Neurology and Neurosurgery: Pseudotumor Cerebri

It was a challenge discovering the heart of the medical condition. Thus, for many years the syndrome was called by many names, such as 1) hypertensive hydrocephalus; 2) otitic hydrocephalus; 3) hypertensive meningeal hydrops, and 4) pseudoabscess (Sullivan 1991). In the beginning, physicians were puzzled by the etiological factors associated with the disease (Noggle and Dean 2012). They wanted to know the reason for the increased intracranial pressure minus the presence of a mass lesion or ventricular obstruction (O’Donnell 2005). The disease usually affects adults. However, in some cases, children are also victims of the syndrome.

General Description and Symptom

Normal and happy life is characterized by the absence of pain (Mayo Clinic 2014). Those who suffer from the affliction called pseudotumor cerebri experience discomfort, because the pressure inside the skull increases without warning (Noggle and Dean 2012). This particular disease mimics the symptoms of a brain tumor, hence the name pseudotumor cerebri (O’Donnell 2005). Due to the similarity, a test designed to detect brain tumors is recommended. However, no tumor is detected after a series of tests are completed (Quattromani and Handal 2008). In some cases, the inability to explain the cause of the discomfort leads doctors to pronounce the illness as idiopathic intracranial hypertension (Mayo Clinic 2014).

Patients who suffer from this illness must have immediate access to appropriate care because increased intracranial pressure causes the swelling of the optic nerve (Sullivan 1991). One of the problematic consequences of pseudotumor cerebri is vision loss (Merikangas 1991).

Pseudotumor cerebri has been associated with obesity, menstrual irregularities, adrenal insufficiency, hyperadrenalism, hypoparathyroidism, venous sinus thrombosis, hypervitaminosis A, and Guillain-Barre syndrome (Sullivan 1991). It is a challenge to arrive at a correct diagnosis. Those who suffer from this disease are expected to suffer unnecessarily because they oftentimes go through a series of tests that usually includes steps to eliminate the following: hydrocephalus, brain tumor, focal mass lesion, and venous sinus thrombosis (Leestma 2009).

If a person has a predisposition towards this disease, he must see a specialist if suffering from a combination of the following symptoms: 1) moderate or severe headaches that usually originates from behind the eyes; 2) moderate or severe headaches that worsen with eye movement; 3) ringing in the ears; 4) nausea; 5) vomiting; 6) dizziness; 7) blurred or dimmed vision; 8) brief episodes of blindness that lasts only a few seconds; 8) difficulty seeing to the side; 9) double vision or diplopia; 10) seeing light flashes or photopsia; 11) neck or shoulder pain (Mayo Clinic 2014). The disease is commonly associated with hypervitaminosis A (Rapoport 2008). In fact, pseudotumor cerebri occurs in 30 to 50 percent of patients with hypervitaminosis A (O’Donnell 2005).

Severe headache and visual disturbance are good indicators because these are the typical symptoms in 80% to 90% of patients afflicted with the disease (Sullivan 1991). Nevertheless, it must be made clear that there is nothing specific about the headache (Siddiqi 2006). The headache can be described as unilateral, mild, moderate, and severe (Siddiqi 2006). Once the symptoms are identified, medical specialists often perform a spinal tap to assure the accuracy of the diagnosis. The spinal tap will reveal an abnormally high pressure caused by the cerebrospinal fluid.

It must be pointed out that women are affected more by this medical problem than men (Quattromani and Handal 2008). There are several reasons for this assertion. The risk factor is linked to pregnancy (Johnston and Owler 2007). It is also linked to the menstrual cycle of women. Furthermore, women who suffer from polycystic ovary syndrome have a predisposition towards this disease (Johnston and Owler 2007). In addition, the use of contraceptives may have contributed to the presence of excessive amounts of exogenous estrogens, which are triggering factors for this disease (Lefkowitz 2009).

In order to have a better prognosis, health experts suggested the use of magnetic resonance imaging or MRI (Drummond 2006). The MRI and other imaging techniques will help determine normal or small-sized cerebral ventricles, as well as elevated levels of cerebrospinal fluid (O’Donnell 2005).

Treatment Strategies

One of the effective treatment strategies is through the use of medication (Mayo Clinic 2014). However, in severe cases, surgery is necessary (Siddiqi 2006). Powerful narcotics and invasive surgery are treatments strategies of last resort. Prevention is the first line of defense when it comes to medical problems. However, it is important to find out the root cause of the medical condition in order to develop an effective prevention strategy. In the case of this particular disease, the exact cause is unknown (Mayo Clinic 2014). Experts in the field of neurology suggested that the root cause of the problem is the excess amount of cerebrospinal fluid within the skull of the patient (Sartor and Haehnel 2008). Although the exact etiology is not clear, researchers discovered that the problem is exacerbated by the decreased absorption of cerebrospinal fluid with the subarachnoid space (Quattromani and Handal 2008). Others suggested that the root cause is the disruption of the outflow on the venous sinuses with cerebral venous pressure made evident in such malfunction (Brownell and Priff 2008). The said outflow obstruction leads to the increased resistance of cerebrospinal fluid absorption (Brownell and Priff 2008). Thus, the accumulation of fluid increases the pressure inside particular areas within the cranial cavity.

