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

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.

• Authors: Nelia Zamponi, Franco Rychlicki, Roberto Trignani, Gabriele Polonara, Maria Ruggiero, Elisabetta Cesaroni
• Journal: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery (Childs Nerv Syst) Vol. 21 Issue 5 Pg. 350-4 (May 2005) ISSN: 0256-7040 [Print] Germany
• PMID: 15449088 (Publication Type: Case Reports, Comparative Study, Journal Article)

Topics

• Child, Preschool
• Cysts (complications, pathology, surgery)
• Humans
• Hydrocephalus (complications, pathology, surgery)
• Magnetic Resonance Imaging (methods)
• Male
• Movement Disorders (complications, pathology, surgery)
• Review Literature as Topic
• Third Ventricle (pathology, surgery)