Spontaneous intracranial hypotension: diagnostic and therapeutic workup

AbstractSpontaneous intracranial hypotension (SIH) is an orthostatic headache syndrome with typical MRI findings among which engorgement of the venous sinuses, pachymeningeal enhancement, and effacement of the suprasellar cistern have the highest diagnostic sensitivity. SIH is in almost all cases caused by spinal CSF leaks. Spinal MRI scans showing so-called spinal longitudinal extradural fluid (SLEC) are suggestive of ventral dural tears (type 1 leak) which are located with prone dynamic (digital subtraction) myelography. As around half of the ventral dural tears are located in the upper thoracic spine, additional prone dynamic CT myelography is often needed. Leaking nerve root sleeves typically associated with meningeal diverticulae (type 2 leaks) and CSF-venous fistulas (type 3 leaks) are proven via lateral decubitus dynamic digital subtraction or CT myelography: type 2 leaks are SLEC-positive if the tear is proximal and SLEC-negative if it is distal, and type 3 leaks are always SLEC-negative. Although 30–70% of SIH patients show marked improvement following epidural blood patches applied via various techniques definite cure mostly requires surgical closure of ventral dural tears and surgical ligations of leaking nerve root sleeves associated with meningeal diverticulae or CSF-venous fistulas. For the latter, transvenous embolization with liquid embolic agents via the azygos vein system is a novel and valuable therapeutic alternative.

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Comparisons Between Heavily T2-Weighted MR and CT Myelography Studies in Two Patients with Spontaneous Intracranial Hypotension

Cephalalgia ◽

10.1111/j.1468-2982.2008.01562.x ◽

2008 ◽

Vol 28 (6) ◽

pp. 653-657 ◽

Cited By ~ 5

Author(s):

P-H Tsai ◽

J-L Fuh ◽

J-F Lirng ◽

S-J Wang

Keyword(s):

Nerve Root ◽

Intracranial Hypotension ◽

Large Scale ◽

Scale Validation ◽

Spontaneous Intracranial Hypotension ◽

Time Saving ◽

Ct Myelography ◽

Computed Tomographic ◽

Csf Leakage ◽

Mr Myelography

Our previous study demonstrated that heavily T2-weighted magnetic resonance (MR) myelography could successfully detect abnormal cerebrospinal fluid (CSF) collections in patients with spontaneous intracranial hypotension (SIH); however, its ability to demonstrate ongoing CSF leakage sites is uncertain. Currently, computed tomographic (CT) myelography is still considered the study of choice for such a purpose. In this study, we compared the results of the two imaging modalities in two patients with SIH. CSF leakages, such as C1-2 extraspinal collections, CSF along nerve root sleeves, and epidural fluid collections were noted on both studies with minor discrepancies. CSF collection along nerve root sleeves demonstrated by MR myelography was the most likely ongoing leakage site in comparison with CT myelography. Targeted epidural blood patches in both patients resulted in complete headache resolution. Noninvasive, non-contrasted and time-saving MR myelography is a promising study for patients with SIH. Further large-scale validation studies are needed.

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Comparisons of clinical characteristics, brain MRI findings, and responses to epidural blood patch between spontaneous intracranial hypotension and post-dural puncture headache: retrospective study

BMC Neurology ◽

10.1186/s12883-021-02279-5 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Gha-Hyun Lee ◽

Jiyoung Kim ◽

Hyun-Woo Kim ◽

Jae Wook Cho

Keyword(s):

Intracranial Hypotension ◽

Dural Puncture ◽

Epidural Blood Patch ◽

Brain Mri ◽

Spontaneous Intracranial Hypotension ◽

Vein Of Galen ◽

Mri Findings ◽

Post Dural Puncture Headache ◽

Straight Sinus ◽

Blood Patch

Abstract Background Spontaneous intracranial hypotension and post-dural puncture headache are both caused by a loss of cerebrospinal fluid but present with different pathogeneses. We compared these two conditions concerning their clinical characteristics, brain imaging findings, and responses to epidural blood patch treatment. Methods We retrospectively reviewed the records of patients with intracranial hypotension admitted to the Neurology ward of the Pusan National University Hospital between January 1, 2011, and December 31, 2019, and collected information regarding age, sex, disease duration, hospital course, headache intensity, time to the appearance of a headache after sitting, associated phenomena (nausea, vomiting, auditory symptoms, dizziness), number of epidural blood patch treatments, and prognosis. The brain MRI signs of intracranial hypotension were recorded, including three qualitative signs (diffuse pachymeningeal enhancement, venous distention of the lateral sinus, subdural fluid collection), and six quantitative signs (pituitary height, suprasellar cistern, prepontine cistern, mamillopontine distance, the midbrain-pons angle, and the angle between the vein of Galen and the straight sinus). Results A total of 105 patients (61 spontaneous intracranial hypotension patients and 44 post-dural puncture headache patients) who met the inclusion criteria were reviewed. More patients with spontaneous intracranial hypotension required epidural blood patch treatment than those with post-dural puncture headache (70.5% (43/61) vs. 45.5% (20/44); p = 0.01) and the spontaneous intracranial hypotension group included a higher proportion of patients who underwent epidural blood patch treatment more than once (37.7% (23/61) vs. 13.6% (6/44); p = 0.007). Brain MRI showed signs of intracranial hypotension in both groups, although the angle between the vein of Galen and the straight sinus was greater in the post-dural puncture headache group (median [95% Confidence Interval]: 85° [68°-79°] vs. 74° [76°-96°], p = 0.02). Conclusions Patients with spontaneous intracranial hypotension received more epidural blood patch treatments and more often needed multiple epidural blood patch treatments. Although both groups showed similar brain MRI findings, the angle between the vein of Galen and the straight sinus differed significantly between the groups.

