Last updated:
5/29/2024
Years published: 2017, 2020, 2024
NORD gratefully acknowledges Connie Deline, MD, Spinal CSF Leak Foundation, and Wouter I. Schievink, MD, Professor of Neurosurgery, Department of Neurosurgery, Cedars-Sinai, for the preparation of this report.
Summary
Spontaneous intracranial hypotension (SIH) is secondary to a cerebrospinal fluid (CSF) leak at the level of the spine and the resulting loss of CSF volume that bathes the brain and spinal cord. Males and females of all ages are affected, but the diagnosis is more common in females. Annual incidence of 4 per 100,000 may be an underestimate, and the overall prevalence is unknown. It most often results in a new-onset headache that is worse with upright posture, along with other neurologic signs and symptoms. Variability in presenting signs and symptoms, along with low awareness of the disorder, contributes to delayed diagnosis, although this is improving with a growing volume of publications and interest among physicians. Diagnostic imaging is quite specialized, both in techniques and in interpretation. Because there is a structural cause, a hole or defect in the spinal dura (a tough layer of connective tissue) that normally holds cerebrospinal fluid in, it is both treatable and curable. The most common treatment is epidural patching with blood or fibrin sealant, though venous embolization is increasingly used, and surgery is sometimes needed. Outcomes are better with more prompt diagnosis and treatment. Despite good outcomes for many patients, a significant number continue to suffer for years to decades.
The onset of symptoms and signs may be relatively abrupt or more gradual. Patients may be minimally affected or profoundly disabled with limited ability to function while upright. Quality of life measures in this population are very poor. The hallmark of intracranial hypotension is a positional headache. This headache is worse when upright and improves when lying down. It usually occurs within 15 minutes of assuming the upright position and is relieved after lying down within 15-30 minutes, however it may take hours to worsen or improve with change of position. Over time, the positional aspect of the headache tends to lessen and may even disappear. Headache is not universally positional and may be absent. The location of the headache is most often in the back of the head or base of the skull, but can also occur in the front, sides or all over the head. The headache is rarely on just one side of the head. The quality of the headache is often described as a “pulling sensation” from the back of the head to the neck although many other qualities are reported. The severity of the headache can range from mild to very severe and disabling.
Other characteristic symptoms include neck pain, neck stiffness, nausea, vomiting, sensitivity to light and/or sound, sense of imbalance, ringing in the ears, changes in hearing and profound fatigue. Pain between the shoulder blades and into the upper arms is commonly reported. Patients may also experience visual changes, dizziness or vertigo, facial numbness or pain, or changes in taste. Similar to head pain, these symptoms are often more severe with upright posture.
Specific signs are often seen on brain MRI and are described below under the diagnosis section.
Atypical and serious neurologic complications do occur, so prompt recognition and treatment in such cases is important. Rarely, patients can present with signs and symptoms typical of behavioral variant frontotemporal dementia, Parkinsonism, superficial siderosis, ataxia (very unsteady gait) or bibrachial amyotrophy. Spinal cord herniation, cerebral venous thrombosis, stupor or coma, seizures, stroke and death have also been reported.
The underlying cause of spontaneous intracranial hypotension is a loss of cerebrospinal fluid (CSF) volume through a hole or tear in the spinal dura. The dura is the tough outermost layer of the meninges (connective tissues that surround the brain and spinal cord) that holds in the CSF. When this fluid volume is reduced, there is less fluid available to cushion the brain inside the skull. This loss of CSF volume is thought to cause headache and other neurological signs and symptoms and may result in a range of complications. With upright posture, the loss of CSF volume has a greater effect on the brain.
There is evidence that an underlying weakness of the spinal dura is present in a subset of patients. Several genetic disorders of connective tissue have been associated with spontaneous intracranial hypotension. See related disorders below.
Also, many cases are associated with calcified discs and bone spurs of the spine that can tear the dura on the front side or beside the spinal cord.
The etiology of the CSF-venous fistula type of leak, first recognized in 2014, is not yet understood.
CSF leaks that occur spontaneously in the head (base of the skull) are very rarely associated with intracranial hypotension.
Males and females of any age may develop spontaneous intracranial hypotension, but it is diagnosed more often in females. The peak age of diagnosis is age 40.
The prevalence of spontaneous intracranial hypotension is unknown. It is suspected that many cases never present for medical care or resolve without treatment. The estimated annual incidence from two recent studies is about 4 cases per 100,000 per year.
