Bobble-head Doll Syndrome Research Articles

Long Lead of a Bobble-Head Doll Syndrome

Abstract Bobble-head doll syndrome is a rare and interesting childhood movement disorder, which often presents with involuntary, repetitive, stereotyped head movements, though other presentations such as neuroendocrine disturbances, visual and gait disturbances, and memory and psychiatric disturbances are also described. Imaging reveals hydrocephalus. If the cause can be identified and resolved using surgery, it is one of the treatable movement disorders of childhood.

Bobble Head Doll Syndrome

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.

Bobble-head doll syndrome in an infant with an arachnoid cyst: a case report

BackgroundBobble-head doll syndrome is a rare and unique movement disorder most commonly affecting children younger than 5 years of age. It is characterized by continuous or episodic movement at the frequency of 2–3 Hz. The exact mechanism of bobble-head doll syndrome has not been elucidated. Endoscopic ventriculocisternostomy is the optimal treatment option. In a literature review, there were less than 75 cases of bobble-head doll syndrome with suprasellar arachnoid cyst.Case presentationWe report a case of a 1.5-year-old Asian-Syrian girl who presented with a history of excessive head nodding for 3 months that increased with walking, emotions, and stress; decreased during periods of concentration; and was absent during sleep. On physical examination, she was alert and normal, with no medical history. Laboratory assessment and ophthalmological examination were normal. Cranial magnetic resonance imaging demonstrated a well-defined thin-walled suprasellar arachnoid cyst measuring 3 × 5 × 7 cm that obstructed the foramina of Monro, with resulting hydrocephalus ventriculomegaly. The patient underwent endoscopic cystoventriculostomy and cystocisternostomy for the suprasellar arachnoid cyst. During the 6 months of follow-up, the head bobbing disappeared completely, and her growth was normal.ConclusionDespite the rareness of bobble-head doll syndrome, it is considered an important condition that must be investigated early to detect the cause and treated promptly to avoid potential complications.

Obstructive Hydrocephalus Presenting with Bobble-Head Doll Syndrome.

Obstructive Hydrocephalus Presenting with Bobble-Head Doll Syndrome.

Letter to the Editor Regarding “Prospective Study of Surgery for Traumatic Brain Injury in Addis Ababa, Ethiopia: Trauma Causes, Injury Types, and Clinical Presentation”

The bobble-head doll syndrome (BHDS) is a rare acquired head movement disorder characterized by up and down or side-to-side movement, most commonly seen in the first decade of life. The syndrome occurs more often in lesions causing third ventricle dilatation such as suprasellar or third ventricle cyst, but it is also found in other pathologies associated with hydrocephalus like shunt dysfunctions, trapped fourth ventricle, congenital aqueductal stenosis, Dandy-Walker syndrome, and cerebellar malformations. The pathophysiology of this head movement has different origins theories; one states that this stereotyped movements empties the cyst and move the dome away from the foramina of Monro, which relieves the symptoms of hydrocephalus; the other suggests that the extrapyramidal tracts (rubrotegmentospinal and reticulospinal) are stimulated by the compression of dorsomedial nucleus of the thalamus by the cyst, whose tracts innervate the neck muscles resulting in the bobbling head movements. This video (Video 1) presents a clinical case of BHDS caused by suprasellar cyst in a 10- year-old boy treated by endoscopic procedure. A ventricular-cyst-cisternostomy was performed resulting in complete improvement of the head movements and uneventful recovery. Postoperative images demonstrate decreasing of the cyst lesion and resolution of the hydrocephalus.

Presumptive pituitary macroadenoma in an English Setter resembling human bobble-head doll syndrome

Head bobbing (HB) or idiopathic head tremor is a benign condition characterised by episodic uncontrolled head tremors that start and stop spontaneously or when dogs are focused on a goal-oriented...

Bobble-Head Doll Syndrome in a Child with Suprasellar Arachnoid Cyst.

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Hypomania in Bobble-Head Doll Syndrome: A Case Report of Surgically Treated Stereotypy and Hypomania.

A 22-year-old man was admitted with gradually aggravating stereotypic head movement with hypomania. Brain magnetic resonance imaging showed a large suprasellar arachnoid cyst extending into the third ventricle, with obstructive hydrocephalus, characteristic of bobble-head doll syndrome. Endoscopic fenestration of the suprasellar arachnoid cyst was performed. Stereotypic head movement stopped immediately after surgery and hypomanic symptoms gradually improved within a month. During 4 years of follow-up observation without medication, neuropsychiatric symptoms did not relapse. We report our experience of surgically treating stereotypy and hypomania in a case of bobble-head doll syndrome and discuss the possible neuropsychiatric mechanisms of this rare disease.

Large intraventricular epidermoid with bobble head doll syndrome in an adult

Abstract Intracranial epidermoid tumors are rare (0.2-1.8%), histologically benign, slow-growing, congenital neoplasms of the central nervous system arising from the retained ectodermal implants. We report an unusual case of intra third ventricular epidermoid in an adult presenting with the Bobble-head doll syndrome and review the relevant literature.

