José Gilberto De Brito Henriques, M.D.1,2,3,5,8, Karina Santos Wandeck Henriques, M.D.2,3,4,8, Geraldo Pianetti Filho, M.D.1, Luiz Fernando Fonseca, M.D.2,5, Francisco Cardoso, M.D., Ph.D.6, and Márcia Cristina Da Silva, M.D.7,8
1Hospital das Clinicas, Federal University of Minas Gerais 2Hospital Mater Dei 3Unifenas University 4Belo Horizonte Hospital 5Centro Geral de Pediatria, Federação Hospitalar do Estado de Minas Gerais 6Movement Disorders Service, Hospital das Clinicas, Federal University of Minas Gerais 7Hospital João XXIII; and 8São Camilo, Belo Horizonte, Minas Gerais, Brazil
Abbreviations used in this paper: BHDS = bobble-head doll syndrome; CT = computed tomography; MR = magnetic resonance; VP = ventriculoperitoneal.
Address reprint requests to: José Gilberto de Brito Henriques, M.D., Rua Araguari, 1045/301 30190-111 Belo Horizonte-Minas Gerais, Brazil. email:
henriques_jgb@hotmail.com.
DOI: 10.3171/PED-07/09/248
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 cause BHDS.