Bobble-head doll syndrome: report of 2 cases and a review of the literature, with video documentation of the clinical phenomenon

Bryan Renne PhD 1 , Stefan Rueckriegel MD 2 , Sudheesh Ramachandran MCh 1 , Julia Radic MD, MPH, FRCSC 1 , Paul Steinbok MBBS, FRCSC 1 and Ash Singhal MD, MSc, FRCSC 1
View More View Less
  • 1 Faculty of Medicine and the Division of Neurosurgery, University of British Columbia and British Columbia Children’s Hospital, Vancouver, British Columbia, Canada; and
  • 2 Department of Neurosurgery, University Hospital Würzburg, Germany
Full access

Bobble-head doll syndrome (BHDS) is a rare pediatric movement disorder presenting with involuntary 2- to 3-Hz head movements. Common signs and symptoms also found on presentation include macrocephaly, ataxia, developmental delay, optic disc pallor or atrophy, hyperreflexia, tremor, obesity, endocrinopathy, visual disturbance or impairment, headache, and vomiting, among others. The syndrome is associated with suprasellar cysts, third ventricular cysts, or aqueductal obstruction, along with a few other less common conditions. The cause of involuntary head motions is not understood. Treatment is surgical. The authors present 2 cases of BHDS. The first is a 14-year-old boy with BHDS associated with aqueductal obstruction and triventricular hydrocephalus secondary to a tectal tumor. He was successfully treated by endoscopic third ventriculostomy, and all symptoms resolved immediately in the recovery room. This case is unusual in its late age of symptom onset, the primacy of lateral (“no-no”) involuntary head rotations, and the associated tectal tumor. The second case is a 7.5-year-old girl with BHDS associated with a suprasellar cyst. She was successfully treated with an endoscopic fenestration but preexisting endocrinopathy persisted, and the patient was diagnosed with autism spectrum disorder at age 12 years. This second case is more typical of BHDS. A comprehensive and up-to-date review of the literature of BHDS and video documentation of the phenomenon are presented.

ABBREVIATIONS BHDS = bobble-head doll syndrome; CVP = cystoventriculoperitoneal; ETV = endoscopic third ventriculostomy; VP = ventriculoperitoneal.

Bobble-head doll syndrome (BHDS) is a rare pediatric movement disorder presenting with involuntary 2- to 3-Hz head movements. Common signs and symptoms also found on presentation include macrocephaly, ataxia, developmental delay, optic disc pallor or atrophy, hyperreflexia, tremor, obesity, endocrinopathy, visual disturbance or impairment, headache, and vomiting, among others. The syndrome is associated with suprasellar cysts, third ventricular cysts, or aqueductal obstruction, along with a few other less common conditions. The cause of involuntary head motions is not understood. Treatment is surgical. The authors present 2 cases of BHDS. The first is a 14-year-old boy with BHDS associated with aqueductal obstruction and triventricular hydrocephalus secondary to a tectal tumor. He was successfully treated by endoscopic third ventriculostomy, and all symptoms resolved immediately in the recovery room. This case is unusual in its late age of symptom onset, the primacy of lateral (“no-no”) involuntary head rotations, and the associated tectal tumor. The second case is a 7.5-year-old girl with BHDS associated with a suprasellar cyst. She was successfully treated with an endoscopic fenestration but preexisting endocrinopathy persisted, and the patient was diagnosed with autism spectrum disorder at age 12 years. This second case is more typical of BHDS. A comprehensive and up-to-date review of the literature of BHDS and video documentation of the phenomenon are presented.

ABBREVIATIONS BHDS = bobble-head doll syndrome; CVP = cystoventriculoperitoneal; ETV = endoscopic third ventriculostomy; VP = ventriculoperitoneal.

Bobble-head doll syndrome (BHDS) is a rare movement disorder first identified in the literature by Benton et al.5 The cardinal feature of BHDS is an involuntary 2- to 3-Hz movement of the head in the anterior-posterior direction (as if nodding “yes-yes” like the “bobbing” of a bobble-head doll toy), in the lateral rotational direction (as if shaking the head “no-no”), or in some irregular alternating combination of the 2 directions.

We present 2 new cases of BHDS. The first has several unusual features, perhaps the most striking being the late age of symptom onset, a greater tendency toward the rarer lateral (“no-no”) motions, and the patient’s inability to voluntarily suppress the motions. The second is a more typical case, although the age of symptom onset is also unusually late.

A comprehensive review of the literature was performed by the first author (B.R.). A search for the term “bobble head” in both PubMed and MEDLINE was carried out. The cases from these reports were combined with those found in previous reviews.17,19,23,28,33,44,48,49 Various cases were then excluded: those lacking the cardinal head movement feature4,38,41 and those originally reported in publications (often non–English language) that we could not obtain. This yielded a total of 70 previous cases of BHDS. The list of cases extracted from the literature was not confirmed by any of the authors except first author. The list of previously published cases of BHDS combined with the 2 current cases, as well as a summary of their features, is compiled in Table 1.

TABLE 1.

Two BHDS cases currently presented and summary of those reported in the literature

