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Year : 2014  |  Volume : 3  |  Issue : 1  |  Page : 48-50

Suprasellar arachnoid cyst presenting with Bobble-head doll syndrome

Department of Neurosurgery, Kurnool Medical College, Government General Hospital, Kurnool, Andhra Pradesh, India

Date of Web Publication10-Mar-2014

Correspondence Address:
Ramanjulu Mala
Department of Neurosurgery, Kurnool Medical College, Government General Hospital, Kurnool - 518 002, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2277-8632.128440

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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

Keywords: Bobble-head doll syndrome, obstructive hydrocephalus, suprasellar arachnoid cysts, ventriculocystostomy

How to cite this article:
Mala R, Lakshmi AM, Nagireddy NB, Chiniga VR. Suprasellar arachnoid cyst presenting with Bobble-head doll syndrome. J NTR Univ Health Sci 2014;3:48-50

How to cite this URL:
Mala R, Lakshmi AM, Nagireddy NB, Chiniga VR. Suprasellar arachnoid cyst presenting with Bobble-head doll syndrome. J NTR Univ Health Sci [serial online] 2014 [cited 2022 Jan 22];3:48-50. Available from: https://www.jdrntruhs.org/text.asp?2014/3/1/48/128440

  Introduction Top

Barlow in 1935 reported the first case of suprasellar arachnoid cyst. [1] Arachnoid cysts are developmental anomalies often diagnosed in childhood as incidental findings on imaging. Arachnoid cysts account for 1% of all intracranial lesions. [2] Suprasellar arachnoid cysts account for 9% of all intracranial arachnoid cysts. Bobble-head doll syndrome was first reported by Benton et al. in 1966. [3] Suprasellar arachnoid cysts can present as Bobble-head syndrome, hydrocephalus, psychiatric disturbance, macrocephaly, endocrinological disturbances and visual disturbances. [4] Ventriculocystostomy has been found to be effective and safe. [5]

  Case Report Top

This was a case report of 4-year-old boy presented with involuntary movements of head forward and backward of 1 year duration. There were no movements during sleep or at rest. Routine hematological and biochemical parameters were within the normal limits. Computerized tomography scan revealed suprasellar cystic lesion isodense with cerebrospinal fluid (CSF) with obstructive hydrocephalus [Figure 1]. Magnetic resonance imaging (MRI) shows a suprasellar mass lesion, which follows CSF signal in all sequences [Figure 2] and [Figure 3] and obstructive hydrocephalus with characteristic ~Mickey-mouse~ appearance. Ventriculocystostomy [Figure 4] done. Patient developed seizures on the 2 nd post-operative day and died due to aspiration. Histological examination of the cyst wall revealed arachnoid cyst.
Figure 1: Computed tomography scan axial view showing suprasellar arachnoid cyst with obstructive hydrocephalus with "Mickey-mouse" appearance

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Figure 2: Magnetic resonance imaging coronal view showing large suprasellar cyst

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Figure 3: Magnetic resonance imaging sagittal view showing large suprasellar cyst

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Figure 4: Endoscopic view showing fenestration of cyst into the lateral ventricle

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  Discussion Top

Arachnoid cysts contain clear colorless liquid resembling normal CSF. Starkman et al. [6] consider these lesions arise from a developmental aberration involving duplication or splitting of the arachnoid membrane. Most of the suprasellar arachnoid cysts occur in children and there is a known male prevalence. The syndrome of Bobble-head doll movements is noticed more often in lesions, which cause third ventricle dilation such as suprasellar arachnoid cyst, colloid cyst, aqueductal stenosis, craniopharyngiomas and rarely choroid plexus papilloma of the third ventricle. [7] It is a rare neurological movement disorder in which patient usually children around the age of 3 began to bob their head and shoulders forward and back, or sometimes side to side involuntarily. The bobbing is volitional, or be able to be stopped by making a conscious decision. The bobbing also disappears while patient is asleep. Compression or intermittent pressure by the cyst on the dorsomedial nucleus stimulates these diencephalic extra pyramidal pathways and results in bobbling of the head. [8] In contrast Weise et al. suggested that head bobbling is a learned behavior, through which the patient is able to reduce the cyst size or relieve the ventricular dilatation by rocking the cyst back and forth. [9] Differential diagnosis of suprasellar arachnoid cysts includes cystic craniopharyngiomas, Rathke, s cleft cysts, epidermoid cysts. On MRI these lesions generally have the signal intensity of CSF in all sequences, do not enhance do not demonstrate restricted diffusion. [5] The most important differential diagnostic consideration is between the arachnoid and epidermoid cysts. The aim of surgical treatment is to normalize CSF flow by establishing a permanent communication between the cyst cavity and the intraventricular or/and subarachnoid space. Two types of endoscopic procedures described in the literature. The first one is a ventriculocystostomy. This consists of creating a large fenestration in superior wall of the cyst. The second one is a ventriculocystocysternostomy consists of creating a second fenestration in the lower wall of the cyst, usually connecting to prepontine cistern. [4]

  Conclusion Top

Early diagnosis and treatment is highly important in successful treatment of Bobble-head doll syndrome. Suprasellar arachnoid cysts should be approached endoscopically, dual endoscopic fenestration into the intraventricular compartment and the basal cistern is safe and efficacious treatment for these lesions.

  Acknowledgments Top

The authors would like to thank Professor Dr. W. Seetharam. M.Ch. for his suggestions and review of this paper and Dr. Ananth. M.Ch. Dr. B. Nagaraju, Dr. Venkateswara Rao for collecting data for this paper and for preparing the manuscript.

  References Top

1.Barlow A. Suprasellar arachnoid cyst. Arch Ophthalmol 1935;14;53-60.  Back to cited text no. 1
2.Yadav YR, Parihar V, Sinha M, Jain N. Endoscopic treatment of the suprasellar arachnoid cyst. Neurol India 2010;58:280-3.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.Benton JW, Nellhaus G, Huttenlocher PR, et al. The bobble-head doll syndrome: A report of unique trunkal tremor associated with third ventricular cyst and hydrocephalus in children. Neurology 1966;16:725-9.  Back to cited text no. 3
4.Hoffman HJ, Hendrick EB, Humphreys RP, Armstrong EA. Investigation and management of suprasellar arachnoid cysts. J Neurosurg 1982;57:597-602.  Back to cited text no. 4
5.Wang JC, Heier L, Souweidane MM. Advances in the endoscopic management of suprasellar arachnoid cysts in children. J Neurosurg 2004;100:418-26.  Back to cited text no. 5
6.Starkman SP, Brown TC, Linell EA. Cerebral arachnoid cysts. J Neuropathol Exp Neurol 1958;17:484-500.  Back to cited text no. 6
7.Desai KI, Nadkarni TD, Muzumdar D, Goel A. Suprasellar arachnoid cyst presenting with bobble-head doll movements: A report of 3 cases. Neurol India 2003;51:407-9.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
8.Russo RH, Kindt GW. A neuroanatomical basis for the bobble-head doll syndrome. J Neurosurg 1974;41:720-3.  Back to cited text no. 8
9.Wiese JA, Gentry LR, Menezes AH. Bobble-head doll syndrome. Review of the pathophysiology and CSF dynamics. Pediatr Neurol 1985;1:361-6.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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