Spontaneous intracranial hypotension (SIH) is a significant cause of chronic, postural headaches. Spontaneous intracranial hypotension is generally believed to be associated with cerebrospinal fluid (CSF) leaks, and these leaks can be posttraumatic, iatrogenic, or idiopathic in origin. An integral part of the management of patients with this condition consists of localizing and stopping the leaks. Radiologists play a central role in the workup of this condition detecting leaks using computed tomography, magnetic resonance imaging, or nuclear imaging. In this article, we briefly review SIH and the various imaging modalities, which can be used to identify and localize a spontaneous CSF leak.
SchaltenbrandG. Neuere anschauungen zur pathophysiologie der liquorzirkulation. Zentralbl Neurochir. 1938;3:290–300.
2.
SchievinkWIRoiterV. Epidemiology of cervical artery dissection. Front Neurol Neurosci. 2005;20:12–15. doi:10.1159/000088125.
3.
LabovitzDLHalimAXBrentBBoden-AlbalaBHauserWASaccoRL. Subarachnoid hemorrhage incidence among whites, blacks and Caribbean Hispanics: the northern Manhattan study. Neuroepidemiology. 2006;26(3):147–150. doi:10.1159/000091655.
4.
OlesenJ. International Classification of Headache Disorders, Second Edition (ICHD-2): current status and future revisions. Cephalalgia. 2006;26(12):1409–1410. doi:10.1111/j.1468-2982.2006.01214.x.
5.
SchievinkWI. Misdiagnosis of spontaneous intracranial hypotension. Arch Neurol. 2003;60(12):1713–1718. doi:10.1001/archneur.60.12.1713.
KranzPGMalinzakMDAmrheinTJGrayL. Update on the diagnosis and treatment of spontaneous intracranial hypotension. Curr Pain Headache Rep. 2017;21(8):37. doi:10.1007/s11916-017-0639-3.
8.
PleasureSJAboschAFriedmanJ, et al.Spontaneous intracranial hypotension resulting in stupor caused by diencephalic compression. Neurology. 1998;50(6):1854–1857. doi:10.1212/wnl.50.6.1854.
9.
SatoYHondaYMaruokaHFujimatsuYOizumiK. Recurrence of spontaneous intracranial hypotension with subdural hematomas. Cephalalgia. 1997;17(1):42–45. doi:10.1046/j.1468-2982.1997.1701042.x.
10.
RussoATessitoreACirilloM, et al.A transient third cranial nerve palsy as presenting sign of spontaneous intracranial hypotension. J Headache Pain. 2011;12(4):493–496. doi:10.1007/s10194-011-0345 -1.
11.
PakiamASLeeCLangAE. Intracranial hypotension with Parkinsonism, ataxia, and bulbar weakness. Arch Neurol. 1999;56(7):869–872.
YousryIFörderreutherSMorigglB, et al.Cervical MR imaging in postural headache: MR signs and pathophysiological implications. AJNR Am J Neuroradiol. 2001;22(7):1239–1250.
14.
ChiappariniLFarinaLD’IncertiL, et al.Spinal radiological findings in nine patients with spontaneous intracranial hypotension. Neuroradiology. 2002;44(2):143–150; discussion 151-152.
15.
SchlosserRJWoodworthBAWilenskyEMGradyMSBolgerWE. Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension. Ann Otol Rhinol Laryngol. 2006;115(7):495–500. doi:10.1177/000348940611500703.
SchievinkWIMayaMMJean-PierreSNuñoMPrasadRSMoserFG. A classification system of spontaneous spinal CSF leaks. Neurology. 2016;87(7):673–679. doi:10.1212/WNL.0000000000002986.
18.
ErossEJDodickDWNelsonKDBoschPLyonsM. Orthostatic headache syndrome with CSF leak secondary to bony pathology of the cervical spine. Cephalalgia. 2002;22(6):439–443. doi:10.1046/j.1468-2982.2002.00385.x.
SchievinkWIGordonOKTourjeJ. Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. Neurosurgery. 2004;54(1):65–70; discussion 70-71. doi:10.1227/01.neu.0000097200.18478.7b.
21.
LloydKMDelGaudioJMHudginsPA. Imaging of skull base cerebrospinal fluid leaks in adults. Radiology. 2008;248(3):725–736. doi:10.1148/radiol.2483070362.
22.
SchievinkWIMayaMMLouyCMoserFGTourjeJ. Diagnostic criteria for spontaneous spinal CSF leaks and intracranial hypotension. AJNR Am J Neuroradiol. 2008;29(5):853–856. doi:10.3174/ajnr.A0956.
