An unusual presentation of bobble-head doll syndrome in a patient with hydranencephaly and Chiari 3 malformation
- 65 Downloads
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.
KeywordsBobble-head doll Hydranencephaly Chiari type 3
Compliance with ethical standards
The authors declare that the study complies with the current law in Zimbabwe.
The authors declare that this manuscript has not been published elsewhere and is not under consideration by another journal.
Conflict of interest
The authors declare that they have no conflict of interest.
(MP4 55,699 kb)
- 6.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
- 8.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
- 13.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 CrossRefPubMedGoogle Scholar
- 14.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
- 16.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
- 17.Zimani N et al (2018) A rare case of bobble head doll syndrome. Curr Opin Neurol Sci 2(2):437–440Google Scholar
- 18.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 CrossRefPubMedGoogle Scholar
- 19.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
- 22.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
- 26.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