• Mechanical factors combine with an underlying connective tissue disorder to cause the CSF leaks. An orthostatic headache is the prototypical manifestation but other headache patterns occur as well, and associated symptoms are common.
• Once considered an exceedingly rare disorder, recent evidence suggests that spontaneous intracranial hypotension is not that rare and has to be considered an important cause of new daily persistent headaches
• The precise cause of spontaneous spinal CSF leaks remains largely unknown, but an underlying structural weakness of the spinal meninges generally is suspected.¹¹
• The dural weakness predisposes to the formation of dural defects that allow CSF to leak into the epidural space.
• The volume of the CSF leak is quite variable as well, ranging from a minimal amount of seeping CSF only detectable when applying a Valsalva maneuver to large amounts of CSF spontaneously pouring out into the paraspinal soft tissues.
• There is good evidence to suggest that a generalized connective tissue disorder plays a crucial role in the development of spontaneous spinal CSF leaks. First reported in 1994,²⁰ this association has been confirmed by numerous subsequent studies.^11,22- 25
• occipital or suboccipital regions
• “pulling sensation from my head down to my neck,” offering a clue to the diagnosis
• Neck stiffness
• Tinnitus
• Nausea
• headaches that mainly occur at the end of the day, or even paradoxical headaches that worsen when lying down.^49- 50 Intermittent headaches, presumably caused by intermittent spinal CSF leaks, may occur at intervals of weeks, months, or even years.
• Posterior neck pain or stiffness, nausea, and vomiting are the most common, being reported by approximately 50% of patients, and suggest meningeal irritation, particularly when photophobia or phonophobia also is present.^1,11- 12
• Spinal manifestations include interscapular pain or
• those with normal initial MRI findings and a diffuse multilevel spinal CSF leak have a poor prognosis.¹⁰⁰
• Some patients have persistent symptoms following treatment, in spite of documented resolution of CSF leakage.

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• RESULTS:

  The events precede symptoms of SIH were categorized as traumatic, secondary and strictly spontaneous (62%). The location of the spinal CSF leak remains undetectable in approximately 50% of cases reported. In 93% of patients, the CSF leakage sites were detected at the cervical or thoracic level of the spine. On recent MRI studies, 88% of patients showed spinal epidural fluid collections that most likely represent CSF leakage.

• CONCLUSIONS:

  SIH is a condition that typically occurs without a traumatic event, although it can be associated with minor trauma. It occurs when cerebrospinal fluid (CSF) leaks through a focal weakness in the dural sac or meningeal diverticula, resulting in CSF hypovolemia. Patients usually present with an orthostatic headache. The most common brain magnetic resonance imaging findings are diffuse pachymeningeal enhancement, descent of the cerebellar tonsils, and subdural fluid collections. Treatment options range from management of symptoms to surgical repair of the leak

• • Headache
• Alterations in cerebrospinal fluid (CSF) pressure lead to neurologic symptoms, the most common clinical manifestation of which is headache. Typically, the headache is orthostatic and related to traction on pain-sensitive intracranial and meningeal structures, distention on periventricular pain-sensitive areas, and direct pressure on pain conveying cranial nerves. Low CSF headache is a distinct and familiar syndrome

• CONCLUSIONS:

  Since some patients with SIH syndrome have normal CSF pressure and since a downward displacement of the brain due to a reduction of the buoyant action of CSF may induce symptoms, CSF hypovolemia, not intracranial hypotension, may be the cause. Based on the Monro-Kellie doctrine, detecting leaked CSF and venous engorgement (epidural vein dilatation and pachymeningeal enhancement) is an important clue to diagnose so-called SIH syndrome. Dilatation of epidural veins suggests CSF hypovolemia in appropriate conditions

• The 4 variants of misalignment of the Atlas

   

  In the case of misalignment of the Atlas there are 4 basic variants. These variants affect the posture in different ways:

• The vertebral veins are actually part of a large network of veins called the vertebral venous plexus or VVP which is one aspect of the vertebral venous systems. The VVP is located alongside the spinal vertebra within the spinal canal. Even though the veins are small in size they are many in number and extend from the head all the way down to the tail end of the spinal cord. Thus, the drainage capacity of the VVP is huge.

• The Waterfall in the Brain  

  Upright posture causes a waterfall in the brain that follows a steep descent back to the heart. I

• The VVP and Inversion Flows  

  In contrast to most veins, the VVP like the cranial veins, have no valves to check or prevent back flow, known as inversion flows. This allows blood to flow in either direction depending on posture. Standing on one's head, for example causes inversion flows in the VVP toward the brain.
• In addition to inversion flows, upright posture and bipedal motion cause blood to oscillate back and forth within the brain and skull bones similar to walking with a cup full of water.

