Intracranial hypertension: Beyond CSF. Diagnosis and treatment

A variable degree of intracranial hypertension (ICH) is a common affliction amongst patients with myalgic encephalomyelitis  / chronic fatigue syndrome (Higgins 2013, 2015, 2017; Hulens 2018), vestibular dysfunction (Higgins 2015, Liu 2019), endolymphatic hydrops (Ranieri 2017), chronic headache or migraine (Digre 2002), and [pulsatile] tinnitus (Chiarella 2012). Yet, the majority of these patients remain undiagnosed and continue to suffer. The main reason for this, is that the body may quite subtly demonstrate intracranial hypertension on imaging studies, despite often obvious clinical symptoms. This article will briefly discuss some common causes of intracranial hypertension, its variants, and potential treatment strategies.

“Idiopathic” intracranial hypertension, especially, is a common but underdiagnosed problem that is postulated to mainly affect obese women in child-bearing age. Empirically, I’ve found that other patients also have ICH, but develop secondary CSF leaks (Osborn’s brain 2nd ed., p1144; Higgins 2014, 2019; Perez 2013; Alkhotani 2019; Bidot 2019; Morki 2002) and therefore do not test positive for papilledema and elevated lumbar punctures. IIH is diagnosed when there is no clear cause for the elevated CSF pressures, yet most patients with IIH are known to demonstrate venous anomalies that reduce cranial venous outflow. Pseudotumor cerebri is another term for ICH which implies that the CSF elevations are secondary to another pathology, for example venous sinus stenosis or thrombosis. As we will discuss in this article, lacking CSF indicators does not rule out intracranial hypertension, as they are unreliable due to frequent secondary leakage, and because they do not cover the important concomitant craniovascular hypertensive aspect (Larsen 2018, 2020) that comes with venous drainage impairment.

Acute versus chronic ICH

Acute variants of ICH are easily diagnosed in hospital settings, as the body does not have time to compensate. Obstructive hydrocephalus (aqueduct stenosis), tumors, subdural hematomae or meningitis are common acute or unbearably expansive pathologies that will almost certainly result in pathological elevation of cerebrospinal fluid pressures and papilledema. Because papilledema and high CSF pressures are the main diagnostic indicators for pathological CSF pressures, these patients tend to be easily diagnosed. Geeraerts (“Non-invasive assessment of intracranial pressure using ocular sonography in neurocritical care patients”; 2008) found that, in intensive care settings, ie., generally acute settings, rapid dilation of the optic nerve sheaths may be noted due to acutely elevated CSF pressures. They found that an optic nerve sheath diameter greater than 5,8 mm Ø correlated with approximately 25 cm H2O CSF pressures, and make it easier and quicker for clinicians to determine when to schedule the patient for shunting or craniectomy.

Fig. Obstructive hydrocephalus

Blood clots in the cerebral venous drainage system, also called dural sinus thrombosis, is a known potential cause of intracranial hypertension and even hydrocephalus. However, in many circumstances, clots in the venous system may not severely affect CSF pressures, but may still greatly impair cerebral blood drainage and thus increase the craniovascular pressures despite the CSF pressures being normal or borderline. A very large venous sinus thrombosis will usually cause a venous infarct, but not always. Actually, up to 50% of clots may occur without secondary venous infarcts (Skalina T, Gaillard F. Cerebral venous thrombosis. Web article. Available from:; Rodallec MH, et al. Cerebral venous thrombosis and multidetector CT angiography: tips and tricks. 2006). A positive MR or CT venogram (demonstrating severe venous obstruction) without a compatible infarct will very frequently be misdiagnosed as a normal venous anomaly (venous variant) despite the patient’s compatible symptoms, and he or she will be sent home.

Mechanical stenosis (narrowing) of the venous sinuses, especially the transverse venous sinuses is yet another phenomenon causing great confusion. Literature has suggested that up to 50% of sinuses may be “idiopathically stenosed”, ie. narrowed. However, how reliable is this? A cranio-venographic study is rarely done unless significant suspicion already forelies with regards to craniovenous pathology. Most of these studies are done due to compatible symptoms, and rarely does there forelie pre-existing venographic images for comparison. In other words, if the scan comes back showing obstruction, but there is no infarct, and no compelling signs of CSF pressure increases (which are unreliable, more on this later), the imaging study will almost definitely be deemed normal. However, the only reliable way to know if the venous obstruction is a normal variant, is either 1. to have pre-existing venograms (prior to symptom onset) that shows similar appearance, or 2. to perform a catheter venography and manometry to ensure that the intradural venous pressures are low and relatively symmetrical, and that the stenotic site can be easily examined with the catheter (ie. without resistance upon catheter entry to stenosed segment). Difficulty entering the stenosed site suggests thrombosis.

Some common risk factors for venous sinus thrombosis include oral contraceptives, hypercoagulability, infection, malignancy, and pregnancy. If gross sinus obstruction is evident on MRI, the patient has obstructed jugular outlets and/or other risk factors, and of course, acute onset of symptoms, the likelihood that the MRV findings are normal variants, is low. Catheter manometry should be done.

Fig. Cerebral venous sinuses

CSF versus craniovascular pressures

Almost all diagnostic measures in the detection of intracranial hypertension are based on CSF pressure markers. Unfortunate, this is very unreliable. First of all, because many if not most of chronic intracranial hypertension sufferers develop secondary CSF leaks through minor (secondary) dural defects or through defects (again, secondary to pressure increase) in the maxillary, ethmoid, frontal, sphenoid or mastoid sinuses. This makes the patient drip CSF and thus the CSF pressures will reduce to where it is borderline high or at the high end of normal ranges.

