Idiopathic intracranial hypertension resolves on its own. Benign intracranial hypertension (pseudotumor cerebri). Cerebrospinal Fluid Shunt

Symptoms of intracranial hypertension (including unilateral or bilateral papilledema).
. With lumbar puncture, an increase in intracranial pressure above 200 mm H2O is determined.
. Absence of focal neurological symptoms (with the exception of paresis of the VI pair of cranial nerves).
. Absence of deformation, displacement or obstruction of the ventricular system, other pathology of the brain according to magnetic resonance imaging, with the exception of signs of increased pressure in the cerebrospinal fluid.
. Despite the high level of intracranial pressure, the patient's consciousness is usually preserved.
. The absence of other causes of increased intracranial pressure.
For the first time the syndrome of idiopathic intracranial hypertension is mentioned in 1897 by Quincke. The term "pseudotumor cerebri" was proposed in 1914 by Warrington. Foley in 1955 introduced the name "benign intracranial hypertension" into practice, but Bucheit in 1969 objected to the concept of "benign", emphasizing that for visual functions the outcome of this syndrome may be "poor quality". He proposed the name "idiopathic" or "secondary" intracranial hypertension, depending on whether the pathological condition with which it is associated is known.
Etiology and pathogenesis
The reason for the development of pseudotumor cerebri remains not completely clear, but the occurrence of this syndrome is associated with a number of different pathological conditions, and their list continues to grow. Among them, the most frequently mentioned are: obesity, pregnancy, menstrual disorders, eclampsia, hypoparathyroidism, Addison's disease, scurvy, diabetic ketoacidosis, heavy metal poisoning (lead, arsenic), medications (vitamin A, tetracyclines, nitrofuran, nalidixic acid, oral contraceptives, long-term corticosteroid therapy or its withdrawal, psychotropic drugs), some infectious diseases, parasitic infections (torulosis, trepanosomiasis), chronic uremia, leukemia, anemia (often iron deficiency), hemophilia, idiopathic thrombocytopenic purpura, systemic lupus erythematosus, sarcoidosis, syphilis, Paget's disease, Whipple's disease, Guillain-Barré syndrome, etc. In these cases, hypertension is regarded as secondary, since the elimination of these pathological factors contributes to its resolution. However, in at least half of the cases, this condition cannot be associated with other diseases, and it is regarded as idiopathic.
Clinic
This pathology occurs in all age groups (most often in 30-40 years); in women - approximately 8 times more often than in men (1 case per 100,000 of the general population and 19 cases per 100,000 overweight young women).
The most common symptom in patients with pseudotumor cerebri is headache of varying intensity, occurring in 90% of cases (according to Johnson, Paterson and Weisberg 1974). As a rule, such a headache is generalized, most pronounced in the morning, aggravated by the Valsalva maneuver, coughing or sneezing (due to increased pressure in the intracranial veins). Visual impairment, according to various sources, occurs in 35-70% of cases. Symptoms of visual impairment are similar to those of any other type of intracranial hypertension. As a rule, they precede the headache, include bouts of short-term blurred vision, loss of visual fields and horizontal diplopia.
An objective examination may reveal unilateral or bilateral paresis of the VI pair of cranial nerves and an afferent pupillary defect. Ophthalmoscopy reveals bilateral or unilateral optic disc edema of varying severity, which over time in 10-26% of cases leads to an irreversible decrease in vision as a result of damage to the nerve fibers.
Visual field defects in varying degrees of severity occur in at least half of patients with pseudotumor cerebri, most often at the initial stage they represent a narrowing of the isopter in the lower nasal quadrant. In the future, there is a generalized narrowing of all isopters, loss of central vision or loss of visual fields along the horizontal meridian.
Neurological examination reveals signs of increased intracranial pressure in the absence of focal neurological symptoms (with the exception of unilateral or bilateral paresis of the VI pair of cranial nerves).
In many cases, pseudotumor cerebri resolves on its own, but recurs in 40% of cases. A transition to a chronic form is possible, which requires dynamic monitoring of patients. For at least two years after diagnosis, such patients should also be observed by a neurologist with repeated MRI of the brain to completely exclude occult tumors.
The consequences of even a self-resolving pseudotumor cerebri can be disastrous for visual function, ranging from moderate narrowing of visual fields to almost complete blindness. Atrophy of the optic nerves (preventable with timely treatment) develops in the absence of a clear correlation with the duration of the course, the severity of the clinical picture and the frequency of relapses.
Research methods
Magnetic resonance imaging (MRI) of the brain
According to Brodsky M.C. and Vaphiades M. (1998), intracranial hypertension leads to a range of MRI-detectable changes that suggest the presence of pseudotumor cerebri in the patient. In this case, a prerequisite is the absence of signs of a volumetric process or expansion of the ventricular system.