23.
LuetmerPHMokriB. Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks. AJNR Am J Neuroradiol. 2003;24(8):1711–1714.
24.
SencakovaDMokriBMcClellandRL. The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology. 2001;57(10):1921–1923. doi:10.1212/wnl.57.10.1921.
25.
WatanabeAHorikoshiTUchidaMKoizumiHYagishitaTKinouchiH. Diagnostic value of spinal MR imaging in spontaneous intracranial hypotension syndrome. AJNR Am J Neuroradiol. 2009;30(1):147–151. doi:10.3174/ajnr.A1277.
26.
OhwakiKYanoEIshiiTTakanashiSNakagomiT. Symptom predictors of cerebrospinal fluid leaks. Can J Neurol Sci. 2008;35(4):452–457.
HannerzJDahlgrenGIrestedtLMeyersonBEricsonK. Treatment of idiopathic intracranial hypotension: cervicothoracic and lumbar blood patch and peroral steroid treatment. Headache. 2006;46(3):508–511. doi:10.1111/j.1526-4610.2006.00383.x.
29.
FichtnerJFungCZ’GraggenWRaabeABeckJ. Lack of increase in intracranial pressure after epidural blood patch in spinal cerebrospinal fluid leak. Neurocrit Care. 2012;16(3):444–449. doi:10.1007/s12028-012-9702-4.
30.
FerranteEArpinoICitterioAWetzlRSavinoA. Epidural blood patch in Trendelenburg position pre-medicated with acetazolamide to treat spontaneous intracranial hypotension. Eur J Neurol. 2010;17(5):715–719. doi:10.1111/j.1468-1331.2009.02913.x.
31.
ChoK-IMoonH-SJeonH-JParkKKongD-S. Spontaneous intracranial hypotension: efficacy of radiologic targeting vs blind blood patch. Neurology. 2011;76(13):1139–1144. doi:10.1212/WNL.0b013e318212ab43.
Cohen-GadolAAMokriBPiepgrasDGMeyerFBAtkinsonJLD. Surgical anatomy of dural defects in spontaneous spinal cerebrospinal fluid leaks. Neurosurgery. 2006;58(4 suppl 2):ONS–238-245; discussion ONS-245. doi:10.1227/01.NEU.0000204712.16099.FB.
34.
HyunSHLeeK-HLeeSJ, et al.Potential value of radionuclide cisternography in diagnosis and management planning of spontaneous intracranial hypotension. Clin Neurol Neurosurg. 2008;110(7):657–661. doi:10.1016/j.clineuro.2008.03.014.
35.
HalaçMAlbayramSCeyhanE, et al.Is early bladder activity in radionuclide cisternography an indirect sign of spontaneous intracranial hypotension or sequence of lumbar puncture?Clin Nucl Med. 2007;32(11):850–853. doi:10.1097/RLU.0b013e318156bb2d.
36.
AraiHYamamotoYMaedaYAgaFDobashiHNishiyamaY. SPET/CT imaging in radionuclide cisternography to detect cerebrospinal fluid leakage in spontaneous intracranial hypotension associated with SLE. Eur J Nucl Med Mol Imaging. 2012;39(7):1225–1226. doi:10.1007/s00259-012-2099-7.
37.
LabadieELvan AntwerpJBamfordCR. Abnormal lumbar isotope cisternography in an unusual case of spontaneous hypoliquorrheic headache. Neurology. 1976;26(2):135–139. doi:10.1212/wnl.26.2.135.
38.
WiesemannEBerdingGGoetzFWindhagenA. Spontaneous intracranial hypotension: correlation of imaging findings with clinical features. Eur Neurol. 2006;56(4):204–210. doi:10.1159/000096487.
39.
JungDJKimJSRyuJS, et al.Radionuclide cisternographic findings in patients with spontaneous intracranial hypotension. Korean J Nucl Med. 1998;31(3):482–489.
40.
MoriyamaEOgawaTNishidaAIshikawaSBeckH. Quantitative analysis of radioisotope cisternography in the diagnosis of intracranial hypotension. J Neurosurg. 2004;101(3):421–426. doi:10.3171/jns.2004.101.3.0421.
41.
SakuraiKNishioMYamadaK, et al.Comparison of the radioisotope cisternography findings of spontaneous intracranial hypotension and iatrogenic cerebrospinal fluid leakage focusing on chronological changes. Cephalalgia. 2012;32(15):1131–1139. doi:10.1177/0333102412459571.
42.