Fig. Textbook appearance of intracranial hypotension due to CSF leak.

Venous stenosis has been shown to highly associated with intracranial hypertension, as is elevated dural sinus pressures by catheter manometry (De simone, Advancement in idiopathic intracranial hypertension pathogenesis: focus on sinus venous stenosis, 2010). Although not commonly understood, chronic craniovenous drainage insufficiency will result in both elevations of CSF pressures as well as craniovascular pressures. This is why CSF shunting a patient with intracranial hypertension will not have curative effect if it is venogenic, ie. if it is originating from chronic venous insufficiency, but it may be curative if the patient suffers from obstructive hydrocephalus, for example.

If the patient has an underlying venous pathology that is not being detected, the patient may or may not develop significant indicators of elevated CSF. For example, stenosis or thrombosis of the superior sagittal sinus, which is the main drainage pathway for CSF, will almost inevitably result in papilledema and elevated lumbar punctures, as well as possible hydrocephalus. Venography will be indicated unless other causes of hydrocephalus are already seen. However, if one transverse sinus is obstructed, especially the hypoplastic one, this may not be enough of a problem to cause significant CSF drainage impairment, but will certainly reduce blood drainage in that hemisphere and therefore increase the likelihood for ipsilateral migraine, vestibular dysfunction, tinnitus, etc., due to consequent vascular congestion on that side. Venography should still be done. I reiterate; craniovenous drainage deficiency, indicated by stenosed segments identified upon MR or CT venography, will to a variable degree increase the intracranial blood pressures, regardless of whether or not the CSF pressures appear normal. The actual venous pressures can be determined by catheter manometry, if venography revealed stenosed segments. High venous pressures with compatible symptoms, and lacking markers for CSF pressure elevation, should not automatically be rendered as a coincidental finding.

Fig. left-sided transverse sinus thrombosis. A compatible white-vessel sign also seen on axial T1-weighted images.

Another virtually unknown cause of craniovascular hypertension is thoracic outlet syndrome. TOS, with or without symptoms of brachial arterial insufficiency, may induce what I have called a secondary craniovascular hyperperfusion phenomenon (TOS CVH). It is a fancy word that means that the blood that is prevented from entering the arm in TOS, rather reverts to the head through the vertebral and common carotid arteries, resulting in chronic hypersaturation and dilation of the cranial arteries. This is damaging to the brain’s vasculature and also causes autoregulation impairment. Therefore, another protective response is initiated. Just like excessive CSF pressures may narrow the intracranial arteries and cause an ischemic stroke in ICU settings, low or comparatively low CSF pressures will allow hyperdilation of the intracranial arteries in TOS CVH. Increasing the CSF pressures will prevent hyperdilation from TOS CVH, but will, over time, result in “idiopathic” intracranial hypertension (IIH). Early studies (Pickering 1934, 1952) show that patients with essential (primary) hypertension also developed, seemingly compensatory, increases in CSF pressures, whereas patients with primary CSF hypertension, did not. I strongly believe that this is a protective reaction to prevent arterial hyperdilation. If a patient with significant CVH develops a secondary CSF leak, which are usually asymptomatic, they will develop POTS as the arteries are now allowed to hyperdilate and will be difficult to saturate when being upright. Degree of orthostatic incompetence depends on how impaired the cerebral autoregulation is and how hyperdilated the arteries are. Therefore, I postulate that a scalenectomy may be a better treatment for true IIH (presuming the venous system truly is normal and not merely misinterpreted as normal), than shunting.

Fig. Compression of the distal subclavian artery will increase peripheral resistance in the thoracic outlet, and force increased blood flow towards the head through the vertebral and common carotid arteries. This phenomenon is worse when lying down and better when upright. It may impair cerebro autoregulation, hyperdilate the arteries and induce secondary increased CSF pressures to protect against the hyperperfusion-induced arterial hyperdilation.

An investigation into the factors that might be responsible for the raised intracranial pressure in albuminuric retinitis detected only two, namely, the degree of anemia, and the degree of hypertension.24 The relationship between cerebrospinal fluid pressure and diastolic arterial pressure is shown in figure 3 and is statistically significant. Brain’s29 observations on cerebral tumor showed no relationship between the degree of raised intracranial pressure and the arterial pressure, and it would seem, therefore, that in some way the raised cerebrospinal fluid pressure is a consequence of a sufficiently severe hypertension. Volhard (personal communication) suggested that this relationship was due to ischemic cerebral damage, but the protein concentrations in the cerebrospinal fluid were very little different in the two series. It would seem more likely that the level of arterial pressure is in part transmitted to the choroid plexus, owing to the poor contractility of the cerebral arteries. Curiously enough, when raised cerebrospinal fluid pressure is a consequence of raised venous pressure, as it may be in cardiac failure or superior caval obstruction, papilledema does not usually occur, perhaps because in this instance intraocular and intracranial pressure may be equally affected by the same cause.30 – Pickering, 1952

“Idiopathic” intracranial hypertension

Idiopathic means “without known cause”. That does not mean that there is no cause. IIH is often misdiagnosed due to improper interpretation of the craniovenous system. For example, if thrombosis of one lateral sinus without adherent venous infarct is deemed a “normal variant”, “normal hypoplasia”, despite elevated CSF pressures and clear signs of IIH, then the patient may be improperly scheduled for CSF shunting rather than being put on anticoagulative treatment (thrombolytic treatment), balloon venoplasty or stenting.