1) Flattening of the posterior pole of the sclera was observed in 80% of cases. The occurrence of this symptom is associated with the transfer of increased pressure of cerebrospinal fluid in the subarachnoid space of the optic nerve to the supple sclera. Atta H.R. and Byrne S.F. (1988) found a similar flattening of the sclera also in the B-scan.
2) An empty (or partially empty) Turkish saddle in such patients occurs in 70% of cases (George A.E., 1989). The frequency of occurrence of this feature ranged from 10% in the analysis of plain x-rays to 94% in the evaluation of third-generation computed tomography.
3) An increase in the contrast of the prelaminar part of the optic nerve occurs in 50% of patients. An increase in the contrast of the edematous disc is analogous to an increase in the fluorescence of the optic nerve head during fluorescein angiography: the cause in both cases is diffuse leakage of the contrast agent from the prelaminar capillaries due to severe venous stasis (Brodsky V., Glasier CV, 1995; Manfre L., Lagalla R. , Mangiameli A. 1995).
4) Expansion of the perineural subarachnoid space in patients with pseudotumor cerebri, most pronounced in the anterior sections, and to a lesser extent - at the posterior pole of the orbit, was found in 45% of cases. With the expansion of the perineural subarachnoid space, the optic nerve itself turns out to be narrowed with a slight but statistically significant increase in the average diameter of its membranes. In some cases, the so-called “string symptom” was found on axial magnetic resonance tomograms: thin as a string, the optic nerve, surrounded by an enlarged subarachnoid space, is enclosed in a normal-sized dura mater.
5) Vertical tortuosity of the orbital part of the optic nerve was noted in 40% of patients.
6) Intraocular protrusion of the prelaminar part of the optic nerve was noted in 30% of cases.
Ultrasonography
orbital part of the optic nerve
With the help of ultrasound research methods, it is possible to detect the accumulation of an excess amount of cerebrospinal fluid in the perineural subarachnoid space.
With A-scan in this case, you can detect an enlarged subarachnoid space in the form of an area of ​​​​very low reflectivity, and with B-scan - a transparent signal around the parenchyma of the optic nerve in the form of a crescent or circle - a "doughnut symptom", as well as flattening of the posterior scleral pole.
To confirm the presence of excess fluid in the perineural subarachnoid space, the 30° test developed by Ossoing et al. for A-scan. The 30° test technique is as follows: the diameter of the optic nerve is measured in the anterior and posterior sections while fixing the patient's gaze straight ahead. Then the fixation point is displaced by 30 (or more) towards the sensor and the measurements are repeated. intervals of a few minutes must be observed.
With A-scan it is also possible to measure the cross section of the optic nerve with its sheaths and evaluate their reflectivity. The width of the optic nerve with its sheaths, according to Gans and Byrne (1987), normally ranges from 2.2 to 3.3 mm (average 2.5 mm).
Transcranial dopplerography
Transcranial Dopplerography reveals an increase in systolic blood flow velocity with a decrease in diastolic velocity, which leads to an increase in the pulsation index without significant changes in the average velocity parameters in the main vessels of the brain and is an indirect sign of intracranial hypertension.
Patient Examination Scheme
with suspicion of pseudotumor cerebri
n MRI of the brain
n Examination by a neurologist
n Neurosurgical examination, lumbar puncture
n Neuro-ophthalmologist examination
n Goldman perimetry or computerized perimetry (Humphrey) test 30 - 2.
n Photographing the optic disc.
n Ultrasound examination (B-scan and A-scan with measurement of the diameter of the shells of the orbital part of the optic nerve and a 30° test).
Treatment of patients with pseudotumor cerebri
Indications for the treatment of patients with pseudotumor cerebri are:
1) persistent and intense headaches.
2) signs of optic neuropathy.
The method of treatment is the elimination of the provoking factor (if it is known), the fight against excess weight, drug therapy, and in the absence of a positive effect, various surgical interventions.
Conservative therapy
1. Restriction of salt and water.
2. Diuretics:
a) furosemide: start with a dose of 160 mg per day (adults), evaluate the effectiveness by clinical manifestations and the condition of the fundus (but not by the level of cerebrospinal fluid pressure), if there is no effect, increase the dose to 320 mg per day;
b) acetazolamide 125-250 mg every 8-12 hours (or long-acting drug Diamox Sequels® 500 mg).
3. If the treatment is ineffective, add dexamethasone at a dose of 12 mg per day.
Liu and Glazer (1994) suggest intravenous methylprednisolone 250 mg 4 times a day for 5 days, switching to oral administration with gradual withdrawal, in combination with acetazolamide and ranitidine.
The absence of a positive effect from conservative therapy within 2 months from the start of treatment is an indication for surgical intervention.
Surgery
Repeated lumbar punctures
