Bobble Head Syndrome

The bobble head doll syndrome ( Engl. Bobble-head-doll syndrome , short BHDS ) is a rare neurological syndrome in pediatrics , which is accompanied by rhythmic head movements. It is the result of hydrocephalus and occurs mainly in children under five years of age.

causes

Bobble-headed doll syndrome occurs as a result of hydrocephalus . The hydrocephalus, which leads to this clinical picture, is usually the result of cyst formation (e.g. arachnoid cyst ) or other tumors in the cerebral ventricles , especially the third ventricle . The pathophysiological basis for the occurrence of rhythmic head movements has not yet been clarified with certainty. It is believed that they are triggered by pressure on core areas in the brain stem .

frequency

Bobble-head doll syndrome almost exclusively affects children under the age of five. The disease is very rare. About 30 cases have been published in the medical literature since the syndrome was first described in 1966 by JW Benton and others.

Clinical picture

The clinical picture is characterized by nodding head movements, which occur with a frequency of two to three movements per second. The upper body can be involved in these oscillating movements. It is also characteristic that the movements mainly occur when the child is awake. When the child is asleep and when the child is concentrating, the oscillations disappear or are weakened; when the child is emotionally excited, they are intensified. In addition to these symptoms typical for the clinical picture, numerous other abnormalities can be present. Tremors and so-called pyramidal orbit signs often occur . The development of the psyche and motor skills is restricted or slowed down.

therapy

By eliminating the cause, for example by neurosurgical removal of the cyst, a regression of the symptoms can usually be achieved.

literature

B. Notholt-Heerich, D. Körholz u. a .: The Bobble head doll syndrome. In: Clinical Pediatrics. 199, 1987, p. 77, doi : 10.1055 / s-2008-1026766 .

Individual evidence

↑ ^a ^b ^c Maja Steinlin, Michael Schüpbach: Movement disorders in children and young adults . Swiss Neurological Society October 2008, Neurology.ch, Issue No. 5, Vol 3, October 10, 2008. (Online Version) ( Memento from December 3, 2008 in the Internet Archive )

↑ JW Benton, G. Nellhaus, PR Huttenlocher , RG Ojemann, PR Dodge: The bobble-head doll syndrome: report of unique truncal tremor associated with third ventricular cyst and hydrocephalus in children. In: Neurology . 1966; 16, pp. 725-729, doi : 10.1212 / WNL.16.8.725 .

^ ^A ^b R. Witkowski, O. Prokop , E. Ullrich, G. Thiel: Lexicon of syndromes and malformations: causes, genetics, risks . Springer-Verlag 2003, ISBN 3-540-44305-3 , p. 1283.

[Steinlin-1] Maja Steinlin, Michael Schüpbach: Movement disorders in children and young adults . Swiss Neurological Society October 2008, Neurology.ch, Issue No. 5, Vol 3, October 10, 2008. (Online Version) ( Memento from December 3, 2008 in the Internet Archive )

[2] JW Benton, G. Nellhaus, PR Huttenlocher , RG Ojemann, PR Dodge: The bobble-head doll syndrome: report of unique truncal tremor associated with third ventricular cyst and hydrocephalus in children. In: Neurology . 1966; 16, pp. 725-729, doi : 10.1212 / WNL.16.8.725 .

[Lexikon-3] A ^b R. Witkowski, O. Prokop , E. Ullrich, G. Thiel: Lexicon of syndromes and malformations: causes, genetics, risks . Springer-Verlag 2003, ISBN 3-540-44305-3 , p. 1283.