• The VVP and CSF Flow  

  Lastly, venous pressure in the brain also affects the pressure gradient and thus the flow of cerebrospinal fluid (CSF)

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• Idiopathic intracranial hypertension (IIH) is a disorder of elevated cerebrospinal fluid pressure of unknown cause.
• The signs and symptoms of intracranial hypertension are that the patient maintains an alert and oriented mental state, but has no localizing neurologic findings. There is no evidence of deformity or obstruction of the ventricular system and neurodiagnostic studies are otherwise normal except for increased cerebrospinal fluid pressure
• Neuroimaging signs of increased intracranial pressure include empty sella syndrome, lateral sinus collapse (smooth-walled venous stenoses, Figure 1), flattened globes and fully unfolded optic nerve sheaths. In addition, no secondary cause of intracranial hypertension can be found.
• Clinical Features
• The symptoms of IIH patients are headache (94%), transient visual obscurations (68%), pulse synchronous tinnitus (58%), photopsia (54%), and retrobulbar pain (44%). Diplopia (38%) and visual loss (30%) are less common accompaniments of IIH
• Headache
• The presence of headache is common in patients with IIH and is the usual presenting symptom. The headache profile of the IIH patient³² is that of severe daily pulsatile headaches. They are different from previous headaches, may awaken the patient and usually last hours. The headache is often reported as the worst head pain experienced. Associated nausea is common and vomiting is uncommon
• Transient visual obscurations
• Visual obscurations are episodes of transient blurred vision that usually last less than 30 seconds and are followed by visual recovery to baseline. Visual obscurations occur in about 2/3 of IIH patients.⁸ The symptom may be monocular or binocular. The cause of these episodes is thought to be transient ischemia of the optic nerve head related to increased tissue pressure.
• Pulse-synchronous tinnitus
• Pulsatile intracranial noises or pulse synchronous tinnitus is common in IIH.¹¹ The sound is often unilateral with neither side predominating. In patients with intracranial hypertension, jugular compression or head turning ipsilateral to the sound abolishes it.³⁵ The sound is thought to be due to transmission of intensified vascular pulsations by means of CSF under high pressure and turbulence through smooth walled venous stenoses related to transverse sinus collapse from high CSF pressure
• The major signs of IIH are papilledema and sixth nerve paresis.
• The visual field defects found in IIH are the same types as those reported to occur in papilledema due to other causes. The most common defects are enlargement of the physiologic blind spot and loss of inferonasal portions of the visual field along with constriction of isopters
• Blind spot enlargement is ubiquitous in IIH
• Mechanisms of Visual Loss
• The visual field defects found in IIH patients are optic disc-related. In other words, they are the type found when nerve fiber bundles are damaged at the level of the optic disk. These types of defects also occur with glaucoma and anterior ischemic optic neuropathy. This suggests a common mechanism for the visual loss in these disorders.
• Diagnostic criteria
• These criteria are found in Table 1.

