Bobble-Head Doll Syndrome: Understanding a Rare Neurological Enigma

Abstract

Bobble-Head Doll Syndrome (BHDS) is a rare neurological disorder characterized by repetitive, involuntary, and stereotypical head movements, resembling the motion of a bobble-head doll, often accompanied by various neurological and endocrinological symptoms. Although primarily observed in pediatric patients, BobbleHead Doll Syndrome can also manifest in adults. The pathophysiology remains unclear, but it is often associated with suprasellar cysts, third ventricle cysts, or aqueductal stenosis. Diagnosis typically involves magnetic resonance imaging to visualize the cystic lesions and assess cerebrospinal fluid flow. Additional diagnostic modalities include computed tomography and electromyography. Treatment primarily involves neurosurgical intervention, such as ventriculocystostomy or ventriculocystocisternostomy, aimed at relieving cerebrospinal fluid obstruction. Despite the challenges in diagnosis and management, early recognition and appropriate treatment offer promising outcomes, including symptom resolution and potential recovery. This review provides a comprehensive overview of Bobble-Head Doll Syndrome, including its clinical presentation, pathogenesis, diagnostic approach, and therapeutic strategies, highlighting the need for further research to elucidate its underlying mechanisms and optimize therapeutic strategies.

Keywords

• children
• neuroimaging
• neurological disorder