Bobble-head doll syndrome and Suprasellar Arachnoid cyst: A rare experience and systematic literature review.

Abstract

Background: Bobble-head doll syndrome (BHDS) is a rare movement disorder characterized by continuous or periodic head movements. The head nodding or bobbing occurs either forward and backward (yes-yes) or side-to-side movement (no-no) direction at a 2-3 Hz frequency. It is due to lesions in and around the third ventricle, causing pressure on periventricular neuronal structures (thalami). The most common lesions are suprasellar arachnoid cysts. We intend to analyze the clinical presentation, imaging findings, treatment options, and surgical outcome of BHDS in suprasellar arachnoid cysts.

Methods: We retrospectively reviewed our database for BHDS in suprasellar arachnoid cysts surgically treated between 2016-2023. Patient records were reviewed with special attention to patient demographics, presentation, imaging findings, site and extent, surgery performed, complications, and outcomes.

Results: A total of 5 patients with a mean age of 6.7 years were included in the study. Abnormal head movements were the most common presenting complaint. The mean duration of symptoms was 2.9 ± 2 years. All patients underwent cyst fenestration (endoscopic or microscopic assisted). Approaches used: craniotomy and microscopic-assisted cyst fenestration in 1 patient (20%) and endoscopic-assisted cyst fenestration in 4 patients (80%). One patient had partial resolution of symptoms at 6 weeks' follow-up. The remaining four patients (80%) had complete resolution of symptoms at 3-month follow-up.

Conclusion: BHDS is a rare movement disorder caused by lesions in and around the third ventricle. Neuroimaging can help in diagnosing this lesion. The most common clinical presentations are involuntary head movements related to the dilatation of the third ventricle and obstructive hydrocephalus. It is a surgical condition with a favourable outcome. Various treatment options are described in the literature. Endoscopic fenestration is the optimal and most commonly performed treatment option. The excellent outcome in BHDS with suprasellar arachnoid cysts can be achieved using endoscopic fenestration.