About Bioline  All Journals  Testimonials  Membership  News

Neurology India
Medknow Publications on behalf of the Neurological Society of India
ISSN: 0028-3886 EISSN: 1998-4022
Vol. 59, Num. 4, 2011, pp. 633-634

Neurology India, Vol. 59, No. 4, July-August, 2011, pp. 633-634

Letter to Editor

Spontaneous intracranial hypotension in a patient with classical type Ehlers-Danlos syndrome

W. J. H. M Grosveld1, HJ Gilhuis1, NC Voermans2

1 Department of Neurology, Reinier de Graaf Hospital, Delft, The Netherlands
2 UMC St. Radboud, Nijmegen, The Netherlands
Correspondence Address: H J Gilhuis, Department of Neurology, Reinier de Graaf Hospital, Delft, The Netherlands,

Date of Submission: 25-May-2011
Date of Decision: 29-May-2011
Date of Acceptance: 10-Jul-2011

Code Number: ni11190

PMID: 21891953

DOI: 10.4103/0028-3886.84357


A 37-year-old female, a known case of classical type Ehlers-Danlos syndrome (EDS) developed progressive headache over a period of one month. Headache would occur shortly after assuming an upright position and get relieved on lying down and was associated with nausea, vomiting, and blurred vision. Physical examination revealed typical features of classical type EDS: hyperextensible skin, generalized joint hypermobility, and widened atrophic scars and no neurological deficits. Complete blood picture, blood biochemistry, cerebrospinal fluid (CSF) analysis, and opening pressure of the lumbar puncture were normal. Brain T1-weighted magnetic resonance imaging (MRI) with gadolinium showed diffuse pachymeningeal enhancement [Figure - 1]. Radioisotope cisternography identified a CSF leakage at the level of the cervicothoracic junction [Figure - 2]. A diagnosis of spontaneous intracranial hypotension (SIH) due to CSF leakage was considered and she was advised bed rest for five days with no effect, after which she was successfully treated with an epidural blood patch at the level of the leakage.

SIH is caused by spinal CSF leakage, which is presumed to be related to an underlying weakness of the spinal meninges. This syndrome presents with a postural headache, which is defined as a headache that occurs within 15 min after assuming an upright position and disappears or improves within 30 min after lying down. [1] In addition to headaches, SIH patients often have a wide variety of additional symptoms like nausea, vomiting, neck stiffness, tinnitus, hypacusia, and photophobia. Diffuse pachymeningeal enhancement after administration of gadolinium is the most characteristic feature on MRI, and confirms the diagnosis. Other typical MRI features include subdural fluid collections, engorgement of venous structures, pituitary hyperemia, and sagging of the brain. Computed tomography myelography or radionuclide cisternography are the imaging modalities to identify the site of the CSF leakage. An epidural blood patch is the mainstay of treatment. [1],[2],[3]

Evidence for an inherited connective tissue disorder such as EDS is found on physical examination in two-thirds of SIH patients. Isolated joint hypermobility is found in two-fifths of these patients. One-fifth of patients have subtle skeletal manifestations of Marfan syndrome without a mutation in fibrillin-1. Less frequently, a well-defined connective tissue disorder such as Marfan syndrome or EDS is found. These findings suggest that patients with inherited connective tissue diseases are more susceptible to SIH due to the generalized fragility of tissues, probably including the meninges. [2],[3] The Marfan syndrome has repeatedly been associated with postural headache due to SIH, mostly in children and young adults. [4],[5] Till date, only two cases of classical type EDS with postural headache due to SIH have been reported. [3] In conclusion, the diagnosis of SIH should be considered in EDS patients with postural headache.


1.Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: Clinical review. JAMA 2006;295:2286-96.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Mokri B, Maher CO, Sencakova D. Spontaneous CSF leaks: Underlying disorder of connective tissue. Neurology 2002;58:814-6.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Schievink WI, Gordon OK, Tourje J. Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: Prospective study. Neurosurgery 2004;54:65-71.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Cheuret E, Edouard T, Mejdoubi M, Acar P, Pienkowski C, Cances C, et al. Intracranial hypotension in a girl with Marfan syndrome: Case report and review of the literature. Childs Nerv Syst 2008;24:509-13.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Voermans NC, Dijk KG, Bos MM, Geus-Oei LF, Verrips A, Lindert EJ. Postural headache in Marfan syndrome associated with spinal cysts and liquor hypotension. Neuropediatrics 2009;40:201-4.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]

Copyright 2011 - Neurology India

The following images related to this document are available:

Photo images

[ni11190f2.jpg] [ni11190f1.jpg]
Home Faq Resources Email Bioline
© Bioline International, 1989 - 2024, Site last up-dated on 01-Sep-2022.
Site created and maintained by the Reference Center on Environmental Information, CRIA, Brazil
System hosted by the Google Cloud Platform, GCP, Brazil