
authors.initials: F
authors.lastname: Cardoso
authors.forename: Márcia Cristina
authors.initials: MC
authors.lastname: Da Silva
mesh_terms: D002557:Cerebrospinal Fluid Shunts; D003616:Dandy-Walker Syndrome / Q000473:pathology* / Q000503:physiopathology* / Q000601:surgery; D020820:Dyskinesias / Q000209:etiology / Q000473:pathology*; D005260:Female; D019416:Head Movements; D006801:Humans; D006849:Hydrocephalus / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D007223:Infant; D008279:Magnetic Resonance Imaging; D020542:Third Ventricle / Q000002:abnormalities / Q000473:pathology; D014057:Tomography, X-Ray Computed