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

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Abstract

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.

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References

  1. 1.

    Ahn Y, Cho BK, Wang KC (1997) Bobble-head doll syndrome associated with subduroperitoneal shunt malfunction. Childs Nerv Syst 13:234–7

  2. 2.

    Albright L (1981) Treatment of bobble-head doll syndrome by transcallosal cystectomy. Neurosurgery 8:593–595

  3. 3.

    Benes V, Lehovsky M, Rossler M, Koudelova A (1977) Bobble-head doll syndrome. Cs Neurol Neurochir 40:251–256

  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 16:725–729

  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 108:216–220

  6. 6.

    Coker SB (1986) Bobble-head doll syndrome due to trapped fourth ventricle and aqueduct. Pediatr Neurol 2:115–116

  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 84:696–701

  8. 8.

    Gangemi M, Maiuri F, Donati P, Carandente M (1988) Hydrocephalus and bobble-head doll syndrome: a case report. Acta Neurol 10(2):143–7

  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 6:143–52

  10. 10.

    Mayher WE, Gindin RA (1970) Head bobbing associated with third ventricular cyst. Report of a case. Arch Neurol 23:274–7

  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 93:62–7

  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 12:810–814

  13. 13.

    Obenchain TG, Becker DP (1972) Head bobbing associated with a cyst of the third ventricle. Case report. J Neurosurg 37:457–9

  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 5:241–5

  15. 15.

    Patriquin HB (1973) The bobble-head doll syndrome. A curable entity. Radiology 107:171–2

  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 83:729–732

  17. 17.

    Russo RH, Kindt GW (1974) A neuroanatomical basis for the bobble-head doll syndrome. J Neurosurg 41:720–723

  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 38:133–7

  19. 19.

    Schroeder HW, Gaab MR (1997) Endoscopic observation of a slit-valve mechanism in a suprasellar prepontine arachnoid cyst: case report. Neurosurgery 40:198–200

  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 103:143–6

  21. 21.

    Wiese JA, Gentry LR, Menezes AH (1985) Bobble-head doll syndrome: review of the pathophysiology and CSF dynamics. Pediatr Neurol 1:361–6

  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 21:350–4

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Conflict of interest

None.

Funding support

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

Author information

Correspondence to Helena Guerreiro.

Additional information

A portion of this work was presented in the abstract form in June (19th to 24th), 2005, during the XIII International Congress of Neurosurgery, Marrakech.

Electronic supplementary material

In this recording, we can clearly visualize an arachnoid membrane valve formed around the basilar artery that opens and closes simultaneously with the arterial pulsations. Due to the anticipated one-way direction of the valve flow, the cyst is filled with CSF, which cannot run out from it. This leads to the cyst enlargement.

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Guerreiro, H., Vlasak, A., Horinek, D. et al. Bobble-head doll syndrome: therapeutic outcome and long-term follow-up in four children. Acta Neurochir 154, 2043–2049 (2012). https://doi.org/10.1007/s00701-012-1458-2

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Keywords

  • Bobble-head doll syndrome
  • Suprasellar cyst
  • Neuroendoscopy
  • Ventriculocystostomy
  • Ventriculocystocisternostomy