MoriokaTAokiTTomodaY, et al.Cerebrospinal fluid leakage in intracranial hypotension syndrome: usefulness of indirect findings in radionuclide cisternography for detection and treatment monitoring. Clin Nucl Med. 2008;33(3):181–185. doi:10.1097/RLU.0b013e318162d955.
43.
YooH-MKimSJChoiCG, et al.Detection of CSF leak in spinal CSF leak syndrome using MR myelography: correlation with radioisotope cisternography. AJNR Am J Neuroradiol. 2008;29(4):649–654. doi:10.3174/ajnr.A0920.
44.
GoelGRavishankarSJayakumarPN, et al.Intrathecal gadolinium-enhanced magnetic resonance cisternography in cerebrospinal fluid rhinorrhea: road ahead?J Neurotrauma. 2007;24(10):1570–1575. doi:10.1089/neu.2007.0326.
45.
TahirMZKhanMBBashirMUAkhtarSBariE.Cerebrospinal fluid rhinorrhea: an institutional perspective from Pakistan. Surg Neurol Int. 2011;2:174. doi:10.4103/2152-7806.90689.
46.
ShettyPGShroffMMSahaniDVKirtaneMV. Evaluation of high-resolution CT and MR cisternography in the diagnosis of cerebrospinal fluid fistula. AJNR Am J Neuroradiol. 1998;19(4):633–639.
47.
SchievinkWIMayaMMTourjeJMoserFG. Pseudo-subarachnoid hemorrhage: a CT-finding in spontaneous intracranial hypotension. Neurology. 2005;65(1):135–137. doi:10.1212/01.wnl.0000167192.86419.15.
48.
de NoronhaRJSharrackBHadjivassiliouMRomanowskiCA. Subdural haematoma: a potentially serious consequence of spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry. 2003;74(6):752–755. doi:10.1136/jnnp.74.6.752.
49.
SavoiardoMMinatiLFarinaL, et al.Spontaneous intracranial hypotension with deep brain swelling. Brain J Neurol. 2007;130(Pt 7):1884–1893. doi:10.1093/brain/awm101.
50.
RichardSKremerSLacourJ-CVespignaniHBoyerPDucrocqX. Cerebral venous thrombosis caused by spontaneous intracranial hypotension: two cases. Eur J Neurol. 2007;14(11):1296–1298. doi:10.1111/j.1468-1331.2007.01947.x.
51.
RothfusWEDeebZLDaffnerRHProstkoER. Head-hanging CT: an alternative method for evaluating traumatic CSF rhinorrhea. AJNR Am J Neuroradiol. 1987;8(1):155–156.
52.
WangY-FLirngJ-FFuhJ-LHseuS-SWangS-J. Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension. Neurology. 2009;73:1892–1898. doi:10.1212/WNL.0b013e3181c3fd99.
53.
MedinaJHAbramsKFalconeSBhatiaRG. Spinal imaging findings in spontaneous intracranial hypotension. AJR Am J Roentgenol. 2010;195(2):459–464. doi:10.2214/AJR.09.3289.
54.
StoneJACastilloMNeelonBMukherjiSK. Evaluation of CSF leaks: high-resolution CT compared with contrast-enhanced CT and radionuclide cisternography. AJNR Am J Neuroradiol. 1999;20(4):706–712.
55.
CurnesJTVincentLMKowalskyRJMcCartneyWHStaabEV. CSF rhinorrhea: detection and localization using overpressure cisternography with Tc-99m-DTPA. Radiology. 1985;154(3):795–799. doi:10.1148/radiology.154.3.3881800.
56.
StarlingAHernandezFHoxworthJM, et al.Sensitivity of MRI of the spine compared with CT myelography in orthostatic headache with CSF leak. Neurology. 2013;81(20):1789–1792. doi:10.1212/01.wnl.0000435555.13695.22.
57.
LuetmerPHSchwartzKMEckelLJHuntCHCarterREDiehnFE. When should I do dynamic CT myelography? Predicting fast spinal CSF leaks in patients with spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 2012;33(4):690–694. doi:10.3174/ajnr.A2849.
58.
ThielenKRSilleryJCMorrisJM, et al.Ultrafast dynamic computed tomography myelography for the precise identification of high-flow cerebrospinal fluid leaks caused by spiculated spinal osteophytes. J Neurosurg Spine. 2015;22(3):324–331. doi:10.3171/2014.10.SPINE14209.
59.
SmithT. Internal exposure of patients and staff in diagnostic nuclear medicine procedures. J Soc Radiol Prot. 1984;4(2):
45.
60.