Authors & YearAge (yrs)SexDiagnosisNon-HB Signs & Symptoms1° HBCan Stop HB?*TxPost-Tx HB (complications)
OnsetTx 
Benton et al., 19662.003.25F3VCAtaxia, macrocephaly, intell delay, hyperreflexia, optic disc pallor/atrophy, tremor, visual dist/impairment, headache, tactile hyperesthesia, right Babinski signVYExcision; 1 yr later, VP shunt (for hydrocephalus)Gone w/in 1 yr (diabetes insipidus, hypothyroidism, obesity, hydrocephalus, hypertonicity)
3.506.50F3VCMacrocephaly, hyperreflexia, optic disc pallor/atrophy, tremor, obesity, alternating strabismus, impaired visuomotor perceptionVYFenestrationLess 
Nellhaus, 19679.0010.00MAOAtaxia, macrocephaly, motor delay, hypertonicity, hyperreflexia, mild esophoria, impaired visuomotor perceptionHYVentriculostomy, Pudenz valve; 6 wks later, ventricular drainage, removal of Pudenz valve, insertion of Holter valve (for bilat subdural hematoma)Gone w/in 8 mos (bilat subdural hematoma)
3.006.00M3V massMacrocephalyVNone (HB only while walking)Same 
Nishiura, 19697.5016.00MChronic hydrocephalusAtaxia, intell delay, hyperreflexia, optic disc pallor/atrophy, visual dist/impairment, obesity, behavioral disorder, suicidal ideation, psychopathologyVYVentriculostomy, Pudenz valveLess
Mayher & Gindin, 19700.582.50M3VCMacrocephaly, motor delay, weakness, hyperreflexia, optic disc pallor/atrophy, teeth grindingVSExcisionGone (hemiparesis, seizures, death)
Sakata et al., 197113.00MSCHB only while walkingBFenestration, VP shuntLess
Obenchain & Becker, 19723.0026.00F3VCMacrocephaly, hyperreflexia, optic disc pallor/atrophy, visual dist/impairment, headache, episodic loss of consciousnessVYExcision; 9 days later, ventriculojugular shunt (for headaches, increased ICP)Less
Patriquin, 19733.506.00M3VCAtaxia, macrocephaly, motor delay, weakness, headache, vomiting, obesity, Gower’s signVYExcisionGone w/in 10 days
Ferrey et al., 19742.505.00MSCAtaxia, macrocephaly, optic disc pallor/atrophy, rt local motor seizureVYAspiration & drainageGone
Russo & Kindt, 19740.421.00FAOMacrocephaly, devel delay, hypertonicity, hyperreflexia, lethargyVYVA shuntGone after 8 days
Segall et al., 19741.083.25MSCAtaxia, optic disc pallor/atrophy, tremor, precocious pubertyVYExcisionGone
Görke et al., 19751.677.00MSP cystMotor delay, intell delay, weakness, hypotonicity, hyperreflexia, Grower’s signVYFenestrationLess: after 6 mos, present only when emotionally excited (precocious puberty)
Russman et al., 19752.509.00MCVI cystMacrocephaly, tremor, hyperesthesia to warm water (at 4.5 yrs; resolved by 9 yrs old), bilat dysdiadochokinesia, declining school performanceVYVA shuntLess after 4 mos
5.5010.00MSCMacrocephaly, hyperreflexia, tremor, disturbed behavior, suicidal ideationHYIncision & drainage; 3 wks later, VA shunt (for diabetes insipidus, headaches)Less: after 5 mos, present only w/ anxiety or walking (diabetes insipidus & headaches resolved w/ shunt) 
Tomasovic et al., 19750.330.83FAOAtaxia, macrocephaly, motor delay, hyperreflexia, EMD, bilat ankle clonusVSVP shuntLess w/in 4 mos
4.505.50FAOAtaxia, macrocephaly, motor delay, language delay, tremorVYVP shuntGone after several days 
Deonna & Dubey, 19761.001.58FAOMacrocephaly, motor delayHS/PDrainage via Torkildsen procedureGone
Kirkham, 19770.754.50M3VCAtaxia, optic disc pallor/atrophyVLaceration; 10 mos later, VP shunt (for vomiting, headaches, lethergy, hydrocephalus)Gone, then returned after 1 mo; after shunting, gone w/in 2 yrs
3.5013.00M3VCAtaxia, obesityVYExcisionGone after 2 wks 
Jensen et al., 19780.675.00M3VCAtaxia, hypotonicity, obesityVYExcisionLess after 1 wk
0.92AOShuntSame 
1.50SCShuntSame 
9.003VCExcisionLess 
2.00SCNoneSame 
13.00SCFenestration, shuntLess 
12.00Craniopharyngioma 
SC 
SC 
Albright, 19819.509.50M3VCMacrocephaly, intell delay, hyperreflexia, optic disc pallor/atrophy, obesity, memory loss, precocious puberty, short statureVYExcision; had previous VP shunt age 6 yrs (for ataxia), revised age 9 yrs (for nausea, vomiting, headache)Less
Dell, 198111.00F3VCAtaxia, intell delay, optic disc pallor/atrophy, EMD, chronic seizure disorder, graying of hairVFenestration, drainageGone (fevers that resolved spontaneously after multiple courses of antibiotics)
0.420.50Shunt malfunctionMotor delay, EMD, meningomyelocele L2-L3 to sacrum, preexisting VP shunt (for increasing head circumference), hyporeflexia, absent ankle jerksVPShunt revision; then 3 shunt revisions over 8 mos (for shunt malfunctions)Gone w/in 8 mos of initial revision 
Kaplan et al., 19841.172.00FSCMacrocephaly, optic disc pallor/atrophyHYEndoscopic fenestration, VP shunt; 3 wks later, CVP shunt (for HB recurrence)Gone, then returned after 3 wks; after CVP shunt, gone w/in 1 yr
Wiese et al., 19857.0013.00FSCMacrocephaly, optic disc pallor/atrophy, visual dist/impairment, headache, obesity, intermittent hyperhidrosis but rt-sided anhidrosis, lt lower-extremity hyperalgesia to pinprick below L-1VYLP shuntLess after 5 mos
Harsh et al., 19867.00FSCDevel delayFenestrationLess (transient fever & lethargy)
Coker, 19860.500.50MTrapped 4V (no enlarged 3V)Preexisting shunt at age 4 mos (for lethargy & “setting sun” eye sign sec to AO & fever presumed sec to meningitis), dilation of 4th ventricle & aqueduct at age 6 mosVShunt; 2 mos later, revised (4V dilation, arachnoiditis)Gone, then returned after 2 mos, then gone after revision
Notholt-Heerich et al., 19873.504.10F3VCAtaxia, macrocephaly, motor delay, optic disc pallor/atrophyVDVA shunt, Ommaya reservoir; 6 mos later, CVP shunt (for HB recurrence)Gone, then returned after 6 mos, then less after CVP shunt
Parízek et al., 19893.004.50F3VCAtaxia, optic disc pallor/atrophy, tremor, mild left-sided hemiparesisV2 CVP shunts; 2 days later, 1 replaced (for misplacement)Gone after 3 mos (precocious puberty)
0.3310.00F3VCTremor, vomiting, diabetes insipidus, bulimia, sleeplessness, enuresisVCVP shuntGone after 3 wks (evacuation of pseudocyst at end of catheter after 4.5 yrs) 
3.0014.00M3VCdevel delay, diabetes insipidus, bilat amaurosis, restlessness, confabulation, aggressivenessVDrainage via bilat Torkildsen procedure; 3 yrs later, CVA shuntSame; after CVA shunt, gone w/in 3 mos 
Turgut & Özcan, 19926.008.00M3VCIntell delay, precocious pubertyVExcisionGone after 6 mos
Pollack et al., 19951.752.00MCystic choroid plexus papillomaEpisodic rt anterolat head tilt; drop attacks progressing in hours to comaVYVentriculostomy, endoscopic fenestration SP; 4 days later, resectionGone after resection
Ahn et al., 199710.0011.00MMisplacement catheter tip of shuntAtaxia, intell delay, language delay, hyperreflexia, headache, vomiting, attention deficit disorder, right esotropiaHNRevision (of SDP shunt placed 3 yrs earlier for noncommunicating subdural fluid collection in rt cerebral hemisphere)Gone
Mussell et al., 19971.005.50FSCAtaxia, macrocephaly, hyperreflexia, optic disc pallor/atrophy, tremor, visual dist/impairment, vomiting, obesity, precocious puberty, urinary incontinence, ankle clonusVYEndoscopic fenestration; 3 mos later, VP shunt (for headache, vomiting, incontinence, worsening HB)Less; 6 mos after shunt, HB visible only when walking
Goikhamn et al., 19982.17F3VCAtaxia, macrocephaly, motor delay, hypotonicity, hyperreflexiaVYExcision, CVP shuntGone w/in days
Sharma et al., 200114.0014.00FTrapped 4V (sec to lateral ventricular shunting)Ataxia, headache, vomiting, bilat abducens palsies, neck stiffnessVS/P4V VP shunt (preexisting lateral ventricular VP shunt, revised twice, for congenital hydrocephalus due to AO)Gone w/in 1 wk
Bhattacharyya et al., 200312.0013.00MAO (web)Ataxia, macrocephaly, hyperreflexia, resuscitated from coma (presumed brainstem herniation) 1 wk earlierVYDehydration therapy (due to coma mid-workup); 5 days later, when back to baseline, VP shunt for AOGone
Desai et al., 20032.00FSCVYMarsupializationGone
3.00FSCVYMarsupializationGone 
5.00FSCVYMarsupializationGone 
Fioravanti et al., 20041.00FSCPersistent nocturnal tearing, previous VP shunt (for supratentorial hydrocephalus, still unresolved)SEndoscopic coagulation, fenestration; 18 mos later, endoscopic drainage (for HB recurrence)Less; after 18 mos, returned; after drainage, gone w/in 3 yrs
9.00MSCAtaxia, precocious pubertySEndoscopic fenestrationGone 
Hagebeuk et al., 20050.254.00MSCAtaxia, inability to jumpVYEndoscopic fenestration, balloon dilation of communication w/ basal prepontine cisternGone w/in 3 years
Zamponi et al., 20051.003.003VC, SP cystAtaxia, tremorVSVP shuntGone
Van Benijum et al., 20061.002.00MSCMacrocephaly, motor delayVSEndoscopic fenestrationGone w/in 18 mos
de Brito Henriques et al., 20071.001.00FDandy-Walker syndrome (no 4V hypertension)Posterior fossa cyst, cerebellar vermis hypoplasiaHD/S2 suboccipital punctures testing for hypertension in 4V, then conservative management; had previous VP shunt day 9 of life (for Dandy-Walker syndrome)Same
Guerreiro et al., 20129.0010.00FSCHyperreflexia, nocturnal hunger & enuresis, 13-kg weight gain, impaired short-term memory & attentionVNEndoscopic ventriculocystostomy; 3 mos later, CVP shunt (for HB recurrence)Gone, then returned after 3 mos; after CVP shunt, less, & then gone by age 16 yrs
3.0011.00MSCMacrocephaly, obesityVEndoscopic ventriculocystostomyGone, then returned after 3 mos; lost to follow-up 
0.830.92MSCAtaxia, macrocephaly, devel delay, tremor, tone disturbanceVVentriculocystostomyGone w/in 1 wk 
0.751.00MSCMacrocephaly, devel delay, hyperreflexia, optic disc pallor/atrophy, visual dist/impairmentVVP shunt (to treat hydrocephalus); 17 mos later, endoscopic ventriculocystostomy & removal of VP shuntGone then returned after 3 mo; after endoscopic procedure, gone 
Garg et al., 201220.0020.00MVP shunt drainage malfunction (status post-Tx for tuberculous meningitis)Optic disc pallor/atrophy, visual dist/impairmentBSNone (HB began 24 hrs after VP shunt for ventriculomegaly status 1 mo post-Tx for tuberculous meningitis)Same; lost to follow-up
Ishihara et al., 20130.250.33MPF cyst (causing hydrocephalus)MacrocephalyHSNone (no 3V dilation; had previous endoscopic fenestration day 12 of life for congenital PF cyst causing hydrocephalus)Almost gone by age 1 yr
Alexiou et al., 20134.006.00MSCVNCVP shuntGone
Sridhar et al., 20136.009.00MSCDevel delay, poor height gain, aggression, tactile hallucinationsSEndoscopic fenestration, cauteryGone
Reddy et al., 20148.009.00MSCOptic disc pallor/atrophyVYEndoscopic fenestrationLess
Bhattacharyya et al., 201411.0011.00MAO (pineal mass)Ataxia, optic disc pallor/atrophy, visual dist/impairment, papilledema, sluggish direct & consensual responses, bilateral relative afferent pupillary defectBYVP shuntGone
Mala et al., 20143.004.00MSCVSVentriculocystostomyUnknown (died on postop day 2 w/ seizures)
Ramesh & Raju, 20150.080.83FSC2 tonic-clonic seizuresEndoscopic excision, marsupialization, 3rd ventriculostomy, septostomyGone
8.008.00FSCAtaxia, intermittent fever, 1 tonic-clonic seizureVNEndoscopic ventriculocystostomy, 3rd ventriculostomy, reservoirLess 
1.004.00FSCVSEndoscopic marsupialization, ventriculocystostomy, 3rd ventriculostomyGone 
Present report, Case 114.0014.00MAO (tectal tumor)Macrocephaly, intell delay, optic disc pallor/atrophy, vomiting, lag in upward gazeHPEndoscopic 3rd ventriculostomyGone
Present report, Case 25.507.50FSCHyperreflexia, optic disc pallor/atrophy, upper- & lower-limb proximal weakness, growth hormone deficiency, longstanding polyuria & polydipsia (no diabetes insipidus)VYEndoscopic fenestration, cauteryGone; occasional HB returned after 2 days, then gone after 7.5 yrs