In incidences where the dural sinuses truly appear normal, the jugular outlet should be examined. In many circumstances, severe jugular outlet obstruction will be noted. Jugular outlet obstruction by the styloid process or C1 transverse process is a common problem. Jayaraman et al. 2012 found that up to 30% of patients undergoing neurovascular workups (MRA) demonstrated internal jugular vein stenosis. Ding et al. 2019 found that 70% of patient with cervical spondylosis had some degree of uni- or bilateral jugular vein stenosis. Ahn et al. 2014, interestingly, found that up to 70% of patients with thoracic outlet syndrome also had internal jugular vein stenosis! In my clinical experience, there is a very high prevalence of TOS in ICH patients.

Fig. Internal jugular vein compression by the C1. The degree of compression is often better demonstrated with TOF (time of flight, non-contrast sequences) as the signal will attenuate according to actual flow reduction. In the contrasted scans, normal signal continues post-stenosis and therefore the degree of stenosis will have to be measured while signal strength should be disregarded.

If the jugular outlet appears obstructed on CT venography (A CT is preferable, as the styloid process is difficult to reliably visualize on MRI), a styloidectomy, transversectomy or jugular stenting can be done. Venous stents tend to increase risk of thrombosis (clotting) and this can be lethal in certain circumstances. Therefore, all other options should be done prior to stenting, such as balloon venoplasty and the before-mentioned.

Fig. The dominant internal jugular vein is crushed between the styloid process and C1’s transverse process, clearly demonstrated on this CT venogram. The patient did not demonstrate papilledema on fundus exams, but showed signs of AV nicking and copper wiring, which are early signs in chronic hypertensive retinopathy. 

If both the dural sinuses as well as jugular outlets are indeed completely normal, then TOS CVH is the most likely cause of the patient’s IIH (as explained above). Usually along with severe anxiety or whiplash, as both of these co-morbidities cause TOS. TOS is an undiagnosed epidemic amongst patients with chronic pain and its symptomology is all over the spectrum.

Common imaging findings in ICH

Osborn’s brain states, correctly, that you’ll often only find one single element of these findings. Yet, most radiologist will not diagnose ICH unless many and obvious CSF pathology indicators are seen on imaging. This is rarely seen, and ICH is very underdiagnosed! Therefore, it is the clinician’s job to render clinical suspicion and to interpret the images with greater care. It is nearly impossible for the radiologist do to this, as they do not work with the patients and therefore cannot build proper clinical suspicion. Therefore, it is and must be the clinician’s job: He or she must both examine the patient and review the images to render the diagnosis. Preferably, in cooperation with an open-minded radiologist that understands that book-knowledge does not carry over perfectly to clinical settings.

Common diagnostic findings in ICH, suggestive of increased CSF pressures, are lateral ventricular narrowing (“slit ventricles”; suggestive of brain swelling), pituitary concavity or an empty sella, posterior orbital flattening, increased optic nerve sheath diameter => 5,8 mm but preferably greater than 7mm, cerebellar descent through the foramen magnum (often borderline, and not frank Chiari). Dilation of the ventricles generally suggests a large problem with the superior sagittal sinus, the dominant transverse sinus, or aqueductal obstruction. The increased intraventricular pressures often result in periventricular edemae (also known as transependymal edema). The transverse process of the C1 will obstruct the jugular foramen on sagittal images, preferably black-blood sequences with 3mm slice thickness. Moreover, there may or may not be a white-vessel sign in the distal sigmoid sinuses, suggestive of severe flow stasis or thrombosis.

Fig. Distended optic nerve sheaths with orbital flattening and papilledema, empty sella, and concomitant venous sinus stenosis. A “textbook” appearance of pseudotumor cerebri.

Secondary cerebrospinal leaks, common in intracranial HYPERtension

Chronic elevations in cerebrospinal fluid pressures result in CSF leaks. This is not well known, but is still stated black on white in Osborn’s brain 2nd ed (p. 1144). Chronic CSF increases causes small defects in the thin and porous bony plates of the sinuses and thus may result in minor meningoceles or leakages. Unfortunately, because nearly all ICH imaging-indicators are based on CSF pressures, a CSF leak will reverse all or most of these signs. This is why a venography is important also when the plain head MRI appears normal. Moreover, rendering the venography as a normal variant, if it does demonstrate anomalies, may be easy to do if the plain head MRI is normal, but unfortunate and premature if there are compatible symptoms. Rather, a catheter venogram and manometry should be done to measure the venous sinus pressures, presuming that the signal loss is within the dural sinus system. If the obstruction is at the skull base by the C1 or styloid process, this is never a normal anomaly and should not be interpreted as one.

Imaging signs in CSF leak involve subdural effusions which may be “halo like”; surrounding the brain, or more commonly, only involving the anterior aspects of the brain. The pituitary gland may be convex and swollen, and there may be presence of epidural vein dilation in the spinal canal. Significant sagging of the brain is usually not seen unless the leak is very severe.

In clinical practice, I’ve found that most patients suffering from CSF leaks are symptomatic not due to the leak (as the pressures are not low enough to cause real problems), but due to the underlying elevated blood pressure. The patients who become afflicted with orthostatic incompetence tend to concomitantly suffer from strong TOS CVH, usually along with anxiety or a previous bad whiplash injury. Or, they may have a large leak that needs surgical repair, but in such case, the lumbar puncture will be below reference. Higgins et al. have shown that fixing the cause of ELEVATED pressures will render the body able to automatically repair minor leaks that are seen in secondary CSF leaks due to chronic ICH (Higgins 2014, 2019).