HoxworthJMPatelACBoschEPNelsonKD. Localization of a rapid CSF leak with digital subtraction myelography. AJNR Am J Neuroradiol. 2009;30(3):516–519. doi:10.3174/ajnr.A1294.
61.
FarbRINicholsonPJPengPW, et al.Spontaneous intracranial hypotension: a systematic imaging approach for CSF leak localization and management based on MRI and digital subtraction myelography. AJNR Am J Neuroradiol. 2019;40(4):745–753. doi:10.3174/ajnr.A6016.
TosakaMSatoNFujimakiH, et al.Diffuse pachymeningeal hyperintensity and subdural effusion/hematoma detected by fluid-attenuated inversion recovery MR imaging in patients with spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 2008;29(6):1164–1170. doi:10.3174/ajnr.A1041.
64.
FarbRIForghaniRLeeSKMikulisDJAgidR. The venous distension sign: a diagnostic sign of intracranial hypotension at MR imaging of the brain. AJNR Am J Neuroradiol. 2007;28(8):1489–1493. doi:10.3174/ajnr.A0621.
65.
ShankarJJSChakrabortySLumC. The venous hinge—an objective sign for the diagnosis and follow-up of treatment in patients with intracranial hypotension syndrome. Neuroradiology. 2009;51(7):453–456. doi:10.1007/s00234-009-0518-7.
66.
MoayeriNNHensonJWSchaeferPWZervasNT. Spinal dural enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension. Report of three cases and review of the literature. J Neurosurg. 1998;88(5):912–918. doi:10.3171/jns.1998.88.5.0912.
67.
XieTSunWZhangXLiuTDingHHuF, et al.The value of 3D-FIESTA MRI in detecting non-iatrogenic cerebrospinal fluid rhinorrhoea: correlations with endoscopic endonasal surgery. Acta Neurochir (Wien). 2016;158(12):2333–2339. doi:10.1007/s00701-016-2988-9.
68.
HegartySEMillarJS. MRI in the localization of CSF fistulae: is it of any value?Clin Radiol. 1997;52(10):768–770.
69.
JinkinsJRRudwanMKruminaGTaliET. Intrathecal gadolinium-enhanced MR cisternography in the evaluation of clinically suspected cerebrospinal fluid rhinorrhea in humans: early experience. Radiology. 2002;222(2):555–559. doi:10.1148/radiol.2222010249.
70.
AkbarJJLuetmerPHSchwartzKMHuntCHDiehnFEEckelLJ. The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 2012;33(3):535–540. doi:10.3174/ajnr.A2815.
71.
SchievinkWIMayaMMTourjeJ. False localizing sign of C1-2 cerebrospinal fluid leak in spontaneous intracranial hypotension. J Neurosurg. 2004;100(4):639–644. doi:10.3171/jns.2004.100.4.0639.
72.
HattingenEDuMesnilRPilatusURaabeAKahlesTBeckJ. Contrast-enhanced MR myelography in spontaneous intracranial hypotension: description of an artefact imitating CSF leakage. Eur Radiol. 2009;19(7):1799–1799. doi:10.1007/s00330-009-1347-0.
73.
YoshimitsuKVarmaDGJacksonEF. Unsuppressed fat in the right anterior diaphragmatic region on fat-suppressed T2-weighted fast spin-echo MR images. J Magn Reson Imaging JMRI. 1995;5:145–149.
74.
VanopdenboschLJDedekenPCasselmanJWVlaminckSAPA. MRI with intrathecal gadolinium to detect a CSF leak: a prospective open-label cohort study. J Neurol Neurosurg Psychiatry. 2011;82(4):456–458. doi:10.1136/jnnp.2009.180752.
75.
AlbayramSKilicFOzerHBaghakiSKocerNIslakC. Gadolinium-enhanced MR cisternography to evaluate dural leaks in intracranial hypotension syndrome. AJNR Am J Neuroradiol. 2008;29(1):116–121. doi:10.3174/ajnr.A0746.
76.
RayDECavanaghJBNolanCCWilliamsSC. Neurotoxic effects of gadopentetate dimeglumine: behavioral disturbance and morphology after intracerebroventricular injection in rats. AJNR Am J Neuroradiol. 1996;17(2):365–373.
77.
YoonSHChungYSYoonB-WKimJ-EPaekS-HKimD-G. Clinical experiences with spontaneous intracranial hypotension: a proposal of a diagnostic approach and treatment. Clin Neurol Neurosurg. 2011;113(5):373–379. doi:10.1016/j.clineuro.2010.12.015.