It is interesting to note that obesity is a major risk factor for this disease (Quattromani and Handal 2008). In every 100,000 obese people, 1 or 2 will suffer from pseudotumor cerebri (Noggle and Dean 2012). It is estimated that 4 to 21 women in a group of 100,000 obese women will develop the said condition (Mayo Clinic 2014). Health experts also remarked that obese women under the age of 44 are more likely to suffer from the effects of pseudotumor cerebri (Mayo Clinic 2014). Recent findings indicated that even young obese women could succumb to the said disease (Green and Rothrock 2005). Obese people are more at risk because of hypertension. Since obesity is a serious risk factor, weight management is considered an effective prevention strategy. Weight loss will never serve as a treatment strategy for acute symptoms (Brownell and Priff 2008). However, weight loss is seen as a long-term treatment component (Brownell and Priff 2008). Patients who suffer from pseudotumor cerebri must seriously consider the impact of dietary management and exercise programs.

Aside from managing weight loss, another strategy to lessen the incidence of the syndrome is to veer away from the use of growth hormones, tetracycline, and the consumption of excessive amounts of Vitamin A (Siddiqi 2006). Women have a higher risk factor because many of them require hormone treatment.

In most cases, the pain waxes and wanes (Quattromani and Handal 2008). In others, the symptoms are resolved spontaneously without external intervention (Quattromani and Handal 2008). However, there are patients that require serial lumbar puncture drainage or shunting of excess cerebrospinal fluid (Drummond 2006). The purpose of the drainage is to relieve intracranial pressure (Drummond 2006).

When it comes to treatment strategies, the initial reaction is to relieve the persistent headache. At the same time, steps are made to reduce the probability of vision loss. Health experts are in agreement that in many cases, the medical intervention must be employed (Sismanis and Salley 2007). A common procedure is the application of corticosteroids, oral hyperosmotic agents, and carbonic anhydrase inhibitors (Sismanis and Salley 2007). The purpose of the said drugs is to reduce the pressure caused by the cerebrospinal fluid. In severe cases, doctors recommend drainage or shunting in order to provide immediate and continuous reduction of cerebrospinal fluid pressure (Johnston and Owler 2007).

It is easy to understand the connection between obesity and pseudotumor cerebri if one considers it from the context of hypertension. Obese people are prone to high blood pressure, and hypertension is a contributing factor in the intracranial pressure that characterizes the said disease. Thus, the best way to significantly reduce the incidence of pseudotumor cerebri is through weight loss.

It is important to point out that for clinically obese patients, weight loss programs have high failure rates. In many cases, weight management is impossible to implement. Obesity is a major health issue that has no cure. If a person has a BMI higher than 35, obesity is no longer about excess weight because it is a health problem that can lead to serious complications. Aside from the risk of developing pseudotumor cerebri, those who are diagnosed with severe obesity are susceptible to multiple comorbidities, and these patients will experience a significant reduction in life expectancies (Leestma 2009).

A Body Mass Index greater than 60 kg/m2 usually means that the patient suffers from multiple high-risk comorbidities (Leestma 2009). It is imperative to apply medical intervention, and in some cases, the only way to eliminate obesity is through the use of a weight loss surgery sleeve. This procedure is known as bariatric surgery (Schauer 2008). In this medical procedure, the patient benefits from a restrictive surgery, wherein the anatomy of the gastrointestinal tract is modified to reduce the amount of food that can be ingested (Schauer 2008).

The surgery sleeve is also known as sleeve gastrectomy (Schauer 2008). This type of bariatric surgical procedure significantly reduces the number of calories ingested by the human body (Schauer 2008). This is possible because the surgeon has created a smaller gastric reservoir (Schauer 2008). After performing the needed surgical procedure that restricts the amount of food intake, the surgeon will also modify the stomach in order to have a narrow outlet (Schauer 2008). With a smaller outlet, the emptying process is delayed (Schauer 2008). Thus, the patient will feel that he has consumed a good quantity of food (Schauer 2008).

The restrictive operation reduces the volume of the gastrointestinal tract (Schauer 2008). As a result, the stomach loses as much as 90% of its original size (Schauer 2008). The expert surgeon leaves the smaller curvature of the stomach intact while the larger portion is removed, creating a sleeve-like structure (Schauer 2008). Health experts asserted that the smaller stomach is called non-compliant because it has less capability of expanding while eating (Schauer 2008).

Sleeve gastrectomy or SG was designed to effectively improve the weight loss program employed by obese people (Schauer 2008). There are several types of bariatric surgery that are available for obese patients. There are SG procedures performed in conjunction with sophisticated laparoscopy techniques (Schauer 2008). These techniques enable the surgeon to use minimally invasive strategies (Schauer 2008). This procedure will reduce the trauma caused by surgical procedures (Schauer 2008). It is important to note that SG must be performed in specialized centers (Schauer 2008). It is imperative to know that obese patients must only collaborate with qualified surgeons (Schauer 2008).