The diagnosis of spontaneous intracranial hypotension is initially suspected based on presenting signs and symptoms. Many physician specialties may be involved in the care of patients, including primary care physicians, emergency medicine physicians, neurologists, neuroradiologists, pain management physicians, anesthesiologists, neurosurgeons and geneticists.
The specific diagnostic code for spontaneous intracranial hypotension is G96.811 and the specific code for spontaneous spinal CSF leak is G96.02.
When this diagnosis is suspected, magnetic resonance imaging (MRI) study of the brain with contrast should be done to look for several specific findings. These findings may be absent in up to 20% of patients, more often when this imaging is done weeks or months after onset. The mnemonic SEEPS is used by physicians to recall the findings:
S – subdural fluid collections
E – enhancement of the meninges (layers around the brain)
E – engorgement of venous structures
P – pituitary hyperemia (swelling)
S – sagging of the brain
The brain SIH (bSIH) score, also known as the Bern score, uses specific qualitative and measurable brain MRI findings to predict which patients are more likely to have a spinal CSF leak visible on spinal imaging. Pachymeningeal enhancement, venous sinus engorgement, and the suprasellar cistern measuring 4.0 mm or less each count as 2 points. Subdural fluid, the prepontine cistern measuring 5.0 mm or less, and the mamillopontine distance measuring 6.5 mm or less each count as 1 point. A score of 5-9 suggests a high probability of positive spinal imaging, while a score of 0-2 suggests low probability, and a score of 3-4 is intermediate. This score is also used in post-treatment evaluation. Note that the bSIH score is not intended to predict the likelihood of a patient having spontaneous intracranial hypotension nor does it correlate with symptom severity. Using a normal brain MRI to rule out spontaneous intracranial hypotension is a common diagnostic error.
An additional imaging finding that is more common in intracranial hypotension than in the general population is layered thickening of the skull, known as layered calvarial hyperostosis.
Optic nerve sheath diameter as measured using ultrasonography or MRI may have some predictive value in the diagnostic process.
A lumbar puncture may be done at the time of myelography to measure the CSF opening pressure, but because this often falls in the normal range, it has limited diagnostic value. CSF outflow resistance and spinal elastance can be measured with CSF infusion testing but evolve with longer duration of symptoms. For some patients, spinal imaging to localize their spinal CSF leak may not be necessary, because one or more epidural blood patch procedures will be curative. See the treatment section below.
Different types of spinal CSF leaks have been observed based on findings of spinal imaging and at surgery. Treatment approaches are then tailored to the type and location of the leak. Type 1 CSF leaks are caused by a dural tear located ventral to (in front of) the spinal cord (type 1a) or posterolateral to (behind and to the side of) the spinal cord (type 1b). Type 2 CSF leaks are associated with simple (type 2a) or complex (type 2b [dural ectasia]) meningeal diverticula. Type 3 CSF leaks are CSF-venous fistulas. Type 4 CSF leaks are of indeterminate origin.
Noninvasive imaging in the form of a full spine MRI is preferred as the initial spinal imaging. The findings on spinal MRI guide the need for additional specialized spinal imaging (myelographic) techniques, including standard or dynamic computed tomography (CT), photon-counting computed tomography (PCCT), conebeam CT, dual energy CT, digital subtraction and intrathecal gadolinium-enhanced MR myelography. The yield of imaging can be impacted by patient positioning and respiratory phase. The identification of the type and location of the spinal CSF leak facilitates further treatment planning.
Artificial intelligence is also being explored to improve the diagnostic accuracy of imaging.
Current imaging techniques, while evolving rapidly, are not sensitive enough to identify a spinal CSF leak in all patients, and negative imaging does not rule out the disorder. When imaging is unsuccessful in locating the spinal CSF leak, treatment options are impacted.
When there are findings on history, clinical exam, or imaging that suggest the presence of a genetic disorder of connective tissue, referral to a physician with expertise in these disorders may be of value. These patients may be at higher risk of cardiovascular abnormalities noted above under “Disorders with Similar Symptoms”. Screening with echocardiography and/or other tests may be considered on a case-by-case basis.
Treatment
An uncertain percentage of patients with spontaneous intracranial hypotension have milder symptoms and/or symptoms that may resolve without any treatment. Simple measures including bed rest, fluids for hydration and caffeine intake may help to reduce the severity of symptoms temporarily. Head pain in intracranial hypotension is rarely responsive to medications used to treat migraine and other headache disorders, although medications for nausea are often needed and helpful. The severity of headache may warrant the judicious use of opiate pain medications.
