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Table of Contents    
Year : 2011  |  Volume : 59  |  Issue : 6  |  Page : 946-947

Spontaneous cerebrospinal fluid leak associated with idiopathic intracranial hypertension

1 Department of Neurology, Rigshospitalet, Copenhagen University Hospital, Copenhagen; Faculty of Health Sciences, University of Southern Denmark, Odense, Denmark
2 Department of Neurology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark

Date of Submission15-Sep-2011
Date of Decision16-Sep-2011
Date of Acceptance16-Sep-2011
Date of Web Publication2-Jan-2012

Correspondence Address:
D Kondziella
Department of Neurology, Rigshospitalet, Copenhagen University Hospital, Copenhagen
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.91403

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How to cite this article:
Kurtzhals C, Hansen K, Kondziella D. Spontaneous cerebrospinal fluid leak associated with idiopathic intracranial hypertension. Neurol India 2011;59:946-7

How to cite this URL:
Kurtzhals C, Hansen K, Kondziella D. Spontaneous cerebrospinal fluid leak associated with idiopathic intracranial hypertension. Neurol India [serial online] 2011 [cited 2020 Feb 28];59:946-7. Available from:


We read with interest the recent review by Vaghela et al., in Neurology India, entitled "Spontaneous intracranial hypo- and hypertension: An imaging review". [1] We write in order to stress the point that recent reports from the otorhinolaryngological literature suggest that spontaneous cerebrospinal fluid (CSF) leaks are strongly associated with idiopathic intracranial hypertension (IIH). [2],[3],[4],[5] This association has not been mentioned in the article by Vaghela et al., and indeed, to our knowledge, it has not yet been described in the neurological literature.

In 2000 a 29-year-old obese woman was diagnosed with IIH based on a history of headache, papilledema, increased CSF opening pressure and a magnetic resonance imaging (MRI) of her brain that was reported as normal. She was treated with diuretics, acetazolamide and dietary advice. At a two-year follow-up headaches had disappeared and fundus examination was normal. Drug treatment was discontinued. However, during the following years she experienced continuous watery nasal discharge, consistent with CSF rhinorrhea, and two episodes of bacterial meningitis. In 2011 a computed tomography and second MRI revealed a meningocele in the left upper nasal cavity [Figure 1]a-c. Reassessment of her first MRI showed that the meningocele had increased significantly in size (data not shown). The site of the CSF leak was confirmed intraoperatively prior to operative closure of the CSF leak.
Figure 1: Coronal (a) and sagittal (b) bone algorithm computed tomography and coronal contrast-enhanced T1-weighted MRI (c) reveal a left-sided nasal cavity meningocele (asterix)

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Recent evidence from the otorhinolaryngological literature indicates that patients with spontaneous CSF leaks have very similar epidemiological and clinical features compared to patients with IIH. [2],[3],[4],[5] In the largest case series of spontaneous CSF leaks, 85% of patients were obese (mean BMI 36.2 kg/m 2 ), 77% were women and the median CSF lumbar opening pressure was significantly increased following closure of spontaneous CSF leaks. [3] In a smaller case series, 13 out of 16 patients with spontaneous CSF leaks were woman, 15 were obese and intracranial pressure (ICP) was increased in 10 out of 10 patients in whom it was assessed. [5] In another recent paper a diagnosis of IIH, using the modified Dandy criteria, [6] was definite in 8 out of 11 patients with spontaneous CSF leaks and likely in the remaining three. [5] It has therefore been suggested that spontaneous CSF leaks represent a variant of IIH. [4] Increased ICP and constant pulsatile pressure applied to the skull base over time may lead to erosion at sites of inherent structural weakness, such as the dura of the sellar diaphragm, perforations in the cribriform plate and natural foramina of the skull base. [2] This was likely the mechanism responsible for CSF rhinorrhea and enlargement of the meningocele in our patient. Importantly, after operative closure of a spontaneous CSF leak, patients must be carefully followed for signs of increased ICP in order to prevent recurrence of the leak. [2]

  References Top

1.Vaghela V, Hinqwala DR, Kapilamoorthy TR, Kesavadas C, Thomas B. Spontaneous intracranial hypo and hypertensions: An imaging review. Neurol India 2011;59:506-48.  Back to cited text no. 1
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2.Woodworth BA, Palmer JN. Spontaneous cerebrospinal fluid leaks. Curr Opin Otolaryngol Head Neck Surg 2009;17:59-65.  Back to cited text no. 2
3.Woodworth BA, Prince A, Chiu AG, Cohen NA, Schlosser RJ, Bolger WE, et al. Spontaneous CSF leaks: A paradigm for definitive repair and management of intracranial hypertension. Otolaryngol Head Neck Surg 2008;138:715-20.  Back to cited text no. 3
4.Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous cerebrospinal fluid leaks: A variant of benign intracranial hypertension. Ann Otol Rhinol Laryngol 2006;115:495-500.  Back to cited text no. 4
5.Schlosser RJ, Wilensky EM, Grady MS, Bolger WE. Elevated intracranial pressures in spontaneous cerebrospinal fluid leaks. Am J Rhinol 2003;17:191-5.  Back to cited text no. 5
6.JJ, Thompson HS. The rational management of idiopathic intracranial hypertension. Arch Neurol 1989;46:1049-51.  Back to cited text no. 6


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