Acta Neurochirurgica

, Volume 154, Issue 11, pp 2043–2049 | Cite as

Bobble-head doll syndrome: therapeutic outcome and long-term follow-up in four children

  • Helena GuerreiroEmail author
  • Ales Vlasak
  • Daniel Horinek
  • Michal Tichy
  • Jiri Lisy
  • Petr Vanek
  • Petr Liby
  • David Hoza
  • Vladimir Beneš
  • Christopher Nimsky
Case Report


Bobble-head doll syndrome (BHDS) is a complex syndrome with the dominant symptom of repetitive anteroposterior head movement. Only 57 patients are quoted in the literature. The etiology of this syndrome remains unknown and no standard treatment has yet been established. We hereby report four cases treated at our department. All the patients presented a psychomotor retardation due to an obstructive hydrocephalus. All the patients were treated using neuroendoscopic techniques: two with ventriculocystostomy, and two with ventriculocystocisternostomy. Cyst decompression was achieved in all four cases and clinical recovery was evident in three of the four patients observed. After surgery, BHDS persisted longer the more the subsequent treatment was delayed. In this article, we provide a concise overview of the theories of pathogenesis, presentation, and management of this syndrome. Based on our own experience, we state that the method of choice should be the neuroendoscopy and this must be performed promptly after diagnosis is made.


Bobble-head doll syndrome Suprasellar cyst Neuroendoscopy Ventriculocystostomy Ventriculocystocisternostomy 


Conflict of interest


Funding support

This project is supported by the European Regional Development Fund-Project FNUSA-ICRC (No.CZ.1.05/1.1.00/02.0123).

Supplementary material


(MPG 6604 kb)


