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

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