Bobble-head doll syndrome in an 80-year-old man, associated with a giant arachnoid cyst of the lamina quadrigemina, treated with endoscopic ventriculocystocisternotomy and cystoperitoneal shunt

Bobble-head doll syndrome (BHDS) is a rare entity, characterized by antero-posterior head bobbing, which is of the type "yes-yes." Less frequently, having a head movement of the type "no-no" is described. We report an unusual case of an 80-year-old man with a cystic mass of the lamina quadrigemina, extending to the posterior fossa. We conclude that ventriculocystocisternotomy associated with a cystoperitoneal shunt is an effective treatment for a symptomatic giant arachnoid cyst in the lamina quadrigemina.

Suprasellar arachnoid cyst presenting with Bobble-head doll syndrome

The incidence of intracranial suprasellar arachnoid cysts is 9% of all arachnoid cysts. This case report describes a patient with a suprasellar arachnoid cyst who presented at the age of 4 with a rare bobble-head doll syndrome. Endoscopic ventriculocystostomy was performed. The literature on suprasellar arachnoid cysts is reviewed

Bobble-head doll syndrome: therapeutic outcome and long-term follow-up in four children

Bobble-head doll syndrome (BHDS) is a complex syndrome with the dominant symptom of repetitive anteroposterior head movement. Only 57 patients are quoted in the literature. The etiology of this syndrome remains unknown and no standard treatment has yet been established. We hereby report four cases treated at our department. All the patients presented a psychomotor retardation due to an obstructive hydrocephalus. All the patients were treated using neuroendoscopic techniques: two with ventriculocystostomy, and two with ventriculocystocisternostomy. Cyst decompression was achieved in all four cases and clinical recovery was evident in three of the four patients observed. After surgery, BHDS persisted longer the more the subsequent treatment was delayed. In this article, we provide a concise overview of the theories of pathogenesis, presentation, and management of this syndrome. Based on our own experience, we state that the method of choice should be the neuroendoscopy and this must be performed promptly after diagnosis is made.

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.

Bobble-head doll syndrome associated with Dandy–Walker syndrome

Bobble-head doll syndrome (BHDS) presents in childhood and is usually associated with lesions of the third ventricle. This disorder is characterized by stereotypical head movements of the type "yes-yes" (up and down) at a frequency of 2 to 3 Hz. Rarely, movements of the type "no-no" (side-to-side) are described. There are a few hypotheses to explain the mechanism responsible for BHDS, but its real pathophysiological characteristics are still unknown. The authors describe the case of a child born with hydrocephalus and Dandy-Walker syndrome. A ventriculoperitoneal shunt was implanted in the child because of progressive head enlargement. One year after shunt placement, she began making frequent horizontal head movements of the type "no-no". There were no other signs or symptoms. Imaging studies demonstrated small ventricles and a posterior fossa cyst with no signs of hypertension. The child's growth, development, and head circumference (within the 5th percentile) remained satisfactory. Three aspects of this case were of interest: the association of BHDS with Dandy-Walker syndrome, the rare occurrence of BHDS of the "no-no" type, and the absence of third ventricle dilation. The authors' findings support the hypothesis that cerebellar malformations themselves can

Teaching Neuro Image : Tremor in aqueductal stenosis and response to endoscopic third ventriculostomy

Idiopathic aqueductal stenosis (AS) may account for up to 59% of cases presenting with triventricular noncommunicating hydrocephalus.1 The clinical presentations associated with triventricular hydrocephalus differ depending on age at onset and the acuity of obstruction. Acute syndromes include Parinaud's syndrome (vertical gaze restriction, lid retraction, and pupillary abnormalities), the rostral midbrain syndrome (upward gaze palsy, retraction nystagmus, pyramidal and extrapyramidal signs), and deficits in arousal. In the very young, chronic diencephalic compression can produce the bobble-head doll syndrome (high frequency head movements, limb ataxia, tremor, and cognitive deficits). Resolution of transtentorial pressure gradients by CSF diversion typically produces rapid improvement.2 We describe an adult patient with upper extremity tremor due to decompensated hydrocephalus from AS, who demonstrated improvement following endoscopic third ventriculostomy. An 18-year-old right-handed African American man presented with chronic, progressive tremor of the right hand. He was the product of an uncomplicated pregnancy and satisfied normative criteria for both growth and developmental milestones including head circumference. There was no history of head injury or encephalitis. At age 9 he developed action tremor in his dominant hand, which began in the distal upper extremity and progressed over 6 months to include the proximal arm and right leg. These symptoms were aggravated by stress, and interfered with activities of daily living. While he was considered less agile than his peers, he had no functional impairment or problems with falls. Rest tremor was not appreciated. By age 13, …

Navigated laser-assisted endoscopic fenestration of a suprasellar arachnoid cyst in a 2-year-old child with bobble-head doll syndrome

