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Bobble head doll syndrome in a child with a third ventricular cyst and hydrocephalus

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Abstract

Introduction

The bobble head doll syndrome, mainly affecting children under 10 years of age, is a rare and surgically treatable movement disorder characterized by head bobbing occurring at a rate of 2–3 times/s. Its pathophysiological mechanism is not well known but two main factors are commonly associated with the condition: a dilatation of the third ventricle and, more frequently, a cystic lesion rather than a solid mass in the region of the third ventricle.

Illustrative case

The illustrative case concerns a child with a third ventricular cystic lesion and hydrocephalus who had experienced abnormal head movements since the age of 1 year as well as ataxia and tremor of the arms. Contrast cranial MRI, at the age of 3, demonstrated enlargement of the third and lateral ventricles, a ballooned cyst inside the third ventricle with compression of all the diencephalic structures, a funnel dilation of the cranial part of the aqueduct, and a cyst in the septum pellucidum. A ventriculoperitoneal shunt (Hakim-Cordis) was placed and the head bobbing, tremor of the arms, and ataxia disappeared immediately.

Conclusion

The good and immediate clinical result in our case emphasizes the opinion that the reduction of CSF pressure is the best therapeutical option in the bobble head doll syndrome.

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Correspondence to Nelia Zamponi.

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Zamponi, N., Rychlicki, F., Trignani, R. et al. Bobble head doll syndrome in a child with a third ventricular cyst and hydrocephalus. Childs Nerv Syst 21, 350–354 (2005). https://doi.org/10.1007/s00381-004-1030-3

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  • DOI: https://doi.org/10.1007/s00381-004-1030-3

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