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An unusual presentation of bobble-head doll syndrome in a patient with hydranencephaly and Chiari 3 malformation

  • Serge Eddy MbaEmail author
  • Aaron Musara
  • Kazadi Kalangu
  • Brighton Nyamapfene
Case Report
  • 9 Downloads

Abstract

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.

Keywords

Bobble-head doll Hydranencephaly Chiari type 3 

Notes

Compliance with ethical standards

The authors declare that the study complies with the current law in Zimbabwe.

Disclaimer

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.

Supplementary material

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Surgery, Division of Neurosurgery, College of Health SciencesUniversity of ZimbabweHarareZimbabwe

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