Bobble-head doll syndrome is a rare movement disorder that is usually associated with lesions involving the third ventricle. It is characterised by stereotypical rhythmic up-and-down or side-to-side head movements. The pathophysiology and anatomical basis for this unusual manifestation is still a subject of intense scrutiny. The syndrome has never been described in a patient with both hydranencephaly and Chiari type 3 malformation. We describe a 2-year-old female patient who presented with congenital hydrocephalus, an occipital encephalocele and rhythmic bobbling of the head. Imaging investigation revealed a Chiari type 3 malformation and hydranencephaly. The patient was taken to theatre for a ventriculoperitoneal shunt insertion, and at day 3 post operatively, the patient had a markedly decreased head circumference and a decrease in the frequency of the bobbling of the head. A further review at 2 weeks showed that the bobbling of the head had ceased. Although the pathophysiology of bobble-head doll syndrome is yet to be fully understood, there has been postulation of either a third ventricular enlargement or a cerebellar dysfunction to explain bobble-head doll syndrome. Our case illustrates that the pathophysiology is most likely multifactorial as illustrated by the fact that by just addressing the high intracranial pressure with a shunt was sufficient to treat the condition.
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Ahn Y et al (1997) Bobble-head doll syndrome associated with subduroperitoneal shunt malfunction. Childs Nerv Syst 13(4):234–237. https://doi.org/10.1007/s003810050074
Alexiou GA et al (2013) Giant suprasellar arachnoid cyst with head bobbing. Mov Disord 28(9):1216–1216. https://doi.org/10.1002/mds.25511
Benton JW, Nellhaus G, Huttenlocher PR, Ojeman RG, Dodge PR (1966) The bobble-head doll syndrome: report of a unique truncal tremor associated with third ventricular cyst and hydrocephalus in children. Neurology 16:725–729
Bhattacharyya KB et al (2014) Bobbling head in a young subject. Ann Indian Acad Neurol 17(4):371. https://doi.org/10.4103/0972-2327.143992
Henriques DB, Gilberto J et al (2007) Bobble-head doll syndrome associated with Dandy–Walker syndrome. J Neurosurg Pediatr 107(3):248–250. https://doi.org/10.3171/PED-07/09/248
Dublin AB, French BN (1980) Diagnostic image evaluation of hydranencephaly and pictorially similar entities, with emphasis on computed tomography. Radiology 137(1 Pt 1):81–91. https://doi.org/10.1148/radiology.137.1.7422865
Fioravanti A et al (2004) Bobble-head doll syndrome due to a suprasellar archnoid cyst: endoscopic treatment in two cases. Childs Nerv Syst 20(10):770–773. https://doi.org/10.1007/s00381-004-0925-3
Garg RK, Singh SK, Malhotra HS, Singh MK (2012) Abnormal head movement in a patient with tuberculous meningitis. BMJ Case Reports. https://doi.org/10.1136/bcr-2012-006663
Goikhman I, Zelnik N, Peled N, Michowiz S (1998) Bobble-head doll syndrome: a surgically treatable condition manifested as a rare movement disorder. Mov Disord 13(1):192–194. https://doi.org/10.1002/mds.870130144
Hagebeuk EEO et al (2005) Bobble-head doll syndrome successfully treated with an endoscopic ventriculocystocisternostomy. J Neurosurg Pediatr 103(3):253–259. https://doi.org/10.3171/ped.2005.103.3.0253
Hahm MH et al (2018) Hypomania in bobble-head doll syndrome: a case report of surgically treated stereotypy and hypomania. Psychiatry Investig 15(5):546–549. https://doi.org/10.30773/pi.2017.10.25.2
Hottinger-Blanc PMZ et al (2002) A special type of head stereotypies in children with developmental (?cerebellar) disorder: description of 8 cases and literature review. Eur J Paediatr Neurol 6(3):143–152. https://doi.org/10.1053/ejpn.2002.0582
