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
