Bobble-head doll syndrome: report of 2 cases and a review of the literature, with video documentation of the clinical phenomenon

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Bobble-head doll syndrome (BHDS) is a rare pediatric movement disorder presenting with involuntary 2- to 3-Hz head movements. Common signs and symptoms also found on presentation include macrocephaly, ataxia, developmental delay, optic disc pallor or atrophy, hyperreflexia, tremor, obesity, endocrinopathy, visual disturbance or impairment, headache, and vomiting, among others. The syndrome is associated with suprasellar cysts, third ventricular cysts, or aqueductal obstruction, along with a few other less common conditions. The cause of involuntary head motions is not understood. Treatment is surgical. The authors present 2 cases of BHDS. The first is a 14-year-old boy with BHDS associated with aqueductal obstruction and triventricular hydrocephalus secondary to a tectal tumor. He was successfully treated by endoscopic third ventriculostomy, and all symptoms resolved immediately in the recovery room. This case is unusual in its late age of symptom onset, the primacy of lateral (“no-no”) involuntary head rotations, and the associated tectal tumor. The second case is a 7.5-year-old girl with BHDS associated with a suprasellar cyst. She was successfully treated with an endoscopic fenestration but preexisting endocrinopathy persisted, and the patient was diagnosed with autism spectrum disorder at age 12 years. This second case is more typical of BHDS. A comprehensive and up-to-date review of the literature of BHDS and video documentation of the phenomenon are presented.

ABBREVIATIONS BHDS = bobble-head doll syndrome; CVP = cystoventriculoperitoneal; ETV = endoscopic third ventriculostomy; VP = ventriculoperitoneal.

Article Information

Correspondence Bryan Renne: BC Children’s Hospital, Vancouver, BC, Canada. bryan@renne.org.

INCLUDE WHEN CITING Published online January 5, 2018; DOI: 10.3171/2017.9.PEDS16704.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

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    Case 1. MR images obtained in a 14-year-old boy with BHDS associated with a tectal mass (white and black arrows). A: Presurgical axial T2-weighted image showing obstructive triventriculomegaly. B: Post-ETV axial T2-weighted image showing reduced size of the third and lateral ventricles. C: Presurgical sagittal turbo inversion recovery magnitude image showing aqueductal obstruction by tectal tumor causing triventricular dilation, bowing of the lamina terminalis and the floor of the third ventricle, and sellar erosion. D: Post-ETV sagittal magnetization-prepared rapid gradient-echo image showing a relaxed floor of the third ventricle. Post-ETV images were taken 9 months after the presurgical images and showed that the tectal mass increased in size to 19 × 25 × 24 mm3 (craniocaudal × anteroposterior × right-left) from a presurgical size of 19 × 20 × 20 mm3.

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    Case 2. MR images acquired in a 7.5-year-old girl with BHDS associated with suprasellar cyst. Presurgical axial T2-weighted image (A) and presurgical sagittal constructive interference in steady-state (CISS) image (C) showing a large suprasellar cyst. Post-fenestration axial T2-weighted image (B) and post-fenestration CISS image (D) showing reduced size of the cyst.

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