The authors present the case of a 2-year-old boy with bobble-head doll syndrome (BHDS) associated with a large suprasellar arachnoid cyst and enlarged ventricles, who was successfully treated with neuronavigated laser-assisted endoscopic ventriculocystocisternostomy. The clinical history, surgical treatment, and clinical follow up of the patient are described. A navigated laser-assisted endoscopic ventriculocystocisternostomy of the suprasellar arachnoid cyst led to cessation of the head bobbing, and notable reduction of the cyst and ventricles was visible on the postoperative magnetic resonance images. Caused by a suprasellar arachnoid cyst, BHDS can be successfully treated with navigated laser-assisted endoscopic ventriculocystocisternostomy. The advantages of this procedure are minimal invasiveness and facilitated guidance of the neuronavigation system to the target area when normal anatomical landmarks are not visible.

Bobble-head doll syndrome successfully treated with an endoscopic ventriculocystocisternostomy

The bobble-head doll syndrome (BHDS) is characterized by a back-and-forth movement of the head with a frequency of 2 to 3 Hz, which increases during walking and excitement and decreases during concentration. The head movements are accompanied by macrocephaly, ocular disturbances, psychomotor retardation, and sometimes endocrine dysfunction. The BHDS is frequently associated with a suprasellar arachnoid cyst. The authors present the case of a 4-year-old patient with BHDS; an endoscopic cystoventriculostomy was performed by fenestrating a cyst in the suprasellar region. After wide fenestration of the cyst wall that was protruding and obstructing the foramen of Monro, the cyst was entered with the endoscope and a small, natural, valvelike communication of the cyst with the basal prepontine cistern was seen close to the basilar artery. This communication was widened by balloon dilation. After completion of the ventriculocystocisternostomy, the cyst collapsed and the obstruction of the aqueduct was resolved. In view of the source mechanism and cerebrospinal fluid dynamics of the suprasellar arachnoid cyst, a ventriculocystocisternostomy is an important treatment option for BHDS arising from a suprasellar cyst. Three years after treatment, the head bobbing had resolved completely and psychomotor development was improving. Delay of diagnosis and treatment of this condition can cause permanent neurological dysfunction and psychomotor retardation. The authors recommend early ventriculocystocisternostomy as a physiologically based treatment for BHDS originating from a suprasellar cyst.

Bobble-Head Doll Syndrome Treated Surgically

A case of a 4-year-old child with bobble-head doll syndrome (BHDS) caused by a suprasellar arachnoid cyst and successfully treated by endoscopic cystovenriculostomy is reported from Medisch Centrum Haaglanden, The Hague; and Academical Hospital Nijmegen, The Netherlands.

A special type of head stereotypies in children with developmental (?cerebellar) disorder:: description of 8 cases and literature review

The authors report eight children, who presented in the first year of life with isolated head stereotypies, that corresponded neither to the usual normal ‘rhythmic habit patterns of infancy’, nor to various types of abnormal repetitive head movements described in young children. Their head stereotypies closely resembled those described in bobble-head doll syndrome. The neurological status evidenced axial hypotonia, ataxia, oculomotor abnormalities, motor and language delay. The patients were followed for several years clinically and with video recordings. No single aetiology was found. Computed tomography (CT) or magnetic resonance imaging (MRI) show a congenital cerebellar abnormality in two children but no hydrocephalus. The outcome of these children showed in all but one patient a normal cognitive and psychosocial development, even though the head stereotypies are still present in six of eight patients and all remain significantly clumsy. The association of head stereotypies and motor delay should prompt a search for cerebellar congenital malformation. The outcome of those patients was much better than originally anticipated and these head stereotypies are not related either to mental retardation, or to psychopathology. The possible mechanisms involved are also discussed.

CT stereotaxy guided lateral trans-cerebellar programmable fourth ventriculo–peritoneal shunting for symptomatic trapped fourth ventricle

A trapped fourth ventricle is a rare clinico-radiological entity producing symptoms suggestive of a progressive posterior fossa mass lesion. It is mainly reported in children as a late complication of lateral ventricular shunting to relieve infantile post-meningitic or post-hemorrhagic hydrocephalus. Optional treatment of the trapped fourth ventricle remains controversial. Placement of fourth ventricular shunting via a conventional midline approach can be fraught with complications in about 40% of the patients. Authors report a successful CT Stereotaxy guided high pressure (80 mm H 2O) programmable fourth ventriculo–peritoneal shunting via a lateral trans-cerebellar approach in a 14-year-old girl with a trapped fourth ventricle, which occurred as a late complication of ventriculo-peritoneal shunting in her infancy. Her preoperative symptoms of raised intracranial pressure, bobble-head doll syndrome and bilateral abducens palsies completely improved following the surgery. Lateral trans-cerebellar stereotactic placement of the fourth ventricular catheter and the use of high-pressure (low flow) programmable shunt (to avoid complications associated with over drainage) are beneficial in some patients with trapped fourth ventricle.