Interested patients must look into the work of The International Federation for the Surgery of Obesity and Metabolic Disorders or IFSO (Schauer 2008). This is a good starting point when it comes to the need to inquire about the services of a qualified doctor. In addition, the American Society for Metabolic and Bariatric Surgery can also help in providing information about qualified bariatric surgeons in the country (Schauer 2008).

If intracranial pressure is linked to hypertension, then it is imperative to drastically reduce the patient’s weight. Obese men and women suffer from hypertension. It is good to know that those who went through bariatric surgery reported a significant reduction in their weight (Schauer 2008). They reported as high as 40 percent reduction after weight-loss surgery was performed on them (Schauer 2008). Health experts highlighted the reason why weight reduction was a realistic goal (Schauer 2008). They remarked that the smaller stomach prevented the ingestion of excessive amounts of food (Schauer 2008). They also pointed out that the surgery reduced the presence of a hormone called ghrelin (Schauer 2008). Ghrelin is a hormone that induces hunger pangs (Schauer 2008). Thus, the reduced concentration of the said hormone lessens hunger pangs (Schauer 2008). It must be pointed out that even obese patients with a BMI of 50 reported significant changes in their weight loss program (Schauer 2008).

Conclusion

Pseudotumor cerebri is an interesting medical condition because it is hard to pinpoint the root cause of the problem. The symptoms are similar to other diseases, such as brain tumors and hydrocephalus. Patients were known to suffer needlessly because they had to go through a series of tests in order to eliminate suspected causes of symptoms that mimicked those of other diseases. Nevertheless, there are certain people that are at a higher risk of developing the said disease. Obese women are at a higher risk of developing pseudotumor cerebri. There are many cases that involve women, and this is due to hormone-related issues. Thus, pregnant women are known to complain about symptoms related to pseudotumor cerebri. Prevention is the best line of defense. Thus, it is imperative to develop a weight loss program for obese women, especially those who are suffering from the effects of pseudotumor cerebri. It has been made clear that there are obese people who can no longer go through a lengthy weight loss program. They need to lose weight fast. Thus, it is important to consider the impact of bariatric surgery as one of the indirect methods of dealing with the said medical issue. It is prudent to use available medical procedures to relieve the pressure caused by cerebrospinal fluid. It is also prudent to use surgery in order to relieve the pressure in the cranial cavity. However, a long-term strategy is needed. At the same time, a medical procedure is needed to drastically reduce the weight of obese people. Thus, bariatric surgery is the key factor in reducing symptoms and other comorbidities. Patients must understand that only qualified surgeons are allowed to perform a risky surgical procedure like bariatric surgery. For many obese women suffering from the impact of pseudotumor cerebri, there may not be another solution other than to drastically reduce weight using a proven medical procedure.

The yellow arrows are pointing to the swelling of glands that are causing the protrusion of the optic nerve heads
Fig.1: The yellow arrows are pointing to the swelling of glands that are causing the protrusion of the optic nerve heads (Pathak 2011).
Excess amounts of cerebrospinal fluid causes the swelling of the optic disc
Fig. 2. Excess amounts of cerebrospinal fluid causes the swelling of the optic disc (Pathak 2011).

References Cited

Drummond S (2006) Neurogenic Communication Disorders. Charles Thomas Publishers, Illinois.

Johnston I, and B Owler (2007) The Pseudotumor Cerebri Syndrome. Cambridge University Press, Cambridge.

Leestma J (2009) Forensic Neuropathology. CRC Press, Florida.

Lefkowitz D (2009) Approach to the patient with chronic and recurrent headache. Chapter 21, pp. 49-64 in Practical Neurology, J Biller, Ed. Wolters Kluwer, New York.

Merikangas J (1991) Headache syndromes. Chapter 11, pp. 393-423 in Medical Psychiatric Practice, A Stoudemire and B Fogel, Eds. American Psychiatric Press, Washington, D.C.

O’Donnell, J (2005) Drug Injury: Liability, Analysis, and Prevention. Lawyers and Judges Publishing Company, Arizona.

Quattromani F, and G Handal (2008) Pediatric Imaging. Thieme Medical Publishers, New York.

Rapoport A (2008) Classification and diagnosis of secondary headaches due to traumatic and vascular causes. Chapter 7, pp. 115-206 in Comprehensive Review of Headaches and Medicine, M Levin, Ed. Oxford University Press, New York.

Sartor K and S Haehnel (2008) Brain Imaging. Thieme Medical Publishers, New York.

Schauer P (2008) Minimally Invasive Bariatric Surgery. Springer, New York.

Siddiqi J (2006) Neurosurgical Intensive Care. Thieme Medical Publishers, New York.

Sismanis A, and D Salley (2007) Pseudotumor cerebri and jugular foramen syndrome. Chapter 24, pp. 343-358 in Head and Neck Manifestation of System Disease, J Harris and M Weisman, Eds. CRC Press, New York.

Sullivan, H (1991) Diagnosis and management of pseudotumor cerebri. Journal of the National Medical Association 83: 916-918.

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