BOBBLE-HEAD DOLL SYNDROME AND DROP ATTACKS IN A CHILD WITH A CYSTIC CHOROID-PLEXUS PAPILLOMA OF THE 3RD-VENTRICLE - CASE-REPORT

The authors report an unusual case of a 2-year-old boy with a 3-month history of episodic rightward anterolateral head tilt and large-amplit ude positional anteroposterior head bobbing reminiscent of bobble-head doll syndrome. This child experienced a sudden onset of drop attacks and then, within several hours, deep coma. The causative lesion was a contrast-enhancing, partially cystic third ventricular mass, which ult imately obstructed the aqueduct,producing profound obstructive hydroce phalus. An emergency ventriculostomy and endoscopic fenestration of th e septum pellucidum was performed. Four days later, the tumor was comp letely resected by a transcallosal-transforaminal approach. The lesion was freely mobile within the third ventricle and contained a large cy st within its posterior pole; following drainage of the cyst, the lesi on was easily delivered through the foramen of Monro. The histopatholo gical diagnosis was choroid plexus papilloma. The child's neurological deficits, head tilt, and head bobbing resolved immediately after oper ation. To the best of the authors' knowledge, this represents the firs t well-documented report of bobble-head doll syndrome and drop attacks secondary to a choroid plexus papilloma. The highly mobile nature of the cystic lesion presumably led to its intermittent impaction within the foramen of Monro and/or proximal aqueduct; this produced the inter mittent head tilt and bobble-head symptoms and, ultimately, resulted i n acute obstruction of the aqueduct, causing Be child's precipitous ne urological decline.