A case of Bobblehead-Doll Syndrome: NEJM
A 5-year-old girl presented to the pediatric neurosurgery clinic with a 2-year history of excessive head nodding. On examination, she was alert, with normal cognitive function. She displayed continuous, rhythmic, anteroposterior head-bobbing movements at a frequency of 2 to 3 Hz. The movements diminished in intensity when she engaged in volitional activity, such as talking (see video).

MRI head revealed a well-defined, thin-walled, suprasellar cystic lesion (Panel A, arrow). The lesion caused obstruction at the foramina of Monro or at the third ventricle, with resulting ventriculomegaly (Panel B).

A diagnosis of a suprasellar arachnoid cyst with bobblehead-doll syndrome was made.

The patient underwent endoscopic cystoventriculostomy and cystocisternostomy for the suprasellar arachnoid cyst. At follow-up 6 weeks after the procedure, she had partial resolution of her symptoms, with a reduction in both the frequency and intensity of head movements.

Clinical pearls:-
- This is a rare pediatric movement disorder characterized by continuous or episodic involuntary head nodding at a frequency of 2 to 3 Hz.

- The movements stop during sleep and may disappear or attenuate with volitional activity.

- The syndrome is associated with cystic abnormalities in the region of the anterior third ventricle.

Source: https://www.nejm.org/doi/full/10.1056/NEJMicm1808747
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