Patients who have been diagnosed with primary leaks should be careful to examine whether or not they have underlying venous congestion. Treating the leak in such a case will not help; rather, it may make you worse. If it works, the improvement will usually be very short-lived. They have no, or poor response to blood patches. Again, I am referring to secondary CSF leak. Most CSF “leakers” that I have consulted with, have underlying severe venous congestion, TOS, and also, usually, a history of anxiety or whiplash. In other terms, their leak is secondary to longstanding high pressure. The leak is usually not primary. It is also important to be aware that numerous, repeated lumbar punctures and blood patches may result in adhesive arachnoiditis, a nasty condition that is very hard to treat. Avoid repeated blood patches unless there is no doubt that the condition is primary and does not have underlying factors of venous drainage compromise.


A GP should always exclude other causes first. This is why the patient does not see a specialist before they see a general practitioner. Compatible symptoms, either sudden (to some extent suggestive of aqueduct stenosis or dural sinus thrombosis) or insidious onset of headache, tinnitus, visual impairment without frank ocular pathology, vestibular dysfunction, headache, dizziness or presyncope when bending down, and more, are common symptoms that render suspicion for a potential intracranial hypertension and warranting further diagnostic studies.

First, one would have to identify the presence as well as the most likely cause of the eventual increased pressure. This is difficult and requires knowledge about clinical neurology as well as radiology. A plain head MRI along with a venogram is a good start. Look for narrowing or dilation of the lateral ventricles, depression or swelling of the pituitary, cerebellar tonsillar descent, dilation of the optic nerve sheaths, orbital flattening, or epidural vein dilation in the spinal canal. Some of these signs are for ICH, some are for leaks.

Signs of severe CSF elevation such as brutally distending optic nerve sheaths, papilledema or hydrocephalus warrants a lumbar puncture. However, the lumbar puncture is usually not helpful in circumstances where plain head MRI findings are borderline normal, despite obvious clinical symptoms (suggesting that these are of craniovascular origin rather than CSF).

If there are signs of leak, the most likely cause is underlying ICH, unless, as stated, the lumbar puncture truly is below reference range and this is a trauma case. Generally, large primary leaks will demonstrate a positive myelography, whereas secondary leaks, even when substantial, will not show a positive myelography. With regards to sampling the leak and confirming the fluid as CSF, false negatives are common. CSF rhinorrhea may have to be sampled several times before finally being deemed CSF.

If venous anomalies are detected on MRV or CTV, then where? If the jugular outlet demonstrates signal loss, follow up with a contrasted venous phase CTV (Run CT 45 seconds after contrast infusion). The patient should not be lying on the head wedge, but rather have the head and neck lying flat (this improves sensitivity, as jugular outlet obstruction to great extent is a postural problem). Look for obstruction of the IJV between the styloid process and C1 lateral mass. Lacking papilledema or high lumbar puncture opening pressure does not mean that the patient is healthy, as the intracranial blood pressures can be very high despite normal or borderline CSF pressures. This can be seen on ultrasound doppler scans (Larsen 2020) either as increased pulsatility (early phase) or systolic dampening (late / severe phase).

If the anomaly is within dural sinuses, it can be hard to know if the lesion is a partially obstructing thrombus, a fully obstructing thrombus, or “mere” stenosis. Perform bloodwork for increased clot risk, and ask the patient whether or not they have any risk factors such as hormonal aberrancy, hormonal supplementation, dehydration at time of onset, stroke risk in family, history of malignancy, smoking, etc. A follow up with catheter venography and manometry allows the clinician to estimate the likelihood of the anomaly being normal anatomy or pathology. If it is truly a normal variant, the manometric pressures will be low (ref. range 2-6 mm Hg; Cheyuo et al. Venous Manometry as an Adjunct for Diagnosis and Multimodal Management of Intracranial Hypertension due to Meningioma Compressing Sigmoid Sinus. 2019) and there will be no significant pressure gradients (according to the literature, less then 10 mm Hg, but probably even less if the stenosis is truly a natural variant). It should be relatively easy to pull the catheter through the stenosed segment. Difficulty pulling it through suggests thrombosis, especially if the patient had acute onset with no compatible history or additional risk factors for thrombogenicity.

Fig. Patient with sudden onset of severe headache reminiscent of thunderclap headache. No compatible history. Was dehydrated and had known hormonal aberrancies. Was diagnosed with left-sided transverse sinus stenosis, but it was not possible to pull the catheter through the stenosed segment. Catheter venography and manometry showed a completely occluded left-distal TS with collateral filling, suggestive of thrombosis. Manometry showed clearly abnormal pressures. This was a sudden thrombosis of the left transverse sinus, misdiagnosed for three years.

In incidences where the images are equivocal, and the clinician is unsure whether or not normal hypoplasia or factual stenosis is the cause of the signal decrease seen on MRV or CTV, a simple volume-flow ultrasound doppler (VF-USD) measurement can be done. This is cheap, and takes 5 minutes. It can also be done in flexion, extension, rotation, etc. Studies show that normal bilateral jugular vein drainage equates to around 700-1200 ml/min in healthy people (Müller 1985, 1988, 1990; Brunhölzl 1990; Özen 2014). Generally, I expect taller patients to bend towards the higher end and shorter patients toward the lower end of normalcy, but this is just empirical data. Özen et al. showed that even hypoplastic sinuses drain approximately 250-350 ml/min when measured with volume flow on USD. The dominant vessel tends to drain between 500-900 ml/min (unilaterally) in healthy patients, empirically. Özen also showed that unilateral flow rates lower than 160ml/min were associated with near-occlusive states on MRV, whereas 55ml/min or less was associated with occlusive thrombosis. Thus, if one wishes to grade the jugular or intracranial venous stenosis, a total flow less than 160ml/min, even if the vessel is hypoplastic, would suggest abnormalcy. Moreover, a flow less than 350 ml/min in the dominant vessel is almost always abnormal. As stated; the total flow should be more than 700 ml/min in healthy adults. Empirically, when lower than 400, the patients tend to be very symptomatic.