  • see separate web page at http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908600/table/T1/
• Patients with findings on examination other than papilledema, sixth nerve and rarely seventh nerve paresis should be suspected of having a diagnosis other than IIH. Laboratory evaluation in IIH patients is normal except for increased intracranial pressure.
• Treatment
• Once intracranial hypertension is discovered one should first eliminate presumed causal factors such as excessive vitamin A or tetracyclines and begin a low sodium weight reduction diet. Therapy aimed at reversing and preventing visual loss should then be instituted.
• Then symptomatic headache treatment can be introduced if this symptom persists in the face of intracranial pressure lowering agents and procedures. Many treatments both medical and surgical have been used for idiopathic intracranial hypertension with varying success. All reports to date are anecdotal
• Visual loss is the only serious complication and it may occur anywhere from the time of first appearance to many years later. We therefore recommend tailoring the treatment primarily to the presence and progression of visual loss.
• Medical Therapy
• Lumbar Puncture
• Use of repeated lumbar punctures is controversial. Lumbar puncture has only a short-lived effect on CSF pressure⁶⁰ with a return of pressure to pre-tap level after only 82 minutes
• Corticosteroids
• Steroids are still occasionally used to treat IIH but their mechanism of action remains unclear
• Although patients treated with steroids often respond well, there usually is recurrence of papilledema with rapid tapering of the dose. This may be accompanied by marked deterioration of visual function. A prolonged tapering may prevent return of symptoms and signs in some patients. Use of long-term steroids to treat IIH has largely been abandoned.
• Acetazolamide
• McCarthy and Reed⁶² showed acetazolamide (Diamox®) decreases CSF flow but not until over 99.5% of choroid plexus carbonic anhydrase was inhibited. Gücer and Viernstein ⁶³ used intracranial pressure monitoring before and after treatment in four IIH patients. They monitored acetazolamide treatment in two of the patients and showed gradual CSF pressure reduction in both. They only reported the dose in one of the patients (four grams of acetazolamide was needed per day). Apparent efficacy of acetazolamide has also been shown by others
• We start with ½ to 1 gram a day of acetazolamide in divided doses and gradually increase the dose until either symptoms and signs regress, side effects become intolerable or a dose of 3-4 grams per day is reached. Most patients appear to respond in the 1-2 gram per day range.
• The mechanism of action of acetazolamide is likely multifactorial. It has been found to reduce CSF production; also, it changes the taste of foods and sometimes causes anorexia aiding in weight loss.
• Topiramate (Topomax®) has also been used to treat IIH since it has carbonic anhydrase inhibitor activity and weight loss commonly occurs. In studies to date, it appears comparable to acetazolamide
• Furosemide
• Furosemide has also been used to treat IIH.⁷ It has been well documented that furosemide (Lasix®) can lower intracranial pressure.⁶⁹ It appears to work by both diuresis and reducing sodium transport into the brain. We initiate furosemide at a dose of 20 mg p.o. b.i.d. and gradually increase the dose, if necessary, to a maximum of 40 mg p.o. t.i.d. Potassium supplementation is given as needed.
• Surgical Therapy
• Subtemporal or suboccipital decompression
• Optic nerve sheath fenestration
• CSF Shunting Procedures
• Venous sinus stenting
• Treatment Overview
• Medical and surgical treatment of patients with idiopathic intracranial hypertension is often challenging, requiring integration of the history, examination and clinical course. Many factors are involved and each is weighted in creating individualized therapy. The most important factor is usually the amount and progression of visual loss. Next in importance is the severity of the patient's symptoms with regard to how much they are disrupting the patient's activities of daily living. Headache is the most problematic symptom but pulse synchronous tinnitus, and diplopia, can be difficult to treat. Also factored in is the degree and change in papilledema grade. Figure 12 summarizes the options.

  • See Figure at http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908600/figure/F12/
• Patients with mild or no visual loss are treated with modest weight loss and sodium restriction often with the addition of acetazolamide (Diamox®). In cases with no visual loss, the decision on whether to add acetazolamide is based on the severity of headache or other symptoms and the degree of papilledema; the greater the grade of papilledema, the greater the risk for visual loss.
• Patients with mild or no visual loss are usually followed first at 4 month intervals. If there vision improves or papilledema lessens to Grade I or 0, we use 6 month or one year intervals. All patients are encouraged to enter a weight management program with a goal of 5-10% weight loss along with a low salt diet and modest fluid restriction (drinking only when thirsty rather than forcing fluids as is sometimes done to lose weight).
• Headache Treatment
• Headache in IIH patients may improve after a lumbar puncture, but may remain as a management problem even after medications have been given to reduce edema.
• We have had success treating these patients with standard prophylactic vascular headache remedies. However, we try to avoid medications that cause hypotension, such as beta blockers or calcium channel blockers, because they may cause reduced perfusion of the optic nerve head
• Tricyclic antidepressants can be problematic because of their side effect of weight gain. We use tricyclics in very low doses such as amitriptyline 10 - 25 mg at bedtime.
• Nonsteroidal anti-inflammatory agents are used as an adjunct but their use is limited to two days per week to prevent the development of rebound headaches.
• Topiramate may be useful both for its migraine prophylaxis, side effect of weight loss and for carbonic anhydrase inhibition.
• Uncommonly, a CSF shunting procedure is needed for persistent headache; but it can produce the “hindbrain herniation” headache in return. Patients with IIH also have other headache syndromes. Especially in patients with a migraine history, analgesic rebound or caffeine rebound headaches may coexist. These patients may require IV dihydroergotamine to treat this troublesome headache syndrome.

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• Treatment algorithm for idiopathic intracranial hypertension. Visual loss does not include enlargement of the blind spot unless it is compromising vision. Optic nerve sheath fenestration is preferred over steroids.

