Third ventricular cyst presenting with bobble head doll movements and tactile hallucinations
Subbiah Sridhar1, Moushumi Purkayastha2, Pinaki Dutta1, SS Dhandapani3, Kanchan Kumar Mukherjee3 1 Department of Endocrinology, Post Graduate Institute of Medical Education and Research, Chandigarh, Haryana, India 2 Department of Psychiatry, Maharishi Markandeshwar University, MM Institute of Medical Sciences and Research, Mullana (Ambala), Haryana, India 3 Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Chandigarh, Haryana, India
Date of Submission
23-Sep-2012
Date of Decision
23-Sep-2012
Date of Acceptance
22-Jan-2013
Date of Web Publication
4-Mar-2013
Correspondence Address: Kanchan Kumar Mukherjee Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Chandigarh, Haryana India
Source of Support: None, Conflict of Interest: None
How to cite this article: Sridhar S, Purkayastha M, Dutta P, Dhandapani S S, Mukherjee KK. Third ventricular cyst presenting with bobble head doll movements and tactile hallucinations. Neurol India 2013;61:87-8
How to cite this URL: Sridhar S, Purkayastha M, Dutta P, Dhandapani S S, Mukherjee KK. Third ventricular cyst presenting with bobble head doll movements and tactile hallucinations. Neurol India [serial online] 2013 [cited 2023 Mar 21];61:87-8. Available from: https://www.neurologyindia.com/text.asp?2013/61/1/87/108030
Sir,
Bobble head doll syndrome (BHDS) is a rare movement disorder, characterized by forward, backward, or side-to-side head nodding. [1] We herein report a patient with third ventricular cystic lesion, who presented with psychiatric manifestations and growth retardation.
A 9-year-old boy presented with abnormal head movements, poor scholastic performance, and poor height gain of 3 years duration. The head bobbing was aggravated by activities, diminished during periods of distraction, and disappeared during sleep [Video 1]. History reported by the patient's parent revealed that the patient would complain that people around him were pinching him. On examination, the patient's height was 116 cm (<3 rd percentile) and head circumference was 54 cm. Ophthalmological examination revealed no papilledema. Both testicular volumes were 2 ml each and puberty staging was Tanner 1. Psychological assessment revealed intelligent quotient (IQ) of 67. Baseline hormone profile showed random growth hormone 0.7(N, 0.5-7.0 ng/ml) and IGF-1 113.1 (N, 183-850 ng/ml) with normal thyroid function tests. Magnetic resonance imaging showed a 9 × 8-cm large suprasellar cyst in the third ventricle with compression of the fornix, corpus callosum, and pons along with enlarged lateral ventricle and obstructive hydrocephalus [[Figure 1]: Upper panel]. The patient was subjected to endoscopic fenestration of the cyst with ventriculo-cystocisternostomy. The cyst wall was fulgurated till only a stump remained and the abnormal head movements disappeared immediately after surgery. The patient was re-evaluated up to 14 months after the surgery. The head bobbing had disappeared permanently. In addition, there was 13 cm height gain, appearance of pubic hair growth, and increase in the testicular volume (15 ml each), features suggestive of pubertal onset. The catch-up growth of 13 cm over 14 months indicates the recovery of hypothalamic-pituitary-growth hormone axis. His aggression and tactile hallucinations had disappeared and his IQ had improved to 90. MRI showed reduction in size of the cyst as well as partial resolution of hydrocephalus [[Figure 1]: Lower panel].
Figure 1: Upper panel: Sagittal and axial contrast MR images obtained preoperatively, showing a large third ventricular cyst, compressing fornix, corpus callosum, pons, and mesencephalon with prominently enlarged lateral ventricles Lower panel: Sagittal and axial MR images obtained 4 months postoperatively, showing significant reduction in the size of the cyst and mass effect along with reduction in the hydrocephalus
The location of the cyst close to the hypothalamic-pituitary area can give rise to endocrine abnormalities such as deficiencies of growth hormone, gonadotropins, thyrotropin, and vasopressin. [2] The possible mechanism of this movement disorder is due to the pressure effects of third ventricular tumors or supra sellar cysts. They distort the dorsomedial red nucleus and dentato-rubro-thalamic pathways. [3] Psychiatric manifestations are rarely described in BHDS. [4] Tactile hallucinations have not been described earlier and may be due to the involvement of thalamus. Endoscopic ventriculo-cysto-cisternostomy is an effective, safe, and less invasive treatment option available for the management of third ventricular cyst. Prolonged follow-up is advised after neurosurgical treatment, due to later development of hormone deficiencies.
Adan L, Bussières L, Dinand V, Zerah M, Pierre-Kahn A, Brauner R. Growth, puberty and hypothalamic-pituitary function in children with suprasellar arachnoid cyst. Eur J Pediatr 2000;159:348-55.
Tetraventricular hydrocephalus with aqueductal flow void: an overlooked entity having consistent improvement following endoscopic third ventriculostomy
Sushanta K. Sahoo, Sivashanmugam Dhandapani, Chirag K. Ahuja