Repeated lumbar punctures are performed until remission is obtained (in 25% of cases, remission is achieved after the first lumbar puncture), taking up to 30 ml of CSF. Punctures are made every other day until the pressure reaches the level of 200 mm H2O, then - once a week.
Bypass surgery
Currently, most neurosurgeons prefer lumboperitoneal shunting, pioneered in this pathology by Vander Ark et al. in 1972. Using this method, pressure is reduced in the entire subarachnoid space of the brain and, secondarily, in the associated perineural subarachnoid space in the absence of pronounced adhesions there.
If the presence of arachnoiditis does not allow the use of the lumbar subarachnoid space for shunting, ventriculo-peritoneal shunting is used (which can also be difficult, since often the ventricles in this pathology are narrowed or slit-like). Complications of bypass surgery include infection, blockage of the shunt, or overuse of the shunt leading to increased headaches and dizziness.
Decompression of the optic nerve sheaths
In recent years, there has been increasing evidence of the effectiveness of decompression of the optic nerve itself in order to prevent irreversible loss of visual function.
According to Tse et al. (1988), Corbett et al., Kellen and Burde et al., optic nerve decompression should be performed before visual acuity decline begins. Since a sign of an incipient lesion of the optic nerve is a concentric narrowing of the visual fields while maintaining normal visual acuity, surgical intervention is indicated when the narrowing of the visual fields progresses.
Corbett (1983) notes that in the absence of stabilization of the process (decrease in visual acuity, an increase in existing visual field defects or the appearance of new ones, an increase in an afferent pupillary defect), decompression should be performed without waiting for vision to decrease to any certain level. Expansion of the blind spot or transient blurring of vision in the absence of visual field defects is not in itself an indication for surgical intervention.
The purpose of the operation is to restore visual functions lost as a result of optic disc edema or to stabilize the process by reducing the pressure of cerebrospinal fluid in the subarachnoid space of the orbital part of the optic nerve, which leads to the regression of edema.
Decompression (fenestration of the sheaths) of the optic nerve was first proposed by De Wecker in 1872 as a surgical treatment for neuroretinitis. However, this operation was practically not used until 1969, when Hoyt and Newton, along with Davidson and Smith, again proposed it, already as a method of surgical treatment of chronic congestive optic disc. However, over the next 19 years, only about 60 cases of surgical decompression of the optic nerve sheaths were described in the literature.
This operation gained wide recognition only in 1988, after Sergott, Savino, Bosley and Ramocki, along with Brourman and Spoor, Corbett, Nerad, Tse and Anderson, published a series of successful results of surgical decompression of the optic nerve sheaths in patients with idiopathic intracranial hypertension.
Currently, surgical decompression of the optic nerve sheaths is the treatment of choice in patients with visual impairment due to chronic papilledema in conditions such as pseudotumor cerebri and dural sinus thrombosis. Both medial and lateral access with various modifications are used.
Effect of decompression of the optic nerve sheaths on cerebral liquor dynamics
Kaye et al. in 1981, intracranial pressure was monitored in a patient with pseudotumor cerebri before and after bilateral optic nerve decompression and did not find a statistically significant decrease despite a decrease in disc edema. The authors concluded that the improvement in the condition of the optic disc was not due to a decrease in intracranial pressure in general, but as a result of an isolated decrease in cerebrospinal fluid pressure within its membranes.
A small volume of cerebrospinal fluid flowing through the fistula from the perineural subarachnoid space is sufficient to decompress the sheaths of the optic nerve itself, but this amount may not be enough to decompress the entire subarachnoid space as a whole.
Management of patients with pseudotumor cerebri
Patients with pseudotumor cerebri require constant dynamic monitoring both at the stage of conservative treatment, before deciding on surgery, and in the postoperative period.
Corbett et al. The patients were discharged the next day after the operation. Then all patients were examined a week after the operation, then monthly until stabilization of visual functions. Further examinations were carried out every 3-6 months.
Early signs of the regression of optic disk edema in some cases appeared on days 1-3 and represented the appearance of a clearer outline of the temporal half of the disc. A small area of ​​the nasal half of the disc often remained edematous for a longer time.
As a criterion for the effectiveness of the decompression performed, Lee S.Y. et al. proposed to evaluate also the caliber of the retinal veins. According to their data, the venous caliber significantly decreases after surgery and continues to decrease on average for 3.2 months not only in the operated eye, but also in the other. This once again confirms that the mechanism of action of the operation is the slow filtration of fluid through a surgically formed fistula in the sheaths of the optic nerve.