Fig. 38 year-old female patient develops thunderclap headache (a common symptom of thrombosis) and was rushed to the hospital. MRV done and deemed normal by four different “expert” neuroradiologists; hypoplasia, despite compatible symptoms and sudden onset. A follow-up USD shows occlusive states (vole flow less than 55ml/min) in the same sinus that was deemed hypoplastic.

If the venous system is normal, suspect a thoracic outlet syndrome-induced craniovascular hyperperfusion phenomenon. Again, it implies that the blood restricted from entering the brachium, reverts to the head through the vertebral and common carotid arteries, causing hypersaturation of the intracranial arterial system. This problem may cause severe headache, fatigue, dizziness, bradycardia especially when supine, tinnitus, etc. (Larsen 2020). The patient may also have pain between their shoulderblades, chest pain, brachialgia or shoulder pain. They will usually demonstrate some degree of myotomal weakness when doing upper extremity strength neurological workups. Halstead’s test can be held for 60 seconds, look for tingling or a pain in the brachial plexus-innervated areas. Roos’ test will be positive within 30 seconds, usually. Morley’s test is usually positive. Raising the arms may improve the patient’s POTS when they stand up but worsen their headache or induce syncope when lying down.

Conservative treatment for intracranial hypertension

Treatment with acetazolamide or beta-blockers may be used to reduce the CSF and blood pressures. Wehn evaluating whether CSF- or cranioarterial pressures are the main contributors to the patient’s symptoms, I recommend a quick trial on acetazolamide 250mg daily (say, 7 days), where good and positive response would suggest CSF hypertension. No improvement, or even worsening would usually indicate cranioarterial pathology and thus cessation of Diamox and continuation with propranolol or similar betablocker. I prefer to start with 20mg of propranolol 2 hours prior to bed time. I don’t recommend the usage of diuretics, especially in patients with concurrent venous pathology as this may increase risk of thrombosis due to preexisting slow outflow, especially in the non-dominant (hypoplastic) sinus (Chavarria-Medina et al., 2016). If the patient has thrombosis, early detection and treatment with thrombolytics is important, before the clot fibroses (hardens), which may happen within six weeks in some circumstances. However, one may still respond to anticoagulative treatment after six weeks. Be aware that anticoagulation, especially with concurrent ICH will increase the risk for brain bleeds.

If the atlas is obstructing the jugular outlet, this may be treated conservatively as seen in my Myalgic Encephalomyelitis article or atlas misalignment article. It is a postural and muscular dysfunction, in most circumstances, which can be ameliorated or even cured with conservative treatment, especially in mild/moderate cases. If the patient suffers from TOS CVH, this may also be treated conservatively (but carefully), especially in mild to moderate incidences. Surgery is more viable in advanced cases. Patients with TOS CVH should avoid lying flat more than necessary, and preferably sleep on a bed wedge. Preferably on their sides. For treatment strategies, read my thoracic outlet syndrome article. Patients with anxiety as a significant comorbidity should also read my muscle-bracing article, as chronic somatic tension increases both vascular and CSF pressures. Anxiety is very, very common amongst these patients and is an amplifying factor in its intensity, development and progression.

Secondary leaks

Patients with symptomatic leaks due to underlying high pressures (lumbar puncture will not be below or at the low end of the reference range) should, in absolute contrast to common belief, not be lying flat. This worsens CVH and thus, slowly but surely, worsens the hyperdilation, damages the brain, and its autoregulative mechanisms. Patients with POTS or similar, again in incidences where the lumbar puncture is NOT below or at the low end of reference, without large traumatic leaks, should lie elevated on a bed wedge. Cerebral blood flow reduces when upright, thus the CVH reduces, preventing progression of the disorder. The patient should sleep and rest on a bed wedge or in a comfortable, inclined chair. The underlying ICH problem, whatever caused it (usually CVH and anxiety, with or without concurrent venous drainage impairment), should be treated simultaneously. Gradually, the pressures will decrease and this will allow the body to repair minor leaks automatically (Higgins 2014,2019). Only very large leaks with obvious imaging findings should warrant surgical repair, usually of traumatic origins.

Surgical options for intracranial hypertension

For jugular outlet obstruction, transversectomy or styloidectomy may be beneficial (Dashti 2012, Higgins 2015, 2017, Li 2019). It may also be done by performing atlantoaxial traction, facet joint alignment and fixation, cf. the work of the renowned neurosurgeon Atul Goel (Goel 2015). If the pathology is intradural, stenosis, balloon venoplasty may be attempted. Stenting can also be attempted, but once again, it increases clotting risk. Scalenectomy with pectoralis minor botox injections may be done for TOS CVH.

Fig. Improvement of venous congestion as well as neurological comorbidities after jugular outlet decompression by styloidectomy, in an ME patient.