AO = aqueductal obstruction; B = both horizontal and vertical; CVI = cavum velum interpositum; CVP = cystoventriculoperitoneal; D = head motions stop with distraction; devel = developmental; dist = disturbance; EMD = eye movement disorder; H = horizontal (lateral); HB = head bobbing (involuntary head motions); ICP = intracranial pressure; intell = intellectual; N = head motions cannot be stopped voluntarily; P = head motions stop with supine position; PF = posterior fossa; S = head motions stop with sleep; SC = suprasellar cyst; SDP = subduroperitoneal; sec to = secondary to; SP = septum pellucidum; Tx = treatment; V = vertical (anterior-posterior); VA = ventriculoatrial; VP = ventriculoperitoneal; Y = head motions can be stopped voluntarily; 1° HB = primary direction of head bobbing (involuntary head motions); 3V = 3rd ventricle; 3VC = 3rd ventricular cyst; 4V = 4th ventricle; — = unknown.

In this column only, a forward slash (“/”) indicates that there were multiple reported ways involuntary head motions could be stopped (e.g., “S/P” indicates motions can be stopped with sleep and with being in the supine position); however, if motions could be voluntarily suppressed, only a “Y” was recorded.

The most recent BHDS review was published in 2005.19 This current updated review includes reports published since that time as well as other cases found in the literature (at times part of other series). Thus, in addition to presenting a new atypical case and another more typical one, we provide a comprehensive and up-to-date summary of BHDS as of the time of writing. We also obtained video documentation of the phenomenon, which will provide the clinician with an immediate impression of the clinical features of this condition.

Case Reports

Case 1

History and Presentation

A 14-year-old boy presented with a 3-year history of headache with intermittent vomiting, recent cognitive decline, and a 6-week history of repetitive involuntary motions of the head and trunk that had increased in the past 3 days. Primary motion of the head was in a 2- to 3-Hz lateral-to-lateral rotation (as if shaking his head “no-no”), although some occasional anterior-to-posterior movements (as if nodding “yes-yes”) were intermittently present as well. In addition, the patient would involuntarily flex and extend his trunk from time to time as if attempting to find a comfortable position in the bed. Involuntary head motions would stop whenever the patient would rest the posterior aspect of his head on the bed. Video 1 demonstrates head and truncal motions and gaze testing both before and after surgery.