Idiopathic intracranial hypertension is an increase in intracranial pressure for no apparent reason. The unified theory of idiopathic intracranial hypertension (IVH) explains the predisposition to this disease in obese women of childbearing age, the absence of ventriculomegaly, and the existence of a clinically identical syndrome when exposed to other factors, for example, against the background of the action of exogenous drugs and venous thrombosis.

It can also occur with: traumatic brain injury, mastoiditis, thrombosis, the use of oral contraceptives, an excess of vitamin A, carbon dioxide, hypoparathyroidism, Addison's disease, the use of nalidixic acid, organic insecticides, glucocorticoids, peroxylin, retinol.

Although menstrual irregularities are common in these patients, specific hormonal disorders have not yet been identified; dysmenorrhea may rather be associated with obesity. According to the Monroe-Kelly hypothesis, the intracranial content is anatomically divided into the brain parenchyma, vessels, and cerebrospinal fluid. Intracranial pressure (ICP) is normally maintained due to mutually balanced factors - expansion of the meninges and compression of the vascular volume.

The resistance factor regulates the volume of cerebrospinal fluid (CSF) by draining it through the arachnoid granulations into the cerebral veins. 50% of the CSF is located below the foramen magnum, and almost half of this amount is absorbed in the spinal sac. In the cranial cavity, resistance factors quickly reach critical values, therefore, with an increase in the volume of cerebrospinal fluid, the balancing mechanisms cease to function, and a slight increase in the total volume results in a pronounced increase in ICP.

Some authors suggest that an increase in cerebral venous pressure is the primary cause of IVH as a result of a reversal of the normal gradient between the sinuses and the subarachnoid space and an increase in resistance to CSF ​​flow through pachyon granulations.

Other researchers believe that disturbances in the microstructure of cerebral vessels cause an increase in cerebral blood flow, which reflects tissue swelling due to an increase in total water content. However, the latter or cerebral edema were never detected on IVH. It remains unknown why the cerebral ventricles do not expand, but the venous system is likely a stretching component as the pressure rises.

