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

Czech version

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.

Overview

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.

Key-words:
bobble-head doll syndrome, suprasellar cysts, neuroendoscopy, diagnostic delay

Sources

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.