VIDEO 1. Case 1 was a 14-year-old boy with BHDS associated with a tectal mass. Primary head motions show a 2- to 3-Hz lateral-to-lateral (“no-no”) rotation with occasional anterior-to-posterior (“yes-yes”) movements. Involuntary truncal flexion and extension are present as well. Head motions stop when the patient rests the posterior aspect of his head on the bed. Eye movement testing shows some hesitation in upward gaze. Postsurgical excerpts show absence of involuntary motions and full eye movements. Copyright Bryan Renne. Published with permission. Click here to view.

Examination

On examination, the patient was conscious but slightly confused. He had macrocephaly (60.5-cm head circumference, which is more than 2 standard deviations above the age-sex mean) and pale optic discs. He was not able to stand or walk, so an assessment of gait was not feasible. He did show some lag in upward gaze but otherwise had no neurological deficits other than the repetitive motions of the head and trunk. An MRI study of the head showed a tectal mass causing aqueductal obstruction and obstructive triventriculomegaly (Fig. 1).

Fig. 1.
Fig. 1.

Case 1. MR images obtained in a 14-year-old boy with BHDS associated with a tectal mass (white and black arrows). A: Presurgical axial T2-weighted image showing obstructive triventriculomegaly. B: Post-ETV axial T2-weighted image showing reduced size of the third and lateral ventricles. C: Presurgical sagittal turbo inversion recovery magnitude image showing aqueductal obstruction by tectal tumor causing triventricular dilation, bowing of the lamina terminalis and the floor of the third ventricle, and sellar erosion. D: Post-ETV sagittal magnetization-prepared rapid gradient-echo image showing a relaxed floor of the third ventricle. Post-ETV images were taken 9 months after the presurgical images and showed that the tectal mass increased in size to 19 × 25 × 24 mm3 (craniocaudal × anteroposterior × right-left) from a presurgical size of 19 × 20 × 20 mm3.

Operation and Postoperative Course

An endoscopic third ventriculostomy (ETV) was performed without complications. The patient’s head and truncal motions resolved completely in the recovery room immediately after surgery. On 4-month follow-up, the motions had not returned. An MRI study obtained 9 months after surgery did show mild increased size of the tumor; however, the ventricles had decreased in size. Endocrinological follow-up has been unremarkable, though an adrenocorticotropic hormone (ACTH) stimulation test is scheduled. The patient will be followed up further by endocrinology and oncology departments.

Case 2

History and Presentation

A 7.5-year-old, left-handed girl presented with a 2-year history of recently worsening involuntary anterior-posterior bobbing motions of the head at approximately 3 Hz. The movements were more severe when she was emotionally upset, would stop during sleep, and could be voluntarily suppressed for a couple of minutes. There was no discomfort or exhaustion associated with the head bobbing. A history of headache or vomiting was denied.

Examination

On examination, the patient was alert, oriented, and appeared healthy. She had normal cognitive development. Her head circumference was 53.5 cm (90th percentile). She had short stature (110 cm, which is more than 3 standard deviations below the age-sex mean). Eye movements were full, and there were no visual field deficits. However, bilateral optic atrophy was noted. Motor examination showed Grade 4+ proximal weakness of the upper and lower limbs, with normal strength distally. Reflexes were brisk, more so in the lower limbs (Grade 3+) than in the upper limbs (Grade 2+). Tandem gait was slightly unsteady both backward and forward. Plantar responses were normal, and clonus was absent. The sensory examination showed normal findings. An endocrine assessment revealed normal thyroid, cortisol, and antidiuretic hormone reserve; however, the patient did have growth hormone deficiency. She had a longstanding history of polyuria and polydipsia, but diabetes insipidus was ruled out. An MRI study of the head showed a large suprasellar cyst causing obstructive hydrocephalus (Fig. 2 and Video 2).

VIDEO 2. Case 2 was a 7.5-year-old girl with BHDS associated with a suprasellar cyst. Primary head motions are a 3-Hz anterior-to-posterior (“yes-yes”) movements. Gait testing is demonstrated. Intraoperative excerpt shows cyst fenestration and cautery. Postsurgical excerpt shows absence of involuntary head motions. Copyright Bryan Renne and Paul Steinbok. Published with permission. Click here to view.

Fig. 2.
Fig. 2.

Case 2. MR images acquired in a 7.5-year-old girl with BHDS associated with suprasellar cyst. Presurgical axial T2-weighted image (A) and presurgical sagittal constructive interference in steady-state (CISS) image (C) showing a large suprasellar cyst. Post-fenestration axial T2-weighted image (B) and post-fenestration CISS image (D) showing reduced size of the cyst.

Operation and Postoperative Course

An endoscopic fenestration of the cyst was performed. Cisternostomy was attempted but was aborted due to the occurrence of bradycardia. Because the cisternostomy could not be performed, the cyst wall was shrunk using cautery. The patient’s head bobbing resolved in the recovery room immediately after surgery. However, intermittent anterior-posterior bobbing did return on the 2nd postoperative day, although it was greatly diminished compared with that seen at initial presentation and occurred only during times of fatigue or emotional upset. This intermittent head bobbing eventually ceased as the patient was followed up over a 7.5-year period. The patient was started on growth hormone shortly after surgery. Her growth over the follow-up period put her not far from the height expected based on her parents’ heights. The patient was diagnosed at age 12 with autism spectrum disorder.

Discussion

The cardinal feature of BHDS is an involuntary 2- to 3-Hz movement of the head in the anterior-posterior (“yes-yes”) direction, the lateral (“no-no”) rotational direction, or some irregular alternating combination of the 2 movements. In addition to involuntary head and truncal motions, information on presenting signs and symptoms was available in 58 of the 72 cases (81% of total, see Table 1). Common presenting signs and symptoms are summarized in Table 2. Other less common signs and symptoms not included in Table 2 are listed with individual cases in Table 1.

TABLE 2.

Presenting signs and symptoms of BHDS

Signs & SymptomsNo. of Cases (of 58)% of Cases
Macrocephaly2747
Ataxia2543
Impairment or developmental delay (cognition, motor, or language)2441
Optic disc pallor or atrophy2136
Hyperreflexia1831
Tremor1119
Obesity916
Endocrinopathy (including precocious puberty)916
Visual disturbance or impairment814
Headache610
Vomiting610
Weakness35
Hypotonicity35
Eye movement disorders35
Hypertonicity23

Presenting signs and symptoms other than involuntary head motions were reported in 58 cases (81% of total).

The age of onset of abnormal head movements was documented in 55 cases (76% of total), and in these cases the median onset age was 3.0 years (median absolute deviation of 3.35 years), with a range of 1 month to 20 years. Thus, BHDS is typically a disease of early childhood, and the onset age of 14 years in our Case 1 is the second oldest reported in the literature.