Manometry indicates an increase in CSF pressure in these patients, however, there is a mutual relationship between CSF pressure and pressure in the superior sagittal and transverse sinuses during IVH (i.e. removal of CSF causes a decrease in venous pressure). Patients with IVH-like syndrome exhibited both thrombosis and stenosis of the intracranial sinuses.

The role of systemic (and later - intracranial) hypertension due to abdominal obesity was also assumed, which is associated with direct compression of the inferior vena cava by adipose tissue. If this were correct, then the incidence of IVH in the world would be much higher, especially among pregnant women. In fact, the prevalence of this disease during gestation is not higher compared to age-matched controls. Currently, hypervitaminosis A is being intensively studied as a secondary cause of intracranial hypertension. The specific mechanism of action of intoxication with this vitamin on cerebrospinal fluid homeostasis is unclear - probably, it is a violation of the outflow and blocking the absorption of CSF.

There are conflicting data regarding the content of serum retinol and retinol-binding protein in IVH compared with healthy individuals. The association of IVH with orthostatic edema, depression, and anxiety suggests a possible neurotransmitter involvement. Although it is known from animal studies that serotonin and norepinephrine directly affect CSF synthesis, this has not been studied in humans.

IVH revealed a high level of vasopressin in the CSF, a hormone that regulates the water content in the brain parenchyma and increases ICP by stimulating the extravasation of fluid from the brain capillaries into the epithelium of the choroid plexus and pachyon granulation. Studies examining the level of serum leptin, a hormone associated with obesity, did not reveal any difference between patients with IVH and normal individuals.

Symptoms of Raised Intracranial Pressure - Treatment: Medications, Diet, Surgical Methods - Complications of Raised Intracranial Pressure

Idiopathic Intracranial Hypertension - Raised Intracranial Pressure

Intracranial hypertension syndrome is increased intracranial pressure due to a number of potential causes, such as impaired flow of cerebrospinal fluid (hydrocephalus), infections, obstruction of blood flow, or brain tumors.

Not so long ago, before the introduction of CT and MRI into medical practice, brain tumors were diagnosed on the basis of a neurological examination. One of the frequent combinations was a combination of chronic headache and signs of edema of the optic nerves during examination of the fundus.
In English literature, this syndrome is called false brain tumor , since these signs are present, but the tumor is not. Syndrome idiopathic intracranial hypertension is caused by an increase in brain volume due to the accumulation of fluid in its tissues. The reason for this phenomenon is unknown. An increase in volume leads to an increase in intracranial pressure.

The reason for visiting a doctor in this disease is usually chronic headache and transient visual impairment.

Symptoms of Intracranial Hypertension

Headache

Headache is universal in intracranial hypertension. The nature of the headache and its intensity are very individual. Headache is often present at night. At the same time, the presence of night pain is not necessary. The pain can be either localized or involve the entire head.

The intensity of the headache varies from mild to moderate, only occasionally it can be intense. The nature of the pain may be throbbing, bursting, or described by patients as a sensation of a hoop around the head. There is often an increased sensitivity to light.

The main quality of headache in intracranial hypertension is its constancy. Although, even this quality is not very universal.

The presence of an unexplained chronic headache is only suspicious, but not sufficient to make this diagnosis. Depression, chronic sleep deprivation for any reason, and sleep apnea cause identical headaches.

Short-term visual impairment

Bouts of short-term loss of vision, as if the television screen had momentarily turned off, "sparks in the field of vision," or short-term visual disturbances such as feeling like looking through a cloudy glass, are not uncommon.
They are often triggered by changes in head or body position (especially bending over or standing up) and last from seconds to minutes. Short-term visual impairment can occur in one or both eyes.

All these visual symptoms are a temporary phenomenon and they do not pose any danger in themselves. However, the main potential complication of intracranial hypertension is the loss of one or another part of peripheral vision.
In exceptional cases, with a strong increase in intracranial pressure, a serious decrease in visual acuity up to blindness is possible.