  1. Higgins N, Pickard J, Lever A. Lumbar puncture, chronic fatigue syndrome and idiopathic intracranial hypertension: a cross-sectional study. JRSM Short Rep. 2013 Nov 21;4(12):2042533313507920. doi: 10.1177/2042533313507920. PMID: 24475346; PMCID: PMC3899735.
  2. Higgins JNP, Pickard JD, Lever AML. Chronic fatigue syndrome and idiopathic intracranial hypertension: Different manifestations of the same disorder of intracranial pressure? Med Hypotheses. 2017 Aug;105:6-9. doi: 10.1016/j.mehy.2017.06.014. Epub 2017 Jun 24.
  3. Higgins N, Pickard J, Lever A. Borderline Intracranial Hypertension Manifesting as Chronic Fatigue Syndrome Treated by Venous Sinus Stenting. J Neurol Surg Rep. 2015 Nov;76(2):e244-7. doi: 10.1055/s-0035-1564060. Epub 2015 Sep 14.
  4. Hulens M, Rasschaert R, Vansant G, et al. The link between idiopathic intracranial hypertension, fibromyalgia, and chronic fatigue syndrome: exploration of a shared pathophysiology. Journal of pain and research, 2018:11:p3129-3140
  5. Liu X, Di H, Wang J, Cao X, Du Z, Zhang R, Yu S, Li B. Endovascular stenting for idiopathic intracranial hypertension with venous sinus stenosis. Brain Behav. 2019 May;9(5):e01279. doi: 10.1002/brb3.1279. Epub 2019 Apr 4. PMID: 30950244; PMCID: PMC6520302.
  6. Ranieri A, Cavaliere M, Sicignano S, Falco P, Cautiero F, De Simone R. Endolymphatic hydrops in idiopathic intracranial hypertension: prevalence and clinical outcome after lumbar puncture. Preliminary data. Neurol Sci. 2017 May;38(Suppl 1):193-196. doi: 10.1007/s10072-017-2895-8. PMID: 28527079.
  7. Digre KB. Idiopathic intracranial hypertension headache. Curr Pain Headache Rep. 2002 Jun;6(3):217-25. doi: 10.1007/s11916-002-0038-1. PMID: 12003693.
  8. Chiarella G, Bono F, Cassandro C, Lopolito M, Quattrone A, Cassandro E. Bilateral transverse sinus stenosis in patients with tinnitus. Acta Otorhinolaryngol Ital. 2012 Aug;32(4):238-43. PMID: 23093813; PMCID: PMC3468936.
  9. Osborn AG, Hedlund G, Salzman KL.Osborn’s Brain. 2nd edition. Elsevier;2017.
  10. Higgins N, Trivedi R, Greenwood R, Pickard J. Brain slump caused by jugular venous stenoses treated by stenting: a hypothesis to link spontaneous intracranial hypotension with idiopathic intracranial hypertension. J Neurol Surg Rep. 2015 Jul;76(1):e188–e193. doi: 10.1055/s-0035-1555015
  11. Higgins JN et al. Headache, cerebrospinal fluid leaks, and pseudomeningoceles after resection of vestibular schwannomas: efficacy of venous sinus stenting suggests cranial venous outflow compromise as a unifying pathophysiological mechanism. J Neurol Surg B. DOI: 10.1055/s-0039-1677706
  12. Perez MA, Bialer OY, Bruce BB, Newman NJ, Biousse V. Primary Spontaneous Cerebrospinal Fluid Leaks andIdiopathic Intracranial Hypertension. Journal of Neuro-Ophthalmology 2013;33:330–337doi: 10.1097/WNO.0b013e318299c292
  13. Alkhotani A. Cerebrospinal Fluid Rhinorrhea Secondary to Idiopathic Intracranial Hypertension. Case Rep Neurol 2019;11:295–298
  14. Bidot S, Levy JM, Saindane AM, Oyesiku NM, Newman NJ, Biousse V. Do Most Patients With a Spontaneous Cerebrospinal Fluid Leak Have Idiopathic Intracranial Hypertension? Official Journal of the North American Neuro-ophthalmology Society, 01 Dec 2019, 39(4):487-495 DOI: 10.1097/wno.0000000000000761
  15. Mokri B. Intracranial Hypertension After Treatment of Spontaneous Cerebrospinal Fluid Leaks. Mayo Clin Proc. 2002;77:1241-1246
  16. Larsen K. Occult intracranial hypertension as a sequela of biomechanical internal jugular vein stenosis: A case report. Anaesth Pain & Intensive Care 2018;22(2)
  17. Larsen K, Galluccio FC, Chand SK.Does thoracic outlet syndrome cause cerebrovascular hyperperfusion? Diagnostic markers for occult craniovascular congestion. Anaesth pain intensive care 2020;24(1)69-86. DOI:
  18. Geeraerts T, Merceron S, Benhamou D, Vigue B, Duranteau J. Noninvasive assessment of intracranial pressure using ocular sonography in neurocritical care patients. Crit Care. 2008;12(Suppl 2):P117.
  19. Skalina T, Gaillard F. Cerebral venous thrombosis. Web article. Available from:; Rodallec MH, et al. Cerebral venous thrombosis and multidetector CT angiography: tips and tricks. 2006
  20. De Simone R, Ranieri A, Bonavita V. Advancement in idiopathic intracranial hypertension pathogenesis: focus on sinus venous stenosis. Neurol Sci. 2010 Jun;31 Suppl 1:S33-9. doi: 10.1007/s10072-010-0271-z. PMID: 2046458
  21. Pickering GW. The cerebrospinal fluid pressure in arterial hypertension. Clin. Sc. 1: 397, 1934.
  22. Pickering GW. The pathogenesis of malignant hypertension. Circulation. 1952 Oct;6(4):599-612. doi: 10.1161/01.cir.6.4.599. PMID: 12979074.
  23. Jayaraman MV, Boxerman JL, David LM, Haas RA, Rogg JM. Incidence of Extrinsic Compression of the Internal Jugular Vein in Unselected Patients Undergoing CT Angiography. AJNR Am J Neuroradiol. 2012 Aug;33(7):1247-50. doi: 10.3174/ajnr.A2953. Epub 2012 Feb 9.
  24. Ding JY, Zhou D, Pan LQ, Ya JY, Liu C, Yan F, et al. Cervical spondylotic internal jugular venous compression syndrome. CNS Neurosci Ther. 2019;00:1–8.
  25. Cheyuo C, Rosen CL, Rai A, Cifarelli CP, Qaiser R. Venous Manometry as an Adjunct for Diagnosis and Multimodal Management of Intracranial Hypertension due to Meningioma Compressing Sigmoid Sinus. Cureus. 2019;11(6):e4953. Published 2019 Jun 20. doi:10.7759/cureus.4953
  26. Müller HR, Hinn G, Buser MW. Internal jugular venous flow measurement by means of a duplex scanner. J Ultrasound Med. 1990 May;9(5):261-5.
  27. Mueller HR, Casty M, Buser M, Haefele M (1988) Ultrasonic jugular venous flow measurement. J Cardiovasc Ultrasonogr 7:25–29
  28. Müller HR (1985) Quantitative Bestimmung des Blutflusses in der Vena jugularis interna mittels Ultraschall. Ultraschall 6:51–54
  29. Brunhölzl C, Müller HR. [Doppler sonography measurement of jugular vein blood flow]. 1990;19(1):26-9.
  30. Özen Ö, Ünal Ö, Avcu S. Flow volumes of internal jugular veins are significantly reduced in patients with cerebral venous sinus thrombosis. Curr Neurovasc Res. 2014 Feb;11(1):75-82.
  31. Chavarria-Medina M, Barboza MA, Varela E, et al. Transverse Sinus Hypoplasia as a Predisposing Factor for Cerebral Venous Thrombosis. Conference: International Stroke Conference, AHA/ASA, 2016. Los Angeles, CA, USAAt:
  32. Dashti SR, Nakaji P, Hu YC, Frei DF, Abla AA, Yao T, et al. Styloidogenic jugular venous compression syndrome: diagnosis and treatment: case report. Neurosurgery. 2012 Mar;70(3):E795-9. doi: 10.1227/NEU.0b013e3182333859.
  33. Li M, Gao X, Rajah GB, Liang J, Chen J, Yan F, et al. Styloidectomy and Venous Stenting for Treatment of Styloid-Induced Internal Jugular Vein Stenosis: A Case Report and Literature Review. World Neurosurg. 2019 Oct;130:129-132. doi: 10.1016/j.wneu.2019.06.100. Epub 2019 Jun 21.
  34. Higgins JN, Garnett MR, Pickard JD, Axon PR. An Evaluation of Styloidectomy as an Adjunct or Alternative to Jugular Stenting in Idiopathic Intracranial Hypertension and Disturbances of Cranial Venous Outflow . Journal of Neurological Surgery Part B, Skull Base. 2017;78(2):158-163. doi:10.1055/s-0036-1594238.
  35. Goel A. Goel’s classification of atlantoaxial “facetal” dislocation. J Craniovertebr Junction Spine. 2014;5(1):3‐8. doi:10.4103/0974-8237.135206