• Table 1

  <!--caption a7-->

  Differential diagnosis of IIH (cases must meet the modified Dandy criteria of IIH except that a cause is found)
• The table lists the etiologies of intracranial hypertension that meet the modified Dandy criteria except a cause is associated. The highly likely category is a list of cases with many reports of the association with multiple lines of evidence. Probable causes have reports with some convincing evidence. Possible causes have suggestive evidence or are common conditions or medications with intracranial hypertension as a rare association

• Pseudotumor cerebri syndrome, sometimes called idiopathic intercranial hypertension, is a neurological condition characterized by an increase in intercranial pressure, indicating that there is too much cerebrospinal fluid in the skull
• The name of this condition literally translates as “fake brain tumor,” because the symptoms and presentation can mimic those of a brain tumor. Fortunately, pseudotumor cerebri syndrome is far less dangerous than a brain tumor, and there are several options which can be used to treat it to relieve the pressure and address the symptoms.
• It is caused either by an overproduction of cerebrospinal fluid, or by inadequate reabsorption and drainage, which causes the fluid to start to build up in the skull, pressing on the brain and causing some very distinctive symptoms.
• People with pseudotumor cerebri syndrome tend to experience headaches, vision changes, nausea, vomiting, and a pulsing sensation in the head, all classic signs of a large brain tumor. These symptoms in combination are a signal that it is time to go to the doctor, ideally to a neurologist.
• Typically, a neurologist will request a medical imaging study when the patient presents with these symptoms, to get to the bottom of what is causing them. In the case of pseudotumor cerebri syndrome, no tumor will be visible, but there will be an obvious accumulation of fluid in the skull.
• Medications can sometimes be used to reduce the buildup of fluid in the skull, and in some cases, lifestyle changes such as losing weight or ceasing the use of certain medications such as birth control can also address the issue
• Article Details
   
   
   
  •     Written By:  Mary McMahon   
   
  •     Edited By: O. Wallace   
   
  •     Last Modified Date: 03 August 2014   
   
  •     Copyright Protected:
      2003-2014    Conjecture Corporation
• Once treated, pseudotumor cerebri syndrome can recur, in which case patients may need to seek more aggressive treatment options
• If the condition is not treated, it can lead to permanent vision loss or damage as a result of pressure against the eye, which makes it important to receive the proper treatment, even if it may involve some discomfort or lifestyle adjustment.

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• METHODS
• Patients with newly diagnosed IIH were consecutively included from December 2010 to June 2013. Treatment according to standard guidelines was initiated. Headache history was obtained by headache diaries and standardized interviews performed at baseline and after 1, 2, 3 and 12 months. Parallel changes in papilledema were assessed by optical coherence tomography (OCT). All patients had comprehensive neuro-ophthalmological examinations including automated perimetry.
• Forty-four patients were included. Thirty-five patients completed the 12-month follow-up. Dramatic improvement in headache occurred within the first weeks after diagnosis. After 1 year, 15 patients reported no or only infrequent headache. However, 15 of the remaining 20 patients reported sustained chronic headache. Early age of onset and high diagnostic intracranial pressure (ICP) were associated with better headache outcome (≤1 headache days/month) after a year. Papilledema decreased rapidly within the first 2 months of diagnosis. After 1 year, OCT measures had normalized. Visual outcome was excellent in most patients.
• RESULTS
• Although headache in 43% of patients responded well to ICP management, sustained long-term headache was seen in the remaining patients, despite resolution of papilledema. Headache in IIH may thus be attributed to more complex mechanisms than ICP elevation alone. High ICP and young age were associated with better headache outcome. Early treatment according to standard guidelines seems sufficient to ensure excellent visual outcome in the vast majority of patients.
• CONCLUSIONS

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• BMJ Case Rep. 2014 Jun 3;2014. pii: bcr2014203724. doi: 10.1136/bcr-2014-203724.
• Refractory nausea and vomiting in the setting of well-controlled idiopathic intracranial hypertension
• Initial antiemetic therapy included optimisation of IIH therapy by titrating acetazolamide, in addition to using ondansetron and metoclopramide as needed, with minimal relief.
• Summary A 27-year-old woman with a history of recurrent nausea and vomiting in the setting of idiopathic intracranial hypertension (IIH) was admitted for control of unremitting nausea and vomiting
• She was ultimately treated with palonosetron with complete resolution of her acute nausea.
• Nausea, often treated with 5-hydroxytryptamine (5-HT3) receptor antagonists, approved for perioperative and chemotherapy-induced nausea, are used off-label to treat nausea and vomiting outside of those settings. The efficacy of different regimens has been compared in the literature and continues to remain controversial
• When choosing from different 5-HT3 antagonists there are other considerations, in addition to efficacy to consider: dosing schedule, half-life, time of onset, duration and cost-to-benefit ratio, and although one 5-HT3 antagonist may not have been effective, another one may be. In our case palonosetron, with a significantly longer half-life than other 5-HT3 antagonists, was effective in resolving nausea when compared with the more commonly used ondansetron.
• A 27-year-old woman with a history of recurrent nausea and vomiting in the setting of idiopathic intracranial hypertension (IIH) was admitted for control of unremitting nausea and vomiting.
• PMID:

  24895391

  PMCID:

  PMC4054408

  • https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4054408/
• Free PMC Article

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