Long-term visual impairment is a potentially irreversible process that requires urgent intervention.

Normally, we do not notice the so-called "blind spot", which is formed by a small "blind" area of ​​the retina at the exit of the optic nerve. Swelling of the optic nerves can make the blind spot more visible, resulting in a sensation of movement at the periphery of the visual field.

Double vision

Double vision is another typical early symptom of intracranial hypertension. An increase in brain volume compresses the nerves supplying the oculomotor muscles. The result is double vision. Double vision should disappear when either eye is closed.

Most often, the VI nerve is damaged on both sides, each of which averts the eye in the direction of the temple on its side. In this case, the doubling is horizontal, i.e. the images are side by side. Since the eyes are turned to the nose, the double vision increases when looking into the distance.

Massive swelling of the optic nerves can lead to retinal edema. In this case, there is a distortion of the shape of objects (metamorphopsia). For this reason, sometimes a feeling of "double vision" is created even in one eye. Edema of the optic nerve should be visible on examination of the fundus.

Other Symptoms

Pulsatile tinnitus also often occurs with intracranial hypertension.

Causes of Idiopathic Intracranial Hypertension

idiopathic intracranial hypertension is called in cases where no explanation for the increase in intracranial pressure can be found. The very word "idiopathic" means, in fact, the absence of a known cause. Additional volume is created by an increase in the volume of the brain itself, which retains fluid in its tissues. Why is unknown.

There are some risk factors that contribute to the development of intracranial hypertension, but they are not required for the development of the disease. Here are some of them: iron deficiency, pregnancy, thyroid disease, chronic kidney failure.
Drugs such as tetracyclines, vitamin A overdose, corticosteroids, hormonal contraceptives, sulfonamides, Tamoxifen, Cyclosporine, and some others can cause an increase in intracranial pressure.

In most cases, however, it is idiopathic intracranial hypertension, in which there is no cause.

Although there is no known cause, the syndrome idiopathic intracranial hypertension occurs in young women who are significantly overweight in the vast majority of cases.

Diagnosis of Intracranial Hypertension

Ophthalmological Examination

The cardinal finding in the fundus with an increase in intracranial pressure is edema of the optic nerves on both sides. The absence of such casts the diagnosis of intracranial hypertension into doubt.
Examination of the visual fields may reveal enlargement of the blind spot and a variety of visual field defects.
Oculomotor problems, if present, are most often limited to insufficient abduction of the eye towards the temple on both sides.

Neurological Examination

Neurological examination is normal, except for the ocular abnormalities described above. The presence of additional findings on neurological examination requires the exclusion of other causes of increased intracranial pressure.

MRI of the brain

MRI examination for idiopathic intracranial hypertension is normal in most cases. The purpose of an MRI is to rule out tumors, infections, and hydrocephalus. Sometimes an MRI should be done with contrast.

There are no findings that would both rule out and confirm idiopathic intracranial hypertension. Some anatomical features, in themselves insignificant, can indirectly confirm the fact of increased intracranial pressure.
This is a syndrome of empty sella turcica, flattening of the eyeballs, fluid around the optic nerves, narrow ventricles of the brain for age, or narrowing of the venous sinuses. All of them can be present in healthy people and absent in idiopathic intracranial hypertension.

Thrombosis of the venous sinuses can cause symptoms similar to idiopathic intracranial hypertension. MR Venography or CT of the head with contrast will help with this diagnosis.

Spinal Puncture

A lumbar puncture for suspected intracranial hypertension is done for only one purpose - to measure the pressure of the cerebrospinal fluid. All other laboratory parameters in the idiopathic form should be normal. If not, then the cause of the symptoms is different.

Fluid pressure above 250 mmH2O in adults and >280 mmH2O in children is considered elevated.