13 thoughts on “Intracranial hypertension: Beyond CSF. Diagnosis and treatment

  1. R Long says:

    I pray this becomes enforced learning in all healthcare systems… even Chiropractic / “Wellness” Clinics! As you mention, too many suffering patients, dismissed because they get locked into the void of Idiopathic.

    Excellent Work
    I hate there is only 1 of you.

  2. Theresa Lindhurst says:

    Im supposed to see a nurologist soon ive had a mri sounds like your article fiys my brain pressure and other things. Thank u

  3. Danielle Wright says:

    This is a fantastic article! I am an LMT and PTA working in a chiro wellness clinic. I’m also an IIH patient with herniated Chiari. I found this enlightening, and will continue to educate myself in the subject of TOS. Thank you for your wonderful work!

  4. Geo says:

    If a patient is diagnosed with intracranial hypertension and did not respond to acetozolamide, do you think atenolol could be a replacement for the suggested 20 mg propanolol, how many mg in this case?

  5. Bern Pedit says:

    I was sent here by my virtual physical therapist. For nearly three decades I have been plagued with chronic pain and fatigue, and recently I have been hit with constant headache above and behind both eyes, rapidly increasing brain fog, intermittent sharp piercing pain behind my outer right eye, vision loss, severe tinnitus making it difficult to hear, increasing nausea, worsening fatigue, and an increase in my three decade long cervical pain issues. Failed treatments include a spinal stimulator implant, physical therapy (previous to my current therapist), opioids through a pain management contract, chiropractic treatments, blood pressure medications, dry needling and occipital and trigger point nerve block injections. This article gives me hope that I might find another investigative route and ultimately, some relief from this gift from Hell. Thank you!

  6. Jon says:

    What is your opinion on curve correction using weight on top of head to train the musculature also, and free up space for the jugular veins. Is it something that could work?

    • Tomas says:

      I wonder this too. I know curve correction won’t do the trick and postural corrections should be implemented. But I think small weights, when used carefully and messured properly, could help rehabilitate the weak muscles by the same way the exercises do.