  1. 1.
    Ahn Y, Cho BK, Wang KC (1997) Bobble-head doll syndrome associated with subduroperitoneal shunt malfunction. Childs Nerv Syst 13:234–7PubMedCrossRefGoogle Scholar
  2. 2.
    Albright L (1981) Treatment of bobble-head doll syndrome by transcallosal cystectomy. Neurosurgery 8:593–595PubMedCrossRefGoogle Scholar
  3. 3.
    Benes V, Lehovsky M, Rossler M, Koudelova A (1977) Bobble-head doll syndrome. Cs Neurol Neurochir 40:251–256Google Scholar
  4. 4.
    Benton JW, Nellhaus G, Huttenlocher PR, Ojemann RG, Dodge PR (1966) The bobble-head doll syndrome: report of a unique truncal tremor associated with third ventricular cyst and hydrocephalus in children. J Neurol 16:725–729Google Scholar
  5. 5.
    Bhattacharya KB, Senapati A, Basu S, Bhattacharya S, Ghosh S (2003) Bobble-head doll syndrome: some atypical features with a new lesion and review of literature. Acta Neurol Scand 108:216–220PubMedCrossRefGoogle Scholar
  6. 6.
    Coker SB (1986) Bobble-head doll syndrome due to trapped fourth ventricle and aqueduct. Pediatr Neurol 2:115–116PubMedCrossRefGoogle Scholar
  7. 7.
    Decq P, Brugieres P, Le Guerinel C, Djindjian M, Keravel Y, Nguyen JP (1996) Percutaneous endoscopic treatment of suprasellar arachnoid cysts: ventriculocystostomy or ventriculocystocisternostomy? Technical note. J Neurosurg 84:696–701PubMedCrossRefGoogle Scholar
  8. 8.
    Gangemi M, Maiuri F, Donati P, Carandente M (1988) Hydrocephalus and bobble-head doll syndrome: a case report. Acta Neurol 10(2):143–7Google Scholar
  9. 9.
    Hottinger-Blanc PM, Ziegler AL, Deonna T (2002) A special type of head stereotypies in children with developmental (?cerebellar) disorder: description of 8 cases and literature review. Eur J Paediatr Neurol 6:143–52PubMedCrossRefGoogle Scholar
  10. 10.
    Mayher WE, Gindin RA (1970) Head bobbing associated with third ventricular cyst. Report of a case. Arch Neurol 23:274–7PubMedCrossRefGoogle Scholar
  11. 11.
    Miyajima M, Arai H, Okuda O, Hishii M, Nakanishi H, Sato K (2000) Possible origin of suprasellar arachnoid cysts: neuroimaging and neurosurgical observations in nine cases. J Neurosurg 93:62–7PubMedCrossRefGoogle Scholar
  12. 12.
    Mussell HG, Dure LS, Percy AK, Grabb PS (1977) Bobble-head doll syndrome: report of a case and review of literature. Mov Disord 12:810–814CrossRefGoogle Scholar
  13. 13.
    Obenchain TG, Becker DP (1972) Head bobbing associated with a cyst of the third ventricle. Case report. J Neurosurg 37:457–9PubMedCrossRefGoogle Scholar
  14. 14.
    Parizek J, Nemeckova J, Sercl M (1989) Bobble-head doll syndrome associated with the III ventricular cyst. Three cases in children 7 years after CVP or CVA shunting. Childs Nerv Syst 5:241–5PubMedCrossRefGoogle Scholar
  15. 15.
    Patriquin HB (1973) The bobble-head doll syndrome. A curable entity. Radiology 107:171–2PubMedGoogle Scholar
  16. 16.
    Pollack IF, Schor NF, Martinez AJ, Towbin R (1995) Bobble-head doll syndrome and drop attacks in a child with cystic choroid plexus papilloma of the third ventricle. J Neurosurg 83:729–732PubMedCrossRefGoogle Scholar
  17. 17.
    Russo RH, Kindt GW (1974) A neuroanatomical basis for the bobble-head doll syndrome. J Neurosurg 41:720–723PubMedCrossRefGoogle Scholar
  18. 18.
    Santamarta D, Aguas J, Ferrer E (1995) The natural history of arachnoid cysts: endoscopic and cine-mode MRI evidence of a slit-valve mechanism. Minim Invasive Neurosurg 38:133–7PubMedCrossRefGoogle Scholar
  19. 19.
    Schroeder HW, Gaab MR (1997) Endoscopic observation of a slit-valve mechanism in a suprasellar prepontine arachnoid cyst: case report. Neurosurgery 40:198–200PubMedGoogle Scholar
  20. 20.
    Sharma RR, Pawar SJ, Devadas RV, Dev EJ (2001) CT stereotaxy guided lateral trans-cerebellar programmable fourthventriculo-peritoneal shunting for symptomatic trapped fourth ventricle. Clin Neurol Neurosurg 103:143–6PubMedCrossRefGoogle Scholar
  21. 21.
    Wiese JA, Gentry LR, Menezes AH (1985) Bobble-head doll syndrome: review of the pathophysiology and CSF dynamics. Pediatr Neurol 1:361–6PubMedCrossRefGoogle Scholar
  22. 22.
    Zamponi N, Rychlicki F, Trignani R, Polonara G, Ruggiero M, Cesaroni E (2005) Bobble-head doll syndrome in a child with a third ventricular cyst and hydrocephalus. Childs Nerv Syst 21:350–4PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Helena Guerreiro
    • 1
    • 2
    • 6
    Email author
  • Ales Vlasak
    • 2
  • Daniel Horinek
    • 1
    • 3
    • 4
  • Michal Tichy
    • 2
  • Jiri Lisy
    • 5
  • Petr Vanek
    • 4
  • Petr Liby
    • 2
  • David Hoza
    • 1
  • Vladimir Beneš
    • 4
  • Christopher Nimsky
    • 3
  1. 1.International Clinical Research CenterSt. Annes´s University Hospital BrnoBrnoCzech Republic
  2. 2.Department of NeurosurgeryUniversity Hospital MotolPragueCzech Republic
  3. 3.Department of Neurosurgery, Medical FacultyPhilipps UniversityMarburgGermany
  4. 4.Department of Neurosurgery of 1st Medical FacultyCharles University, Central Military HospitalPragueCzech Republic
  5. 5.Department of Radiology of 2nd Faculty of MedicineCharles UniversityPragueCzech Republic
  6. 6.International Clinical Research CenterSt. Annes´s University Hospital BrnoBrnoCzech Republic

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