Ishihara M et al (2013) ‘no-No’ type bobble-head doll syndrome in an infant with an arachnoid cyst of the posterior fossa: a case report. Pediatr Neurol 49(6, Elsevier Ltd):474–476. https://doi.org/10.1016/j.pediatrneurol.2013.07.013
Ivashchuk G, Loukas M, Blount JP, Tubbs RS, Oakes WJ (2015) Chiari III malformation: a comprehensive review of this enigmatic anomaly. Childs Nerv Syst 31(11):2035–2040. https://doi.org/10.1007/s00381-015-2853-9. Epub 2015 Aug 9
Sutton LN, Bruce DA, Bruce DA, Schut L, Schut L (1980) Hydranencephaly versus maximal hydrocephalus: an important clinical distinction. Neurosurgery 6(1):35–38. https://doi.org/10.1227/00006123-198001000-00004
Malheiros JA, Trivelato FP, Oliveira MM, Gusmão S, Cochrane DD, Steinbok P (2010) Endoscopic choroid plexus cauterization versus ventriculoperitoneal shunt for hydranencephaly and near hydranencephaly: a prospective study. Neurosurgery 66(3):459–64; discussion 464. https://doi.org/10.1227/01.NEU.0000365264.99133.CA
Zimani N et al (2018) A rare case of bobble head doll syndrome. Curr Opin Neurol Sci 2(2):437–440
Olvera-Castro JO et al (2017) Bobble-head doll syndrome in an 80-year-old man, associated with a giant arachnoid cyst of the lamina quadrigemina, treated with endoscopic ventriculocystocisternotomy and cystoperitoneal shunt. Acta Neurochir 159(8):1445–1450. https://doi.org/10.1007/s00701-017-3195-z
Pařízek J, Němečková J, Šercl M (1989) Bobble-head doll syndrome associated with the III ventricular cyst. Child’s Nervous System 5(4):241–245. https://doi.org/10.1007/bf00271027
Pollack IF, Schor NF, Martinez AJ, Towbin R (1995) Bobble-head doll syndrome and drop attacks in a child with a cystic choroid plexus papilloma of the third ventricle. Case report. J Neurosurg 83(4):729–732. https://doi.org/10.3171/jns.1995.83.4.0729
Reddy OJ et al (2014) Bobble head doll syndrome: a rare case report. J Pediatr Neurosci 9(2):175. https://doi.org/10.4103/1817-1745.139350
Renne B, Rueckriegel S, Ramachandran S, Radic J, Steinbok P, Singhal A (2018) Bobble-head doll syndrome: report of 2 cases and a review of the literature, with video documentation of the clinical phenomenon. J Neurosurg Pediatr (3):236–246. https://doi.org/10.3171/2017.9.PEDS16704. Epub 2018 Jan 5
Russo RH, Kindt GW (1974) A neuroanatomical basis for the bobble-head doll syndrome. J Neurosurg 41(6):720–723. https://doi.org/10.3171/jns.1974.41.6.0720
Ramesh S, Raju S (2015) Suprasellar arachnoid cyst presenting with bobble-head doll syndrome: report of three cases. J Pediatr Neurosci 10(1):18–21. https://doi.org/10.4103/1817-1745.154321
Ure RJ et al (2016) Unusual tremor syndromes: know in order to recognise. J Neurol Neurosurg Psychiatry 87(11):1191–1203. https://doi.org/10.1136/jnnp-2015-311693
Van Beijnum J, Hanlo PW, Han KS, Ludo Van der Pol W, Verdaasdonk RM, Van Nieuwenhuizen O (2006) Navigated laser-assisted endoscopic fenestration of a suprasellar arachnoid cyst in a 2-year-old child with bobble-head doll syndrome. Case report. J Neurosurg 104(5 Suppl):348–351. https://doi.org/10.3171/ped.2006.104.5.348
Wiese JA et al (1985) Bobble-head doll syndrome: review of the pathophysiology and CSF dynamics. Pediatr Neurol 1(6):361–366. https://doi.org/10.1016/0887-8994(85)90073-6
Young RM et al (2015) The Chiari 3 malformation and a systemic review of the literature. Pediatr Neurosurg 50(5):235–242. https://doi.org/10.1159/000438487
Zamponi N et al (2005) Bobble head doll syndrome in a child with a third ventricular cyst and hydrocephalus. Childs Nerv Syst 21(5):350–354. https://doi.org/10.1007/s00381-004-1030-3
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Mba, S.E., Musara, A., Kalangu, K. et al. An unusual presentation of bobble-head doll syndrome in a patient with hydranencephaly and Chiari 3 malformation. Childs Nerv Syst 35, 879–882 (2019). https://doi.org/10.1007/s00381-019-04054-x
- Bobble-head doll
- Chiari type 3