In the setting of serious complications, such as coma or large subdural hematomas, urgent treatment will be needed.
For less emergent cases, treatment should be directed at the underlying leak without undue delay to achieve better outcomes. A common initial treatment is a non-targeted epidural blood patch. In this procedure, some blood is taken from the patient’s arm vein and is injected into the spinal canal in the space outside the dura using imaging guidance (fluoroscopy, CT, or CT-fluoroscopy). Epidural patching procedures may be targeted to specific spinal levels and often include the use of fibrin sealant. Fibrin sealant may also be injected directly into a CSF-venous fistula or a meningeal cyst to occlude (plug) them. Interventional neuroradiologists and anesthesiologists are physician subspecialties that perform these spinal injection procedures most often. These procedures may be repeated if the improvement is incomplete or does not last.
Embolization of veins around the spine is a relatively new treatment for the CSF-venous fistula type of leak, first described in 2021. This involves threading a catheter from a larger vein into the target veins in the spinal area, then injecting a material that permanently plugs those veins. Short-term outcomes are favorable in carefully selected patients, but additional study is needed to evaluate long-term safety and efficacy.
In some patients, a neurosurgical repair may be the best treatment option but relies upon precise imaging localization of the spinal CSF leak.
Following treatment, it is not uncommon for patients to develop rebound intracranial hypertension (elevated CSF pressure), which may persist for a variable length of time. The natural history and optimal treatment for this complication have not yet been well studied. Currently, acetazolamide is the most commonly used medication.
Most patients do well, although some patients do have relapsing or persistent symptoms with moderate to profound levels of disability. More prompt diagnosis and treatment offer the best outcomes although cures are possible even after years to decades of symptoms.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
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Schievink WI, Maya M, Moser F, Nuño M. Long-term risks of persistent ventral spinal CSF leaks in SIH: superficial siderosis and bibrachial amyotrophy. Neurology. 2021;97(19):e1964-e1970. doi:10.1212/WNL.0000000000012786
Signorelli F, Caccavella VM, Giordano M, Ioannoni E, Caricato A, Polli FM, Olivi A, Montano N. A systematic review and meta-analysis of factors affecting the outcome of the epidural blood patching in spontaneous intracranial hypotension. Neurosurg Rev. 2021 Dec;44(6):3079-3085. doi:10.1007/s10143-021-01505-5
Tay ASS, Maya M, Moser FG, Nuño M, Schievink WI. Computed tomography vs heavily T2-weighted magnetic resonance myelography for the initial evaluation of patients with spontaneous intracranial hypotension. JAMA Neurol. 2021;78(10):1275-1276. doi:10.1001/jamaneurol.2021.2868
Bond KM, Benson JC, Cutsforth-Gregory JK, Kim DK, Diehn FE, Carr CM. Spontaneous intracranial hypotension: atypical radiologic appearances, imaging mimickers, and clinical look-alikes. AJNR Am J Neuroradiol. 2020;41(8):1339-1347. doi:10.3174/ajnr.A6637
Dobrocky T, Winklehner A, Breiding PS, et al. Spine MRI in Spontaneous intracranial hypotension for CSF leak detection: nonsuperiority of intrathecal gadolinium to heavily T2-weighted fat-saturated sequences. AJNR Am J Neuroradiol. 2020;41(7):1309-1315. doi:10.3174/ajnr.A6592
Häni L, Fung C, Jesse CM, Ulrich CT, Miesbach T, Cipriani DR, Dobrocky T, Z’Graggen WJ, Raabe A, Piechowiak EI, Beck J. Insights into the natural history of spontaneous intracranial hypotension from infusion testing. Neurology. 2020 ;95(3):e247-e255. doi:10.1212/WNL.0000000000009812
Kim DK, Brinjikji W, Morris PP, et al. Lateral decubitus digital subtraction myelography: tips, tricks, and pitfalls. AJNR Am J Neuroradiol. 2020;41(1):21-28. doi:10.3174/ajnr.A6368