The technique of spinal puncture plays a fundamental role. In order to measure pressure, it should be done while the patient is lying horizontally on his side. Wrong technique can lead to artificially high results. The pressure of the cerebrospinal fluid is constantly changing. In obese people, the pressure is usually higher even in the normal range. Spinal tap is useful for the diagnosis of intracranial hypertension. However, only on the basis of increased pressure in the absence of other typical symptoms of intracranial hypertension, this diagnosis is not made.

Treatment of Idiopathic Intracranial Hypertension

Weight loss

The fact that reducing body weight reduces swelling of the optic nerves is a well-known fact, proven by numerous studies. And although this method has an effect, the swelling of the optic nerve subsides slowly. There is a correlation between the degree of weight loss and its therapeutic effect. On average, a loss of at least 6% of body weight is required to relieve significant swelling of the optic nerves.

Weight loss is a mandatory, but not the only necessary component of treatment. Despite a reduction in optic nerve edema, diet alone is not sufficient to improve the prognosis for loss of normal vision. A combination of diet and medication is required to improve prognosis.

Medicinal Treatment

Treatment of idiopathic intracranial hypertension is aimed at reducing intracranial pressure.
Acetazolamide (Diacarb) is the most commonly used. It is a carbonic anhydrase inhibitor with mild diuretic activity. It reduces intracranial pressure by reducing the amount of cerebrospinal fluid produced.

Studies show that acetazolamide can not only reduce swelling of the optic nerves, but, over time, in combination with weight loss, reduce peripheral vision defects.

The dose of Acetazoloamide has to be used quite high. The initial dose is usually 1 gram per day, divided into two doses. If necessary, the dose can be increased up to 4 grams per day, if tolerated.

The most common side effect is numbness and tingling (often pronounced), which is most often experienced in the upper half of the body. Fatigue and intestinal disorders are less common. Long-term use can lead to kidney stones and elevated liver enzymes in blood tests.

Acetazolamide belongs to the group of sulfonamides, but its structure is very different from that of antimicrobials. Therefore, the presence of an allergy to sulfonamides does not necessarily mean an allergy to Acetazolamide.

Some doctors try to use one of the anti-epileptic drugs, Topiramate (Topamah), instead of Diacarb. The logic is that Topiramate is also a carbonic anhydrase inhibitor. In addition, prolonged use of Topiramate often leads to weight loss. Its side effects are similar to Diacarb, but Topiramate is much worse tolerated.

Corticosteroids may temporarily relieve symptoms of intracranial hypertension. However, they increase weight and by themselves can cause an increase in intracranial pressure.

Therapeutic Spinal Puncture

In emergency cases, with a sharp drop in vision, a spinal puncture is done, no longer for diagnostic, but for therapeutic purposes. As a result of drainage of cerebrospinal fluid, the symptoms of intracranial hypertension are reduced, but temporarily.

Considering that about 500 ml of cerebrospinal fluid is produced per day, intracranial pressure very quickly rises to its previous level. However, this procedure buys some time for other treatments.

Surgical Treatments for Intracranial Hypertension

Surgical treatment is indicated for severe disease and in cases of lack of effect of conservative treatment, as well as the threat of loss of vision.

Decompression ( Fenestration) Sheaths of the Optic Nerve

The main goal of the procedure is to prevent vision loss. Headache by itself is not a sufficient indication.

During this procedure, several incisions are made in the dura mater surrounding the optic nerves. Thus, the drainage of cerebrospinal fluid relieves pressure on the optic nerves. This procedure is not too simple and is performed under general anesthesia.

Fenestration of the optic nerve sheaths on only one side in some cases can solve the problem on the opposite side. Initially, a good effect may be short-lived. In about a third of cases, vision begins to deteriorate again after 3 to 5 years.

Cerebrospinal Fluid Shunt

There are several variations of shunting for idiopathic intracranial hypertension. The idea behind shunting is to permanently drain the cerebrospinal fluid.

Any of the shunts is a tube with a built-in valve that regulates the outflow depending on the pressure level. One end of the tube is placed either in the cavity of the ventricle of the brain or inside the spinal canal in the lumbar region. The other end of the tube drains cerebrospinal fluid into the pleural cavity (chest), peritoneal cavity (abdomen), or atrium (one of the chambers of the heart).

