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

Abstract

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.

An inherited episodic head tremor syndrome in Doberman pinscher dogs

“…Brain images of 5 DPs that we evaluated did not reveal any relevant cystic lesions, hydrocephalus, or syringomyelia, but intracranial pressure and CSF flow were not measured. Head bobbing similar to the one in humans has been described in birds 16…”

“…In contrast to episodic HT, cerebellar tremor is an action (intention) tremor of the head that is typically evident when dogs are moving or performing goal‐directed activities; it is not observed when dogs are relaxed 12. Continuous or episodic vertical or horizontal head bobbing in children (referred to as “bobble head doll syndrome”) was found to be associated with the presence of a third ventricular cyst and intermittent occlusion of the foramen of Monroe and/or aquaeduct 13–15. Brain images of 5 DPs that we evaluated did not reveal any relevant cystic lesions, hydrocephalus, or syringomyelia, but intracranial pressure and CSF flow were not measured.…”

Intermittent head drops: the differential spectrum

“…Bobble-head doll syndrome is, also, a rare but characteristic disorder described in children 33 34. It is characterised by episodic, involuntary head movements in flexion, extension or, less frequently, from side-to-side, which occur at a frequency of 2–3 Hz.…”

Abnormal head movement in a patient with tuberculous meningitis

“…It is believed that bobble-head doll syndrome is due to the pressure effect of dilated third ventricle on the dorsomedial nucleus of the thalamus, red nucleus and dentatorubrothalamic pathways. Distortion of the third ventricle is thought to impair the functions of these structures 5 6. An evaluation of the cerebrospinal fluid dynamics with CT cisternography also revealed that the head movements were apparently because of intermittent obstruction at the level of foramina of Monro.…”

“…This syndrome was first described by Benton et al 4 in a child who had hydrocephalus due to third ventricular cysts. Commonly described causes of this syndrome include third ventricular tumours, suprasellar arachnoid cysts, aqueductal stenosis and other lesions in the region of the third ventricle along with communicating hydrocephalus 5. We are reporting an interesting case of bobble-head doll syndrome in patients with tuberculous meningitis following external ventricular drainage.…”