Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature

Bobble-head doll syndrome is a rare and unique movement disorder encountered in children. It is characterized by continuous or episodic involuntary forward and backward and side to side movement of the head at the frequency of 2-3 Hz. Neuroimaging in most of the cases reveals third ventricular tumors, suprasellar arachnoid cysts, aqueductal stenosis and other lesions in the region of the third ventricle along with communicating hydrocephalus. In most of the circumstances, the problem starts in the first decade of life and diversion of cerebrospinal fluid by shunt operation is very often accompanied by dramatic improvement. We report one case where bobbing of the head started at around 12 years of age. Additionally, there was evidence of partial left abducens nerve palsy, tremor in the outstretched hands, difficulty in finger-nose test and tandem walking, hyperreflexia and extensor plantar response. He was unconscious on two occasions and there was evidence of gross hydrocephalus along with a thin membranous web, running transversely across the lower part of the aqueduct of Sylvius without any cerebrospinal fluid flow void. Ventriculo-peritoneal shunt abolished the abnormal movements. We propose that the aqueductal web was the offending agent for the pathogenesis of bobble-head doll syndrome in our case and this lesion has not been identified in the cases reported so far. Relevant literature in this regard has also been reviewed.