Each of the drainage options has pros and cons.
With intracranial hypertension, the ventricles of the brain are very small and difficult to get into.
For this reason, lumboperitoneal shunting (back-abdomen) for idiopathic intracranial hypertension is used more frequently.
The problem is that the lumboperitoneal (back-abdomen) shunt clogs twice as often as the ventriculoperitoneal (head-abdomen).

The initial effect of shunting is simply magnificent. Improvement is seen in 95%. However, after 3 years, the numbers fall by half.

Another problem is shunt dysfunction, which occurs in about 75% within 2 years. So, you have to make frequent revisions of the shunt.

The information on the site is provided for educational purposes only. Please do not self-medicate! The final diagnosis of your health problems remains the prerogative of medical professionals. The material of the site will only help you to get acquainted with the potential ways of diagnosing and treating neurological diseases and increase the productivity of your communication with doctors. The information on the site is updated whenever possible, taking into account recent changes in the approach to the diagnosis and treatment of neurological diseases. However, the author of the articles does not guarantee that the information will be updated immediately as it becomes available. I would be grateful if you share your thoughts: [email protected]
Content copyright 2018. . All rights reserved.
By Andre Strizhak, M.D. Bayview Neurology P.C., 2626 East 14th Street, Ste 204, Brooklyn, NY 11235, USA

The prevalence among normosthenic women is 1 per 100,000, but in obese women, the rate is higher - 20 per 100,000 people. An increase in intracranial pressure is detected, the cause is unknown, the role of a violation of the venous flask from the brain is assumed.

Symptoms and signs of idiopathic intracranial hypertension

Almost all patients complain of almost daily attacks of generalized headache with fluctuating intensity, sometimes accompanied by nausea. Sometimes transient visual disturbances, diplopia (due to impaired function of the 6th pair of cranial nerves) and pulsatile tinnitus develop. Vision loss begins in the periphery and patients may not notice it for a long time. Persistent blindness is the most serious complication of this pathology.

Often bilateral hypostasis of nipples of optic nerves comes to light; in a small proportion of patients, edema is unilateral or absent altogether. In some asymptomatic patients, swelling of the optic nipples is detected during routine ophthalmoscopy. Neurological examination may reveal abducens nerve paresis.

Diagnosis of idiopathic intracranial hypertension

• MRI with venosinusography.
• Lumbar puncture.

A presumptive diagnosis is established on the basis of the clinical picture of the disease, and then confirmed by neuroimaging (preferably MRI with venosinusography) and lumbar puncture, which reveals an increase in CSF outflow pressure and normal CSF composition. A clinical picture resembling idiopathic intracranial hypertension can be caused by certain diseases and the use of certain drugs.

Treatment of idiopathic intracranial hypertension

• Acetazolamide.
• Weight loss.
• Drugs used in the treatment of migraine, especially topiramate.

The goal of treatment is to reduce intracranial pressure and relieve headaches. The carbonic anhydrase inhibitor acetazolamide (250 mg orally four times a day) is used as a diuretic. For obese patients, weight loss interventions are recommended, which may reduce intracranial pressure. Information about the need for multiple lumbar punctures is contradictory, but in some cases it is indicated (for example, when there is a threat of visual impairment). Correction of all possible causes (drugs and / or diseases) of this condition is carried out. Drugs used in the treatment of migraine (especially topiramate, which also inhibits carbonic anhydrase) can stop headache attacks. NSAIDs are used as needed.

In the event that, despite the measures taken, vision deteriorates, shunt operations (lumboperitoneal or ventriculoperitoneal), fenestration of the optic nerve sheath or endovascular stenting of venous vessels are indicated. Bariatric surgery can help obese patients who are otherwise unable to reduce their body weight.

To evaluate the effectiveness of ongoing therapy, frequent repeated ophthalmological examinations (including quantitative determination of visual fields) are necessary; measurement of visual acuity is not sensitive enough to assess progressive loss of vision.