Of the 62 cases (86% of total) for which patient sex is identified, 35 were male (56%) and 27 female (44%) for a male/female ratio of 1.3:1. Previous reviews, which relied on a subset of the cases we have identified, report a higher male/female ratio of 2:1.

Of the 59 cases (82% of total) for which there was enough information to identify a direction of repetitive head movements, 48 were primarily anterior-posterior (i.e., 81% “yes-yes”) and 8 were primarily lateral (i.e., 14% “no-no”). For patients with both anterior-posterior bobbing (“yes-yes”) and lateral shaking (“no-no”) movements, one direction took primacy over the other in all but 3 cases (5%). The BHDS literature often mentions that head motions decrease or cease with sleep, distraction, or with patients in the supine position; increase with excitement or anxiety; and are voluntarily suppressible for a period of seconds to minutes. Regarding the increase or decrease in motion, the literature is often unclear as to whether this change refers to frequency, amplitude, perceived effort on the part of the patient, or something else. As for whether head motions are suppressible, of 50 cases (69% of the total) in which this information was available, movements were reported as voluntarily suppressible for 29 patients (58%); as not voluntarily suppressible for 4 (8%); and as suppressible with one or more distraction, or sleep, or being in the supine position (and either not voluntarily suppressible or without mention of being voluntarily suppressible) in 17 (34%). Of the 37 patients (51% of total) treated at age 3 years or more with available data on whether head motions were suppressible, 24 patients (65%) could suppress motions voluntarily, while 13 (35%) either could not suppress head motions voluntarily or could do so only with one or more of distraction, or sleep, or being in the supine position. This suggests that repetitive head motions in BHDS can usually be suppressed, and, if the child is at least 3 years old at the time of treatment, suppression is most often voluntary. These considerations highlight 2 other rare features of our case 1: This 14-year-old patient could not suppress his head motions voluntarily (although he could do so when supine), and his primary direction of motion was lateral shaking (“no-no”). Our case 2 is more typical, in that the patient could temporarily suppress her head motions and the primary direction of motion was anterior-posterior bobbing (“yes-yes”).

In the 72 cases of this review, the major pathologies associated with BHDS were as follows:

  • 32 cases (44%): suprasellar cyst (including our Case 2).3,12–14,18–20,23,24,26,28,36–38,40,41,43,47,48
  • 18 cases (25%): third ventricular cyst,2,5,10,16,23,25,27,31–34,46,49 in one case concurrent with a septum pellucidum cyst.49
  • 10 cases (14%): aqueductal obstruction (aqueductal stenosis 8,11,23,29,39,44 aqueductal web 1,7 pineal mass 1,6 and tectal tumor 1 [our case 1]); 1 patient had an associated isolated trapped fourth ventricle.8
  • 4 cases (6%): shunt malfunction, misplacement, or complication, of which 2 were due to malfunction,1,10 1 was due to a trapped fourth ventricle after lateral ventricular shunting,42 and 1 was due to catheter placement obstructing the left foramen of Monro.15
  • 3 cases (4%): other cysts impinging on the third ventricle, of which 1 was a septum pellucidum cyst,17 1 was a cyst of the cavum velum interpositum,38 and 1 was a cystic choroid plexus papilloma causing obstructive hydrocephalus.35
  • 2 cases (3%): masses impinging on the third ventricle, 1 a third ventricular mass29 and 1 a craniopharyngioma.23
  • 2 cases (3%): after fenestration of posterior fossa cysts, 1 after endoscopic fenestration but without enlargement of the third ventricle22 and 1 in a patient with Dandy-Walker syndrome who did not have intracranial hypertension at the onset of head bobbing.9
  • 1 case (1%): chronic hydrocephalus.30

The pathophysiology and causative anatomical substrate of BHDS is unknown, although various mechanisms have been suggested: distortion of structures or pathways adjacent to the third ventricle, perhaps involving the basal ganglia (since motions can often be voluntarily suppressed);5,29,30 compression of the dorsomedial nucleus of thalamus or the dentatorubrothalamic pathway;19,39 a possible role of fetal viral infection in anterior horn cells;17 the mammillary-fornical-hippocampal circuit;2 a “learned behavior” causing transient relief of foraminal blockage or cystic volume reduction,35,48 which was demonstrated in one case using a CT cisternogram;48 pressure on the dorsomedial red nucleus;8 and cerebellar dysfunction,9,22 which has been noted to cause abnormal head motions.21,45

Treatment of BHDS is surgical, with the particular procedure depending on the associated pathology. Generally the treatment involves restoration of CSF flow and reducing pressure on compressed structures.

BHDS symptom recurrence has been reported in 8 cases (see Table 1), including our case 2:

  • A patient with third ventricular cyst treated with laceration showed return of head motions within 1 month; a ventriculoperitoneal (VP) shunt ceased motions within 2 years.25
  • A patient with a suprasellar cyst treated with endoscopic fenestration and a VP shunt had return of head motions after 3 weeks; a cystoventriculoperitoneal (CVP) shunt ceased motions within 1 year.24
  • A patient with trapped fourth ventricle treated by shunt placement had return of head motions after 2 months; these were eliminated after shunt revision.8
  • A patient with obstructive hydrocephalus secondary to third ventricular cyst treated with a ventriculoatrial shunt and an Ommaya reservoir had head motion recurrence after 6 months; these were lessened but not eliminated after CVP shunt placement.31
  • A patient with suprasellar cyst treated with endoscopic ventriculocystostomy had head motion recurrence after 3 months; a CVP shunt led to elimination of motions within a 16-year follow-up period.18
  • A patient with a suprasellar cyst treated with endoscopic ventriculocystostomy had head motion recurrence after 3 months; the patient was subsequently lost to follow-up.18
  • A patient with obstructive hydrocephalus secondary to a suprasellar cyst treated with VP shunting had head motion recurrence after 3 months; removal of the shunt and endoscopic ventriculocystostomy eliminated head motions.18
  • In our case 2, an SC was treated with endoscopic fenestration and cautery, and occasional head motions returned after 2 days, but head motions disappeared completely within the 7.5-year follow-up.

Thus, symptom recurrence in BHDS is not often reported (8 [11%] of 72 cases reviewed), has occurred within 6 months of initial treatment in the cases known to us, and does not always require treatment. Nevertheless, given the possibility of growth of mass or recurrence of cyst, we elect to follow our BHDS patients with annual MRI. Assuming successful treatment and stable posttreatment clinical course, we transition to less frequent imaging as time progresses.

In conclusion, we have presented 2 new cases of BHDS and have performed a comprehensive review of the literature. We have also provided video documentation of the phenomenon, which will give the clinician an immediate impression of the clinical features of BHDS.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: Renne, Rueckriegel, Steinbok, Singhal. Acquisition of data: all authors. Analysis and interpretation of data: Renne, Rueckriegel, Radic, Steinbok, Singhal. Drafting the article: Renne, Steinbok, Singhal. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Renne. Study supervision: Singhal.