Wang TY, Karikari IO, Amrhein TJ, Gray L, Kranz PG. Clinical outcomes following surgical ligation of cerebrospinal fluid-venous fistula in patients with spontaneous intracranial hypotension: a prospective case series. Oper Neurosurg (Hagerstown). 2020;18(3):239-245. doi:10.1093/ons/opz134
Amrhein TJ, Kranz PG. Spontaneous intracranial hypotension: imaging in diagnosis and treatment. Radiol Clin North Am. 2019;57(2):439-451. doi:10.1016/j.rcl.2018.10
Beck J, Raabe A, Schievink WI, et al. Posterior approach and spinal cord release for 360° repair of dural defects in spontaneous intracranial hypotension. Neurosurgery. 2019;84(6):E345-E351. doi:10.1093/neuros/nyy312
Chan SM, Chodakiewitz YG, Maya MM, Schievink WI, Moser FG. Intracranial hypotension and cerebrospinal fluid leak. Neuroimaging Clin N Am. 2019;29(2):213-226. doi:10.1016/j.nic.2019.01.002
Dobrocky T, Grunder L, Breiding PS, et al. Assessing spinal cerebrospinal fluid leaks in spontaneous intracranial hypotension with a scoring system based on brain magnetic resonance imaging findings. JAMA Neurol. 2019;76(5):580-587. doi:10.1001/jamaneurol.2018.4921
Farb RI, Nicholson PJ, Peng PW, 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
Fichtner J, Ulrich CT, Fung C, Cipriani D, Gralla J, Piechowiak EI, Schlachetzki F, Z’Graggen WJ, Raabe A, Beck J. Sonography of the optic nerve sheath diameter before and after microsurgical closure of a dural CSF fistula in patients with spontaneous intracranial hypotension – a consecutive cohort study. Cephalalgia. 2019;39(2):306-315. doi:10.1177/0333102418793640
Kranz PG, Gray L, Malinzak MD, Amrhein TJ. Spontaneous intracranial hypotension: pathogenesis, diagnosis, and treatment. Neuroimaging Clin N Am. 2019;29(4):581-594. doi:10.1016/j.nic.2019.07.006
Schievink WI, Maya MM, Moser FG, et al. Lateral decubitus digital subtraction myelography to identify spinal CSF-venous fistulas in spontaneous intracranial hypotension [published online ahead of print, 2019 Sep 13]. J Neurosurg Spine. 2019;1-4. doi:10.3171/2019.6.SPINE19487
Kranz PG, Gray L, Amrhein TJ. Spontaneous intracranial hypotension: 10 myths and misperceptions. Headache. 2018;58(7):948-959. doi:10.1111/head.13328
Schievink WI, Maya MM, Moser FG, Jean-Pierre S, Nuño M. Coma: A serious complication of spontaneous intracranial hypotension. Neurology. 2018;90(19):e1638-e1645. doi:10.1212/WNL.0000000000005477
Schievink WI, Maya MM, Barnard ZR, et al. Behavioral variant frontotemporal dementia as a serious complication of spontaneous intracranial hypotension. Oper Neurosurg (Hagerstown). 2018;15(5):505-515. doi:10.1093/ons/opy029
Amrhein TJ, Befera NT, Gray L, Kranz PG. CT fluoroscopy-guided blood patching of ventral CSF leaks by direct needle placement in the ventral epidural space using a transforaminal approach. AJNR Am J Neuroradiol. 2016;37(10):1951-1956. doi:10.3174/ajnr.A4842
Beck J, Ulrich CT, Fung C, et al. Diskogenic microspurs as a major cause of intractable spontaneous intracranial hypotension. Neurology. 2016;87(12):1220-1226. doi:10.1212/WNL.0000000000003122
Schievink WI, Maya MM, Jean-Pierre S, Nuño M, Prasad RS, Moser FG. A classification system of spontaneous spinal CSF leaks. Neurology. 2016;87(7):673-679. doi:10.1212/WNL.0000000000002986
Pimienta AL, Rimoin DL, Pariani M, Schievink WI, Reinstein E. Echocardiographic findings in patients with spontaneous CSF leak. J Neurol. 2014;261(10):1957-1960. doi:10.1007/s00415-014-7438-0
Reinstein E, Pariani M, Bannykh S, Rimoin DL, Schievink WI. Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study. Eur J Hum Genet. 2013;21(4):386-390. doi:10.1038/ejhg.2012.191
Schievink WI, Maya MM, Louy C, Moser FG, Sloninsky L. Spontaneous intracranial hypotension in childhood and adolescence. J Pediatr. 2013;163(2):504-510. doi:10.1016/j.jpeds.2013.01.055
Schievink WI, Maya MM. Frequency of intracranial aneurysms in patients with spontaneous intracranial hypotension. J Neurosurg. 2011;115(1):113-115. doi:10.3171/2011.2.JNS101805
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