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A still image from the video showing bobble-head doll syndrome prior to ventriculoperitoneal shunt insertion (video can be found in the supplementary data of this article)
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![Discharge pathway from the dorsomedial thalamic nucleus (adapted from [23])](publication/330919307/figure/fig4/AS:960094653128704@1605915811833/Discharge-pathway-from-the-dorsomedial-thalamic-nucleus-adapted-from-23_Q320.jpg)
Bobble-head doll syndrome is a rare movement disorder that is usually associated with lesions involving the third ventricle. It is characterised by stereotypical rhythmic up-and-down or side-to-side head movements. The pathophysiology and anatomical basis for this unusual manifestation is still a subject of intense scrutiny. The syndrome has never...
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Purpose:
Hydranencephaly is a rare condition that occurs during embryogenesis after neurogenesis and is characterized by the near complete absence of the cerebral hemispheres. In general, patients with hydranencephaly have been considered to have a markedly reduced life expectancy. We present 4 patients with hydranencephaly who have survived for o...
Citations
... These movements are rhythmic, may intensify during stress, but are partially controllable by the patient and disappear during sleep [11]. At least 81 other cases have already been described before our cases [2,3,5,8,14,15,17,20] which have a particular clinical character due to the predominance of ‛‛non-non laterality movements''. Since the first description in 1966 [1], the consistency of intracerebral cystic abnormalities has been noticed but the direct causal link remains for the moment subject to controversies. ...
Introduction. Bobble head doll syndrome is a neurological syndrome characterized by abnormal involuntary movements of the head combining repetitive or episodic movements of 2 to 3 Hz back and forth in the anterior-posterior direction that can be assimilated to approval movements (yes-yes) and occasionally lateral rotations that can be assimilated to disapproval signs (no-no). It is a rare entity first described by Benton[1]. We describe three cases managed at the Yalgado Ouédraogo University Hospital in Burkina Faso. Observations. Three children, one girl and two boys, aged respectively 5, 9 and 14 years were seen for involuntary abnormal head movements associating lateral rotations assimilated to signs of disapproval (no-no) in two cases, and back and forth movements of the head in one case. In the 14-year-old adolescent, there was also a picture of intracranial hypertension. Clinical examination revealed ataxia and macrocrania in both cases and a syndrome in 1 case. Imaging revealed triventricular hydrocephalus on aqueductal stenosis in all cases with an associated supra sellar cyst in 2 cases. The treatment consisted of endoscopic treatment in 2 cases and ventriculoperitoneal shunt in 1 case. Surgery allowed a considerable regression of involuntary movements of the head in the immediate postoperative period and a complete recovery in the long term. Conclusion. The bobble head doll syndrome is a rare entity related to the consequences of chronic hydrocephalus responsible for abnormal movements whose management done well and early leads to favourable results.
... The most recent review of bobble head doll syndrome was published in 2018, which included reports of 72 cases between 1966 and 2018 [5] , Since then, additional cases have been reported between 2018 and 2022, totaling 9 cases [13][14][15][16][17][18][19][20][21] . ...
The bobble head doll syndrome is a rare neurological disorder characterized by repetitive and involuntary movement of the head that typically appear in childhood. It is usually associated with the dilatation of the third ventricle and one or more cystic lesions that can be treated surgically. We present the case of a 7-year-old girl with a history of autism, who has experiencing repetitive up and down head movements for 2 years, which were initially thought to be stereotypies. However, 2 months prior to admission, the movements worsened and were accompanied by symptoms of intracranial hypertension. The neurological examination revealed a coordination disorder, specifically a tremor, along with impairment of thermo-algic sensitivity. Ophthalmological examination was unremarkable, but the MRI indicated a colloid cyst of the third ventricle. A minimally invasive neuro-endoscopy procedure was chosen as the treatment of choice for our patient. The bobble head doll syndrome is a complex neurological disorder, and imaging is crucial in the diagnosis and treatment of any movement disorder to enable an early diagnosis and treatment.
Chiari malformation type II (CM-II) is a hindbrain developmental malformation. Movement disorders are rarely described as associated with this condition. We describe the case of a newborn affected by CM-II presenting with head and neck tremors a few days after myelomeningocele repair surgery. Later, self-resolving episodes of expiratory apneas arose. Cranial ultrasounds showed progressive ventricular dilatation. She underwent ventriculoperitoneal shunt followed by craniocervical bony decompression. After surgery, both apnea and tremors disappeared. This particular presentation of CM-II is very rare and potentially difficult to diagnose.