Supplemental Information

References

  • 1

    Ahn Y, Cho BK, Wang KC: Bobble-head doll syndrome associated with subduroperitoneal shunt malfunction. Childs Nerv Syst 13:234237, 1997

  • 2

    Albright L: Treatment of bobble-head doll syndrome by transcallosal cystectomy. Neurosurgery 8:593595, 1981

  • 3

    Alexiou GA, Sfakianos G, Prodromou N: Giant suprasellar arachnoid cyst with head bobbing. Mov Disord 28:1216, 2013

  • 4

    Banerjee T: Abnormal movements with hydrocephalus. Case report. J Neurosurg 46:674676, 1977

  • 5

    Benton JW, Nellhaus G, Huttenlocher PR, Ojemann RG, Dodge PR: The bobble-head doll syndrome: report of a unique truncal tremor associated with third ventricular cyst and hydrocephalus in children. Neurology 16:725729, 1966

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 6

    Bhattacharyya KB, Deb S, Ghosh SN, Mondal S: Bobbling head in a young subject. Ann Indian Acad Neurol 17:371373, 2014

  • 7

    Bhattacharyya KB, Senapati A, Basu S, Bhattacharya S, Ghosh S: Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand 108:216220, 2003

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Coker SB: Bobble-head doll syndrome due to trapped fourth ventricle and aqueduct. Pediatr Neurol 2:115116, 1986

  • 9

    de Brito Henriques JG, Henriques KSW, Filho GP, Fonseca LF, Cardoso F, Da Silva MC: Bobble-head doll syndrome associated with Dandy-Walker syndrome. Case report. J Neurosurg 107 (3 Suppl):248250, 2007

    • Search Google Scholar
    • Export Citation
  • 10

    Dell S: Further observations on the “bobble-headed doll syndrome”. J Neurol Neurosurg Psychiatry 44:10461049, 1981

  • 11

    Deonna T, Dubey B: Bobble-head doll syndrome. Case report with a review on the different types of abnormal head movements in infancy, and their clinical significance. Helv Paediatr Acta 31:221227, 1976

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Desai KI, Nadkarni TD, Muzumdar D, Goel A: Suprasellar arachnoid cyst presenting with bobble-head doll movements: a report of 3 cases. Neurol India 51:407409, 2003

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13

    Ferrey PC, Feldman V, Kerber C: The bobble-head doll syndrome due to a basal arachnoid cyst. Neurology 24:394, 1974

  • 14

    Fioravanti A, Godano U, Consales A, Mascari C, Calbucci F: Bobble-head doll syndrome due to a suprasellar arachnoid cyst: endoscopic treatment in two cases. Childs Nerv Syst 20:770773, 2004

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Garg RK, Singh SK, Malhotra HS, Singh MK: Abnormal head movement in a patient with tuberculous meningitis. BMJ Case Rep 2012:bcr2012006663, 2012

  • 16

    Goikhman I, Zelnik N, Peled N, Michowiz S: Bobble-head doll syndrome: a surgically treatable condition manifested as a rare movement disorder. Mov Disord 13:192194, 1998

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Görke W, Pendl G, Pandel C: Spinal muscular atrophy in a boy with head-nodding resulting from a large septum pellucidum cyst. Neuropediatrics 6:190201, 1975

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 18

    Guerreiro H, Vlasak A, Horinek D, Tichy M, Lisy J, Vanek P, : Bobble-head doll syndrome: therapeutic outcome and long-term follow-up in four children. Acta Neurochir (Wien) 154:20432049, 2012

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19

    Hagebeuk EEO, Kloet A, Grotenhuis JA, Peeters EAJ: Bobble-head doll syndrome successfully treated with an endoscopic ventriculocystocisternostomy. Case report and review of the literature. J Neurosurg 103 (3 Suppl):253259, 2005

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20

    Harsh GR IV, Edwards MSB, Wilson CB: Intracranial arachnoid cysts in children. J Neurosurg 64:835842, 1986

  • 21

    Hottinger-Blanc PMZ, Ziegler AL, Deonna T: A special type of head stereotypies in children with developmental (?cerebellar) disorder: description of 8 cases and literature review. Eur J Paediatr Neurol 6:143152, 2002

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 22

    Ishihara M, Nonaka M, Oshida N, Hamada Y, Nakajima S, Yamasaki M: “No-no” type bobble-head doll syndrome in an infant with an arachnoid cyst of the posterior fossa: a case report. Pediatr Neurol 49:474476, 2013

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 23

    Jensen HP, Pendl G, Goerke W: Head bobbing in a patient with a cyst of the third ventricle. Childs Brain 4:235241, 1978

  • 24

    Kaplan BJ, Mickle JP, Parkhurst R: Cystoperitoneal shunting for congenital arachnoid cysts. Childs Brain 11:304311, 1984

  • 25

    Kirkham TH: Optic atrophy in the bobble-head doll syndrome. J Pediatr Ophthalmol 14:299301, 1977

  • 26

    Mala R, Lakshmi AM, Nagireddy NB, Chiniga VR: Suprasellar arachnoid cyst presenting with Bobble-head doll syndrome. J NTR Univ Health Sci 3:4850, 2014

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 27

    Mayher WE III, Gindin RA: Head bobbing associated with third ventricular cyst. Report of a case. Arch Neurol 23:274277, 1970

  • 28

    Mussell HG, Dure LS, Percy AK, Grabb PS: Bobble-head doll syndrome: report of a case and review of the literature. Mov Disord 12:810814, 1997

  • 29

    Nellhaus G: The bobble-head doll syndrome: a “tic” with a neuropathologic basis. Pediatrics 40:250253, 1967

  • 30

    Nishiura N: A case of bobble-head doll syndrome associated with psychopathic behaviors. Folia Psychiatr Neurol Jpn 23:275283, 1969

  • 31

    Notholt-Heerich B, Körholz D, Voit T, Lumenta C: Das Bobble head doll Syndrom. Klin Padiatr 199:7779, 1987

  • 32

    Obenchain TG, Becker DP: Head bobbing associated with a cyst of the third ventricle. Case report. J Neurosurg 37:457459, 1972

  • 33

    Parízek J, Nĕmecková J, Šercl M: Bobble-head doll syndrome associated with the III ventricular cyst. Three cases in children 7 years after CVP or CVA shunting. Childs Nerv Syst 5:241245, 1989

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    Patriquin HB: The bobble-head doll syndrome. A curable entity. Radiology 107:171172, 1973

  • 35

    Pollack IF, Schor NF, Martinez AJ, Towbin R: Bobble-head doll syndrome and drop attacks in a child with a cystic choroid plexus papilloma of the third ventricle. Case report. J Neurosurg 83:729732, 1995

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 36

    Ramesh S, Raju S: Suprasellar arachnoid cyst presenting with bobble-head doll syndrome: report of three cases. J Pediatr Neurosci 10:1821, 2015

  • 37

    Reddy OJ, Gafoor JA, Suresh B, Prasad PO: Bobble head doll syndrome: a rare case report. J Pediatr Neurosci 9:175177, 2014

  • 38

    Russman BS, Tucker SH, Schut L: Slow tremor and macrocephaly: expanded version of the bobble-head doll syndrome. J Pediatr 87:6366, 1975

  • 39

    Russo RH, Kindt GW: A neuroanatomical basis for the bobble-head doll syndrome. J Neurosurg 41:720723, 1974