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The Syndrome of Spontaneous Intracranial Hypotension

Cephalalgia ◽

10.1046/j.1468-2982.1999.019002080.x ◽

1999 ◽

Vol 19 (2) ◽

pp. 80-87 ◽

Cited By ~ 40

Author(s):

CP O'Carroll ◽

M Brant-Zawadzki

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Subdural Effusion ◽

Resonance Imaging ◽

Mri Findings ◽

The Past ◽

Downward Displacement ◽

Prior Literature ◽

Magnetic Resonance Imaging Mri

The authors report four cases of headache and other symptomatology related to the syndrome of intracranial hypotension. They were seen in a routine clinical practice over the past 3 years. The clinical features, magnetic resonance imaging (MRI) findings, and follow-up of these patients are described. Review of the prior literature on the topic is also included. All four patients presented with orthostatic headache syndrome. Three of the four demonstrated diffuse leptomeningeal thickening and enhancement on MRI studies. One subsequently developed a subdural effusion. One patient demonstrated downward displacement of the posterior fossa initially, which resolved on follow-up MRI scanning. Possible pathophysiologies of the syndrome are discussed.

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Lateral decubitus digital subtraction myelography to identify spinal CSF–venous fistulas in spontaneous intracranial hypotension

Journal of Neurosurgery Spine ◽

10.3171/2019.6.spine19487 ◽

2019 ◽

Vol 31 (6) ◽

pp. 902-905 ◽

Cited By ~ 7

Author(s):

Wouter I. Schievink ◽

M. Marcel Maya ◽

Franklin G. Moser ◽

Ravi S. Prasad ◽

Rachelle B. Cruz ◽

...

Keyword(s):

Prone Position ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Lateral Decubitus Position ◽

Csf Leak ◽

Digital Subtraction ◽

Spinal Imaging ◽

Lateral Decubitus ◽

Venous Fistula ◽

Digital Subtraction Myelography

OBJECTIVESpontaneous spinal CSF–venous fistulas are a distinct type of spinal CSF leak recently described in patients with spontaneous intracranial hypotension (SIH). Using digital subtraction myelography (DSM) with the patient in the prone position, the authors have been able to demonstrate such fistulas in about one-fifth of patients with SIH in whom conventional spinal imaging (MRI or CT myelography) showed no evidence for a CSF leak (i.e., the presence of extradural CSF). The authors compared findings of DSM with patients in the lateral decubitus position versus the prone position and now report a significantly increased yield of identifying spinal CSF–venous fistulas with this modification of their imaging protocol.METHODSThe population consisted of 23 patients with SIH who underwent DSM in the lateral decubitus position and 26 patients with SIH who underwent DSM in the prone position. None of the patients had evidence of a CSF leak on conventional spinal imaging.RESULTSA CSF–venous fistula was demonstrated in 17 (74%) of the 23 patients who underwent DSM in the lateral decubitus position compared to 4 (15%) of the 26 patients who underwent DSM in the prone position (p < 0.0001). The mean age of these 16 women and 5 men was 52.5 years (range 36–66 years).CONCLUSIONSAmong SIH patients in whom conventional spinal imaging showed no evidence of a CSF leak, DSM in the lateral decubitus position demonstrated a CSF–venous fistula in about three-fourths of patients compared to only 15% of patients when the DSM was performed in the prone position, an approximately five-fold increase in the detection rate. Spinal CSF–venous fistulas are not rare among patients with SIH.

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False localizing signs of spinal CSF–venous fistulas in spontaneous intracranial hypotension: report of 2 cases

Journal of Neurosurgery Spine ◽

10.3171/2019.4.spine19283 ◽

2019 ◽

Vol 31 (5) ◽

pp. 764-767 ◽

Cited By ~ 2

Author(s):

Wouter I. Schievink ◽

M. Marcel Maya ◽

Franklin G. Moser

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

The Other ◽

Ct Scans ◽

Csf Leak ◽

Digital Subtraction ◽

Glue Injection ◽

Venous Fistula ◽

Digital Subtraction Myelography ◽

Treatment Surgery

A spinal CSF–venous fistula is one of three specific types of spinal CSF leak that can be seen in patients with spontaneous intracranial hypotension (SIH). They are best demonstrated on specialized imaging, such as digital subtraction myelography (DSM) or dynamic myelography, but often they are diagnosed on the basis of increased contrast density in the draining veins (the so-called hyperdense paraspinal vein sign) on early postmyelography CT scans. The authors report on 2 patients who underwent directed treatment (surgery in one patient and glue injection in the other) based on the hyperdense paraspinal vein sign, in whom the actual site of the fistula did not correspond to the level or laterality of the hyperdense paraspinal vein sign. The authors suggest consideration of DSM or dynamic myelography prior to undertaking treatment directed at these fistulas.

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