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Neuropediatrics 2022; 53(01): 078-079
DOI: 10.1055/s-0041-1733775
Videos and Images in Neuropediatrics

Obstructive Hydrocephalus Presenting with Bobble-Head Doll Syndrome

Igor de Assis Franco
1   Department of Neurology, Hospital e Maternidade São José, Conselheiro Lafaiete, Minas Gerais, Brazil
,
Thiago Cardoso Vale
2   Departament of Internal Medicine, Faculdade de Medicina, Universidade Federal de Juiz de Fora (UFJF), Juiz de Fora, Minas Gerais, Brazil
,
Leonardo Furtado Freitas
3   Department of Neuroradiology, Beneficência Portuguesa de São Paulo, São Paulo, Brazil
,
André Vinícius Soares Barbosa
4   Department of Child Neurology HIJP FHEMIG, Belo Horizonte, Brazil
,
Marcelo de Melo Aragão
5   Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, Sao Paulo, Brazil
,
Marcelo Masruha Rodrigues
5   Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, Sao Paulo, Brazil
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A previously healthy macrocephalic 3-year-old boy presented with a 3-month-history of progressive up-and-down head movements that were increased with distraction, decreased with concentration, and voluntarily suppressed ([Video 1], Segment 1). Brain MRI showed suprasellar cystic lesion causing obstructive hydrocephalus ([Fig. 1]). He was successfully treated with endoscopic third ventriculostomy and movements abated ([Video 1], Segment 2). Bobble-head doll syndrome is a rare and surgically treatable movement disorder usually associated with dilation of the third ventricle and characterized by the up-and-down, two to three second cycles of bobbling movements of the head and neck.[1] [2]

Video 1 Segment one shows the child displaying movements typical of the bobble-head doll syndrome. At the beginning of the video the patient is able to control the movements. Then, when relaxed and talking to the examiner, the movements appear. Segment two shows a 1-year follow-up after the surgical procedure with total resolution of movements.


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Fig. 1 Brain MRI post-gadolinium sagittal T1-weighted and 3D-CISS (A and B), coronal and axial T2-weighted (C, D and E) and Axial FLAIR (F) sequences. Giant supracellular arachnoid cyst (red asterisk) displacing the pituitary stalk, elevating the floor of the third ventricle and obliterating the Monro foramina with consequent accentuated supratentorial obstructive ventriculomegaly. Tortuous optic nerves with prominence of the perioptic liquor (yellow arrows) with rectification of the posterior wall of the eyeballs suggesting intracranial hypertension.

Note

This work was performed at the Hospital e Maternidade São José in Conselheiro Lafaiete, MG, Brazil.


Ethical Statement

Full consent was obtained from the patient for the case report publication.


Financial Disclosure

None.


Authors' Contribution

IAF, TCV, FLF, AVSB, worked on case report project and imaging's conception, organization, execution.


M.M.A. and M.R.M. worked on conception, organization and execution of imaging. They wrote the manuscript and worked as reviewer and critique.




Publikationsverlauf

Eingereicht: 08. März 2021

Angenommen: 24. Juni 2021

Artikel online veröffentlicht:
26. August 2021

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  • References

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