  • 40

    Sakata K, Yamada H, Yamamoto M, Takahashi C: [Bobble-head doll syndrome: report of a case with a large basal arachnoid cyst.] Brain Nerve 23:693700, 1971 (Jpn)

    • Search Google Scholar
    • Export Citation
  • 41

    Segall HD, Hassan G, Ling SM, Carton C: Suprasellar cysts associated with isosexual precocious puberty. Radiology 111:607616, 1974

  • 42

    Sharma RR, Pawar SJ, Devadas RV, Dev EJ: CT stereotaxy guided lateral trans-cerebellar programmable fourth ventriculo-peritoneal shunting for symptomatic trapped fourth ventricle. Clin Neurol Neurosurg 103:143146, 2001

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43

    Sridhar S, Purkayastha M, Dutta P, Dhandapani SS, Mukherjee KK: Third ventricular cyst presenting with bobble head doll movements and tactile hallucinations. Neurol India 61:8788, 2013 (Letter)

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 44

    Tomasovic JA, Nellhaus G, Moe PG: The bobble-head doll syndrome: an early sign of hydrocephalus. Two new cases and a review of the literature. Dev Med Child Neurol 17:777783, 1975

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 45

    Tully HM, Dempsey JC, Ishak GE, Adam MP, Mink JW, Dobyns WB, : Persistent figure-eight and side-to-side head shaking is a marker for rhombencephalosynapsis. Mov Disord 28:20192023, 2013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 46

    Turgut M, Özcan OE: Suprasellar arachnoid cyst as a cause of precocious puberty and bobble-head doll phenomenon. Eur J Pediatr 151:76, 1992

  • 47

    Van Beijnum J, Hanlo PW, Han KS, Ludo Van der Pol W, Verdaasdonk RM, Van Nieuwenhuizen O: Navigated laser-assisted endoscopic fenestration of a suprasellar arachnoid cyst in a 2-year-old child with bobble-head doll syndrome. Case report. J Neurosurg 104 (5 Suppl):348351, 2006

    • Search Google Scholar
    • Export Citation
  • 48

    Wiese JA, Gentry LR, Menezes AH: Bobble-head doll syndrome: review of the pathophysiology and CSF dynamics. Pediatr Neurol 1:361366, 1985

  • 49

    Zamponi N, Rychlicki F, Trignani R, Polonara G, Ruggiero M, Cesaroni E: Bobble head doll syndrome in a child with a third ventricular cyst and hydrocephalus. Childs Nerv Syst 21:350354, 2005

    • Crossref
    • Search Google Scholar
    • Export Citation

If the inline PDF is not rendering correctly, you can download the PDF file here.

Contributor Notes

Correspondence Bryan Renne: BC Children’s Hospital, Vancouver, BC, Canada. bryan@renne.org.

INCLUDE WHEN CITING Published online January 5, 2018; DOI: 10.3171/2017.9.PEDS16704.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

  • View in gallery

    Case 1. MR images obtained in a 14-year-old boy with BHDS associated with a tectal mass (white and black arrows). A: Presurgical axial T2-weighted image showing obstructive triventriculomegaly. B: Post-ETV axial T2-weighted image showing reduced size of the third and lateral ventricles. C: Presurgical sagittal turbo inversion recovery magnitude image showing aqueductal obstruction by tectal tumor causing triventricular dilation, bowing of the lamina terminalis and the floor of the third ventricle, and sellar erosion. D: Post-ETV sagittal magnetization-prepared rapid gradient-echo image showing a relaxed floor of the third ventricle. Post-ETV images were taken 9 months after the presurgical images and showed that the tectal mass increased in size to 19 × 25 × 24 mm3 (craniocaudal × anteroposterior × right-left) from a presurgical size of 19 × 20 × 20 mm3.

  • View in gallery

    Case 2. MR images acquired in a 7.5-year-old girl with BHDS associated with suprasellar cyst. Presurgical axial T2-weighted image (A) and presurgical sagittal constructive interference in steady-state (CISS) image (C) showing a large suprasellar cyst. Post-fenestration axial T2-weighted image (B) and post-fenestration CISS image (D) showing reduced size of the cyst.

  • 1

    Ahn Y, Cho BK, Wang KC: Bobble-head doll syndrome associated with subduroperitoneal shunt malfunction. Childs Nerv Syst 13:234237, 1997

  • 2

    Albright L: Treatment of bobble-head doll syndrome by transcallosal cystectomy. Neurosurgery 8:593595, 1981

  • 3

    Alexiou GA, Sfakianos G, Prodromou N: Giant suprasellar arachnoid cyst with head bobbing. Mov Disord 28:1216, 2013

  • 4

    Banerjee T: Abnormal movements with hydrocephalus. Case report. J Neurosurg 46:674676, 1977

  • 5

    Benton JW, Nellhaus G, Huttenlocher PR, Ojemann RG, Dodge PR: The bobble-head doll syndrome: report of a unique truncal tremor associated with third ventricular cyst and hydrocephalus in children. Neurology 16:725729, 1966

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 6

    Bhattacharyya KB, Deb S, Ghosh SN, Mondal S: Bobbling head in a young subject. Ann Indian Acad Neurol 17:371373, 2014

  • 7

    Bhattacharyya KB, Senapati A, Basu S, Bhattacharya S, Ghosh S: Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand 108:216220, 2003

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Coker SB: Bobble-head doll syndrome due to trapped fourth ventricle and aqueduct. Pediatr Neurol 2:115116, 1986

  • 9

    de Brito Henriques JG, Henriques KSW, Filho GP, Fonseca LF, Cardoso F, Da Silva MC: Bobble-head doll syndrome associated with Dandy-Walker syndrome. Case report. J Neurosurg 107 (3 Suppl):248250, 2007

    • Search Google Scholar
    • Export Citation
  • 10

    Dell S: Further observations on the “bobble-headed doll syndrome”. J Neurol Neurosurg Psychiatry 44:10461049, 1981

  • 11

    Deonna T, Dubey B: Bobble-head doll syndrome. Case report with a review on the different types of abnormal head movements in infancy, and their clinical significance. Helv Paediatr Acta 31:221227, 1976

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Desai KI, Nadkarni TD, Muzumdar D, Goel A: Suprasellar arachnoid cyst presenting with bobble-head doll movements: a report of 3 cases. Neurol India 51:407409, 2003

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13

    Ferrey PC, Feldman V, Kerber C: The bobble-head doll syndrome due to a basal arachnoid cyst. Neurology 24:394, 1974

  • 14

    Fioravanti A, Godano U, Consales A, Mascari C, Calbucci F: Bobble-head doll syndrome due to a suprasellar arachnoid cyst: endoscopic treatment in two cases. Childs Nerv Syst 20:770773, 2004

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Garg RK, Singh SK, Malhotra HS, Singh MK: Abnormal head movement in a patient with tuberculous meningitis. BMJ Case Rep 2012:bcr2012006663, 2012

