Bobble-head doll syndrome associated with Dandy-Walker syndrome. Case report.
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
de Brito Henriques
José Gilberto
JG
Hospital das Clinicas, Federal University of Minas Gerais, Brazil. henriques_jgb@hotmail.com
Henriques
Karina Santos Wandeck
KS
Filho
Geraldo Pianetti
GP
Fonseca
Luiz Fernando
LF
Cardoso
Francisco
F
Da Silva
Márcia Cristina
MC
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