Shaking the head without end – bobble-head doll syndrome in no-no direction

Aims: The bobble-head doll syndrome (BHDS) is a rare movement disorder that is usually characterized by a back-and-forth movement of the head or less frequently by a no-no movement. In most cases, the syndrome is associated with a third ventricular cyst possibly causing dysfunction of surrounding cerebral and cerebellar structures. We report about a today six year old girl with BHDS.

Methods: The girl was a preterm of the 34 gestational week with a birth weight of 1792g. Neonatal asphyxia required mechanical ventilation. She was presented in the neuropediatric department at the age of three years for the first time because of developmental retardation and stereotyped movements of the head.

Results: The repetitive movements were in no-no direction and without external goal-direction or obvious internal intention. Cerebral MRI revealed an intracerebellar arachnoid cyst (extension 7.5×2.3×3.3cm) and a hypoplasia of the cerebellar vermis appearing to be a dandy walker variant. A BHDS was diagnosed. Control MRI scans showed no change of the findings. At the age of six years she was mildly retarded with mainly language development and fine motor skills' impairment. Recent video analysis demonstrated persistent movement of the head in no-no direction while sitting, standing, walking and running, rarely while lying. Frequency was mostly 1–2/s. The symptoms increased in stressful situations whereas they decreased in concentration (e.g. looking at a book). It was more and more a strain that the no-no direction of the movement was interpreted as a negative communication by others causing misunderstandings. Therefore navigated trepanation with ventriculoscopy, pressure measurement and optional fenestration is planned.

Conclusion: Reports of successful treatment of the BHDS with endoscopic ventriculocystosternostomy suggest an influence of decreasing intracranial pressure. However; some authors propose that cerebellar malformations per se can cause BHDS. Psychosocial factors should contribute to the indication of intervention.

ventriculocystosternotomy - Dandy–Walker variant - archnoid cyst