Bobble-Head Doll Syndrome in Suprasellar Cysts: Results of Neuroendoscopic Treatment in Four Children

Authors: D. Hořínek 1,4;  J. Schwab 1;  M. Tichý 1;  M. Kynčl 2;  J. Šanda 2;  L. Paulas 3;  M. Vaculík 1;  Z. Novák 5
Authors‘ workplace: Oddělení dětské neurochirurgie FN v Motole, Praha 1;  Klinika zobrazovacích metod 2. LF UK a FN Motol, Praha 2;  Klinika dětské neurologie 2. LF UK a FN Motol, Praha 3;  Ústav patologické fyziologie 2. LF UK, Praha 4;  Neurochirurgická klinika, Fakultní nemocnice u sv. Anny, Brno 5
Published in: Cesk Slov Neurol N 2007; 70/103(1): 88-93
Category: Case Report

Práce byla podpořena granty Ministerstva zdravotnictví ČR IGA ND/7678-3 a IGA NR/7805-2 a VZ 0064203-6308.


The authors have presented 4 case-reports of children with suprasellar cysts and bobble-head doll syndrome. Obstructive hydrocephalus developed in all the cases given, and severe transient visual disturbance rose in one case. Neuroendoscopic ventriculocystostomy was chosen as a method of therapy in two cases, two other cases underwent neuroendoscopic ventriculocystocisternostomy. A cystoperitoneal shunt was made under neuroendoscopic control in one patient after performing ventriculocystostomy.

The therapy resulted in the cyst decompression in all the patients, clinical symptoms disappeared in 3 cases. Bobble-head doll syndrome is persisting in a boy with a four-year´s delay in diagnosis and therapy.

Bobble-head doll syndrome in suprasellar cysts can be treated successfully using neuroendoscopic ventriculocystostomy or ventriculocystocisternostomy. Bobble-head doll syndrome as a rare syndromological entity has been usually recognized with delay.

bobble-head doll syndrome, suprasellar cysts, neuroendoscopy, diagnostic delay


1. Benton JW, Nellhaus G, Huttenlocher PR, Ojemann RG, Dodge PR. The bobble head doll syndrome: report of a unique truncal tremor associated with third ventricular cyst and hydrocephalus in children. Neurology 1966; 16: 725-9.

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

3. Pollack IF, Schor NF, Martinez AJ, Towbin R. Bobble-head doll syndrome and drop attacks in a child with cystic choroid plexus papilloma of the third ventricle. J Neurosurg 1995; 83: 729-32.

4. Dell S. Further observations on “Bobble Head Doll syndrome“. J Neurol Neurosurg Psychiat 1981; 44: 1046-9.

5. Coker SB. Bobble head doll syndrome due to trapped fourth ventricle and aqueduct. Pediatr Neurol 1986; 2: 115-6.

6. Sharma RR, Pawar SJ, Devadas RV, Dev EJ. CT stereotaxy guided lateral trans-cerebellar programmable fourthventriculo-peritoneal shunting for symptomatic trapped fourth ventricle. Clin Neurol Neurosurg. 2001; 103: 143-6.

7. Bhattacharya KB, Senapati A, Basu S, Bhattacharya S, Ghosh S. Bobblehead doll syndrome: some atypical features with a new lesion and review of literature. Acta Neurol Scand 2003; 108: 216-20.

8. Hottinger-Blanc PM, Ziegler AL, Deonna T. A special type of head stereotypies in children with developmental (cerebellar) disorder: description of 8 cases and literature review. Eur J Paediatr Neurol 2002; 6: 143-52.

9. Decq P, Brugieres P, Le Guerinel C, Djindjian M, Keravel Y, Nguyen JP. Percutaneous endoscopic treatment of suprasellar arachnoid cysts: ventriculocystostomy or ventriculocystocisternostomy? Technical note. J Neurosurg 1996; 84: 696-701.

10. Oberbauer RW, Haase J, Pucher R. Arachnoid cysts in children: a European co-operative study. Childs Nerv Syst 1992; 8: 281-6.

11. Pierre-Kahn A, Capelle L, Brauner R, Sainte-Rose C, Renier D, Rappaport R, et al. Presentation und management of suprasellar arachnoid cysts:Rewiew of 20 cases. J Neurosurg 1990; 73: 355-9.

12. Jakubec J, Zadrobílek K, Pařízek J, Hobza V, Šercl M, Jakubcová O. Syndrom kývavé panenky provázející supraselární cysty III.komory. In II.sympozium praktické neurologie; 2005, Abstrakta 1.-2.6: l3.

13. Starkman SP, Brown TC, Linell EA. Cerebral arachnoid cysts. J Neuropathol Exp Neurol 1958; 17: 484-500.

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

15. Patriquin HB. The bobble-head doll syndrome. A curable entity. Radiology 1973; 107: 171-2.

16. Beneš V, Lehovský M, Rössler M, Koudelová A. Bobble-head doll syndrome. Čes Slov Neurol Neurochir 1977; 40: 251-6.

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

18. Desai KI, Nadkarni TD, Muzumdar D, Goel A. Suprasellar arachnoid cyst presenting with bobble-head doll movements: A report of 3 cases . Neurol India 2003; 51: 407-9

19. Zamponi N, Rychlicki F, Trignani R, Polonara G, Ruggiero M, Cesaroni E. Bobble head doll syndrome in a child with a third ventricular cyst and hydrocephalus. Childs Nerv Syst 2005; 21: 350-4.

20. Mussell HG, Dure LS, Percy AK, Grabb PS. Bobble head doll syndrome: report of a case and review of literature. Mov Disord 1997; 12: 810-14.

21. Albright L. Treatment of Bobble-Head Doll Syndrome by Transcallosal Cystectomy. Neurosurgery 1981; 8: 593-5.

22. Parizek J, Nemeckova J, Sercl M. Bobble-head doll syndrome associated with the III ventricular cyst. Three cases in children 7 years after CVP or CVA shunting. Childs Nerv Syst 1989; 5: 241-5.

23. Russo RH, Kindt GW. A neuroanatomical basis for the bobble head doll syndrome. J Neurosurg 1974; 41: 720-3.

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

25. Santamarta D, Aguas J, Ferrer E. The natural history of arachnoid cysts: endoscopic and cine-mode MRI evidence of a slit-valve mechanism. Minim Invasive Neurosurg 1995; 38: 133-7.

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

27. Miyajima M, Arai H, Okuda O, Hishii M, Nakanishi H, Sato K. Possible origin of suprasellar arachnoid cysts: neuroimaging and neurosurgical observations in nine cases. J Neurosurg 2000; 93: 62-7.

28. Wang JC, Heier L, Souweidane MM. Advances in the endoscopic management of supraselar arachnoid cysts in children. J Neurosurg 2004; 100: 418-26.

Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 1

2007 Issue 1

Most read in this issue
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account