  • 16

    Goikhman I, Zelnik N, Peled N, Michowiz S: Bobble-head doll syndrome: a surgically treatable condition manifested as a rare movement disorder. Mov Disord 13:192194, 1998

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Görke W, Pendl G, Pandel C: Spinal muscular atrophy in a boy with head-nodding resulting from a large septum pellucidum cyst. Neuropediatrics 6:190201, 1975

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 18

    Guerreiro H, Vlasak A, Horinek D, Tichy M, Lisy J, Vanek P, : Bobble-head doll syndrome: therapeutic outcome and long-term follow-up in four children. Acta Neurochir (Wien) 154:20432049, 2012

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19

    Hagebeuk EEO, Kloet A, Grotenhuis JA, Peeters EAJ: Bobble-head doll syndrome successfully treated with an endoscopic ventriculocystocisternostomy. Case report and review of the literature. J Neurosurg 103 (3 Suppl):253259, 2005

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20

    Harsh GR IV, Edwards MSB, Wilson CB: Intracranial arachnoid cysts in children. J Neurosurg 64:835842, 1986

  • 21

    Hottinger-Blanc PMZ, Ziegler AL, Deonna T: A special type of head stereotypies in children with developmental (?cerebellar) disorder: description of 8 cases and literature review. Eur J Paediatr Neurol 6:143152, 2002

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 22

    Ishihara M, Nonaka M, Oshida N, Hamada Y, Nakajima S, Yamasaki M: “No-no” type bobble-head doll syndrome in an infant with an arachnoid cyst of the posterior fossa: a case report. Pediatr Neurol 49:474476, 2013

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 23

    Jensen HP, Pendl G, Goerke W: Head bobbing in a patient with a cyst of the third ventricle. Childs Brain 4:235241, 1978

  • 24

    Kaplan BJ, Mickle JP, Parkhurst R: Cystoperitoneal shunting for congenital arachnoid cysts. Childs Brain 11:304311, 1984

  • 25

    Kirkham TH: Optic atrophy in the bobble-head doll syndrome. J Pediatr Ophthalmol 14:299301, 1977

  • 26

    Mala R, Lakshmi AM, Nagireddy NB, Chiniga VR: Suprasellar arachnoid cyst presenting with Bobble-head doll syndrome. J NTR Univ Health Sci 3:4850, 2014

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 27

    Mayher WE III, Gindin RA: Head bobbing associated with third ventricular cyst. Report of a case. Arch Neurol 23:274277, 1970

  • 28

    Mussell HG, Dure LS, Percy AK, Grabb PS: Bobble-head doll syndrome: report of a case and review of the literature. Mov Disord 12:810814, 1997

  • 29

    Nellhaus G: The bobble-head doll syndrome: a “tic” with a neuropathologic basis. Pediatrics 40:250253, 1967

  • 30

    Nishiura N: A case of bobble-head doll syndrome associated with psychopathic behaviors. Folia Psychiatr Neurol Jpn 23:275283, 1969

  • 31

    Notholt-Heerich B, Körholz D, Voit T, Lumenta C: Das Bobble head doll Syndrom. Klin Padiatr 199:7779, 1987

  • 32

    Obenchain TG, Becker DP: Head bobbing associated with a cyst of the third ventricle. Case report. J Neurosurg 37:457459, 1972

  • 33

    Parízek J, Nĕmecková J, Šercl M: Bobble-head doll syndrome associated with the III ventricular cyst. Three cases in children 7 years after CVP or CVA shunting. Childs Nerv Syst 5:241245, 1989

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    Patriquin HB: The bobble-head doll syndrome. A curable entity. Radiology 107:171172, 1973

  • 35

    Pollack IF, Schor NF, Martinez AJ, Towbin R: Bobble-head doll syndrome and drop attacks in a child with a cystic choroid plexus papilloma of the third ventricle. Case report. J Neurosurg 83:729732, 1995

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 36

    Ramesh S, Raju S: Suprasellar arachnoid cyst presenting with bobble-head doll syndrome: report of three cases. J Pediatr Neurosci 10:1821, 2015

  • 37

    Reddy OJ, Gafoor JA, Suresh B, Prasad PO: Bobble head doll syndrome: a rare case report. J Pediatr Neurosci 9:175177, 2014

  • 38

    Russman BS, Tucker SH, Schut L: Slow tremor and macrocephaly: expanded version of the bobble-head doll syndrome. J Pediatr 87:6366, 1975

  • 39

    Russo RH, Kindt GW: A neuroanatomical basis for the bobble-head doll syndrome. J Neurosurg 41:720723, 1974

  • 40

    Sakata K, Yamada H, Yamamoto M, Takahashi C: [Bobble-head doll syndrome: report of a case with a large basal arachnoid cyst.] Brain Nerve 23:693700, 1971 (Jpn)

    • Search Google Scholar
    • Export Citation
  • 41

    Segall HD, Hassan G, Ling SM, Carton C: Suprasellar cysts associated with isosexual precocious puberty. Radiology 111:607616, 1974

  • 42

    Sharma RR, Pawar SJ, Devadas RV, Dev EJ: CT stereotaxy guided lateral trans-cerebellar programmable fourth ventriculo-peritoneal shunting for symptomatic trapped fourth ventricle. Clin Neurol Neurosurg 103:143146, 2001

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43

    Sridhar S, Purkayastha M, Dutta P, Dhandapani SS, Mukherjee KK: Third ventricular cyst presenting with bobble head doll movements and tactile hallucinations. Neurol India 61:8788, 2013 (Letter)

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 44

    Tomasovic JA, Nellhaus G, Moe PG: The bobble-head doll syndrome: an early sign of hydrocephalus. Two new cases and a review of the literature. Dev Med Child Neurol 17:777783, 1975

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 45

    Tully HM, Dempsey JC, Ishak GE, Adam MP, Mink JW, Dobyns WB, : Persistent figure-eight and side-to-side head shaking is a marker for rhombencephalosynapsis. Mov Disord 28:20192023, 2013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 46

    Turgut M, Özcan OE: Suprasellar arachnoid cyst as a cause of precocious puberty and bobble-head doll phenomenon. Eur J Pediatr 151:76, 1992

  • 47

    Van Beijnum J, Hanlo PW, Han KS, Ludo Van der Pol W, Verdaasdonk RM, Van Nieuwenhuizen O: Navigated laser-assisted endoscopic fenestration of a suprasellar arachnoid cyst in a 2-year-old child with bobble-head doll syndrome. Case report. J Neurosurg 104 (5 Suppl):348351, 2006

    • Search Google Scholar
    • Export Citation
  • 48

    Wiese JA, Gentry LR, Menezes AH: Bobble-head doll syndrome: review of the pathophysiology and CSF dynamics. Pediatr Neurol 1:361366, 1985

  • 49

    Zamponi N, Rychlicki F, Trignani R, Polonara G, Ruggiero M, Cesaroni E: Bobble head doll syndrome in a child with a third ventricular cyst and hydrocephalus. Childs Nerv Syst 21:350354, 2005

    • Crossref
    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 1401 80 0
Full Text Views 1230 934 100
PDF Downloads 699 279 22
EPUB Downloads 0 0 0