  7. Steve German says:

    Outstanding article on IIH! My 18 year old son developed unremitting headache, vision problems and pulsatile tinnitus a year ago. He was initially dx with ocular migraines by a neurologist and prescribed Topamax, and other meds. Did not help. Soon after, he saw a neuro-ophthalmologist who diagnosed IIH. This did not make sense for an 18 year old male, of normal weight. He has since been studied inside and out, from cervical spine to thoracic etc., for CSF leaks, etc. Long-story short, he has seen x2 neuro-ophthalmologists, immunologist, x2 neurologists, ophthalmologists, optometrist, headache specialist, and a vascular specialist. And a shrink. This all started in 1/2022. He has had no less than 10 imaging studies: MRIs, MRV, MRA, CTA, EEG, brain volumetric studies, and spinal tap (opening pressure 18. Lab read fluid as normal, save protein at 64.) The MR imaging was all read as normal too, except the neuro-ophthalmologist and vascular specialist said there might be narrowing of transverse sinus, while all others docs said that there is not. The vascular specialist was going to run testing from femoral artery to sinus to see if its occluded, and if she he planned to do a stent. I hated the idea – seemed too invasive. The doc and I spoke about it, and he said he changed his mind because there is no papilledema. That returned us to square one. In the meantime, my son missed the last six months of high school (somehow still graduated), and took a gap year from college (Berklee College of Music). He’s a jazz guitarist/pianist, but has not been able to practice because of his vision and headaches. I was going to stop his OCD meds again (Luvox). We did it once without change. As a last-ditch effort the vascular surgeon said we should rule out Eagle’s Syndrome aka Styloid Process Syndrome, and ordered CTA studies. We just got result of the CTA scans with MIP and 3d imaging. The doctor has not reviewed (they were available on Friday but he was in surgery all day). We hope to speak with him soon. The latest imaging shows mild atherosclerotic disease in his arch, and “arthrosclerosis disease at the bilateral carotid bulb without flow limiting findings. No flow limiting stenosis or dissection.” He has mild atherosclerotic calcifications on carotid with no aneurism, and a congenital dominant left vertebral artery. The report further notes: “marked aeration of the upper bones including the petrous apices, but no bony dehiscence of the vascular covering, diverticula, or aberrant anatomy.” I found your articles, and finally things are starting to make sense. I have also been studying the actual imaging to see if there is the styloid is elongated, and may be compressing on his carotid. I found it, and think it might be, but in all honesty I have no clue. This illness is causing so much physical and mental anguish. He cannot be with friends, broke up with girl friend, hardly drives and never at night, etc. Even so, in my opinion he has dealt with it like a champ, determined to get better. He needs his life back and the opportunity to launch. Your literature is outstanding. For the first time things came together in my mind. I do not think he has traditional IIH. The only CSF build up or leak was from a lumbar puncture, and followed with a blood patch a week later. He said the headache was the worst from that, but a completely different type of pain. The doctors have talked about a shifting condition going from hypertensive to hypotensive intracranial pressure. He does not fit the picture of a woman of birthing age, that is overweight. I’ve said that from the get-go. I was also not certain it is a venous condition (whether impingement or blockage) because nothing had been read as indicating as much, although the vascular surgeon said he sees narrowing. Now, after reading your article, I believe he has sequela of venous insufficiency and lack of oxygenation to his brain. I’m confused why, at age 18, he has findings of atherosclerotic changes to his arteries. He is so young to have this. I recall his triglycerides and good/bad cholesterol being extremely elevated but more recently they are normal. He had mono six months before this started which may be a red-herring, and we returned from the Caribbean shortly before he became symptomatic. I developed Parsonage-Turner syndrome after having a virus upon return from our trip. I an homozygous for MTHFR, and he has one marker. I do not think his homocysteine levels have been checked, but wouldn’t be surprised if its elevated. We have a strong family history of autoimmune disease, including my mom who has pseudogout and her sed rate runs as high as 150 and is frequently elevated. My brother (age 60) was recently diagnosed with occluded veins/arteries in his leg and has many of the same symptoms as my son. Is this just coincidental – I have no idea. Then, right when I think I figured out what is causing my son to be ill, more testing puts us two steps backwards. I literally spend every night until 4 a.m. reading medical literature trying to figure things out. I’m now worried from his scans that that at age 18 he has atherosclerosis in his carotid bilaterally and his arch (I assume aortic). I’ve looked at genetic and other conditions that may contribute to this, but again I have no idea. Fabry? I want to stop the progression, and return him to his life. Okay, I’ve droned on. I am mostly interested in your thoughts. I believe it was good-fortune to find your article on IIH, and hope you might give us insight. Is this a venous-like process due to impingement of his arteries? Is this consistent with diseased transverse sinus or carotid arteries. Does this have to do with the findings of petrous apices? I feel convinced, rightly or not, it is not due to CSF. Could it be related to thoracic outlet syndrome? Your literature planted that seed. He cannot focus his vision, and complains of bad neck pain, hearing his heart pump in the back of his head and ears, and an awful headache. It does not seem to fit neatly into any box! If you’ve read all this, I thank you profusely, and hope that you will share your insights. We need someone with genuine interest and curiosity if we’re going to run this to ground. I doubt anyone has really studied each and every imaging study done, and formed a holistic opinion and there is only so far I can go without a medical degree. If you choose to, we thank you in advance for providing your esteemed opinions and advice. From a very concerned Dad in Phoenix, Arizona. Thank you. Steve

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