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

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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.

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Authors and Affiliations

  1. Department of Surgery, Division of Neurosurgery, College of Health Sciences, University of Zimbabwe, P.O Box A178, Avondale, Harare, Zimbabwe

    Serge Eddy Mba, Aaron Musara, Kazadi Kalangu & Brighton Nyamapfene

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  1. Serge Eddy Mba
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  2. Aaron Musara
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  3. Kazadi Kalangu
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  4. Brighton Nyamapfene
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Correspondence to Serge Eddy Mba.

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Mba, S.E., Musara, A., Kalangu, K. et al. An unusual presentation of bobble-head doll syndrome in a patient with hydranencephaly and Chiari 3 malformation. Childs Nerv Syst 35, 879–882 (2019). https://doi.org/10.1007/s00381-019-04054-x

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