Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a condition characterized by increased intracranial pressure without evidence of a tumor or other underlying cause. While the exact cause of IIH remains poorly understood, it predominantly affects young, obese women of child-bearing age. As rates of obesity rise, the incidence of IIH is expected to increase as well. This article aims to provide a practical guide to understanding and managing IIH, drawing upon available research and expert recommendations.
Understanding IIH
IIH is a diagnosis of exclusion, meaning it is diagnosed after ruling out other potential causes of increased intracranial pressure. The nervous system generally contains about 140 mL of cerebrospinal fluid (CSF) at a time. The choroid plexus produces the cerebrospinal fluid (CSF) which turns over 3-4 times per day. Arachnoid granulations clear the CSF, though the mechanisms of control are yet to be understood. The pathogenesis of IIH is ill-defined and may be multifactorial.
Several factors are thought to contribute to the development of IIH:
- Obesity: A strong association exists between obesity and IIH. Current studies focus on a possible obesity-related inflammatory component.
- Increased Venous Sinus Pressure: Venous sinus stenoses are thought to be secondary to raised ICP, however, it is being increasingly recognized to nevertheless contribute to IIH.
Symptoms of IIH
Symptoms of intracranial hypertension increase when you're exerting yourself. It can be frustrating to receive a diagnosis for a condition with an unknown cause. Even though it may seem like there aren’t any answers for idiopathic intracranial hypertension (IIH), you can still ask questions to learn more about what’s going on inside of your body. Anyone can develop IIH. Common symptoms include:
- Headaches
- Vision changes
- Increased headaches and shortness of breath after performing moderate to high intensity workouts
Management Strategies for IIH
Although formal guidelines for treatment are limited, standard management of IIH involves diuretic medications and weight loss. Considering that IIH is a disease that is often related, at least in part, to obesity lends itself to management through lifestyle modifications. In the meantime, get regular eye exams and let your provider know if you notice any changes to your vision. Taking medicines, such as water pills (diuretics).
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Lifestyle Modifications
Targeted lifestyle modifications include: weight loss through nutrition and exercise and management of symptoms through relaxation methods. By initiating lifestyle changes, patients are better equipped to make impactful improvements in the reduction and symptomatic management of their IIH, thereby preventing negative sequelae. Review of the literature reveals that focusing on weight loss management by initiating dietary modifications and improving exercise capacities may be tools that patients can leverage. Additional smaller lifestyle modifications may be employed by patients to help with symptom management.
Dietary Changes
Consider a low-sodium, low-energy-dense diet and focus on portion control. Low-energy-dense diets consist of fruits, vegetables, whole grains, lean meats, and low-fat dairy products. It’s a good idea to eat a wide variety of healthy foods that are low in sodium and saturated fats to support your body if you have intracranial hypertension. This might mean giving up salty snacks, such as potato chips, and cutting back on red meat in favor of a salad with cooked chicken breast. Ask your doctor about the dietary choices that might be best for you and your situation. You want to ensure that you are receiving enough proper nutrients and proteins to stay healthy.
Foods to Limit or Avoid:
- Salty and Fatty Foods: It’s wise to avoid food that has too much salt, which causes your body to retain water, and excess fats that add calories. All can contribute to weight gain.
- Foods High in Vitamin A: Foods such as beef liver that are high in vitamin A have been proven to increase the risk of intracranial hypertension or cause complications. Not only can high doses add to that risk, especially when taken as vitamin A supplements, researchers have found that low doses can contribute as well. Ask your doctor if you need to limit foods that are rich in vitamin A and which foods might be best to avoid in your situation.
- Foods High in Tyramine: Because tyramine can cause blood vessels to dilate, people with intracranial hypertension should limit foods that contain it. Before removing foods with high amounts of tyramine from your diet, talk to your doctor.
Exercise
Increased physical activity through various mediums (resistance training, light to moderate aerobic activity) can provide promising results for weight loss. However, no studies have evaluated solely physical activity in IIH, thus interventions should be implemented along with changes to diet. Therefore patients who experience symptoms such as increased headaches and shortness of breath after performing moderate to high intensity workouts may exercise caution, and replace their workouts with low-impact options.
Most people with intracranial hypertension can carry on with their normal activities, including sports activities like running, jumping, and swimming.
Activities to potentially avoid (especially with a lumbar peritoneal shunt):
- Gymnastics
- Judo and some other martial arts
- Horseback riding
- Golf
- Roller coasters
Not all shunts or conditions pose the same risks, however. Be sure to check with your doctor about activities that might be okay for your situation.
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Medical Interventions
Medications
- Topiramate:
- Glucagon Like Peptide-1 (GLP-1) agonists: Glucagon Like Peptide-1 (GLP-1) agonists are medications that were initially used to treat diabetes, but are now being used as weight loss medications. Rodent studies have shown that GLP-1 agonists can decrease Na+ K+ ATPase activity, which leads to reduced CSF secretion at the choroid plexus. GLP-1 agonists may be an effective therapeutic option for IIH by not only promoting weight loss in patients but also decreasing the increased intracranial pressures, which remains of ongoing investigation. Patients should be warned that cessation of GLP-1 therapy can lead to weight regain.
Surgical Options
- Bariatric Surgery: In 66 women who underwent bariatric surgery, weight loss was significantly associated with reduction in ICP - the "greater the weight loss, the greater the reduction in ICP". In particular, roux-en-Y gastric bypass was the most successful. In this study, the mean weight loss needed to reduce the ICP into normal range (<25 cm) was 24% of body weight, which was only able to be achieved by patients in the bariatric surgery arm. Bariatric surgery should be considered in patients with BMI>35.
Important Considerations
- IIH and Weight Loss: Weight loss will also reduce burden of obesity-related comorbidities. In one case report, a 38 year-old woman with IIH and a starting BMI of 34.9 was able to reduce her BMI to 24.6 following extreme diet changes. Weight Management Interventions for Adults With Idiopathic Intracranial Hypertension: A Systematic Review and Practice Recommendations. Losing weight is very hard, but don't give up.
- Other medications: The medication lithium has been prescribed to treat bipolar disorder and other health issues that may require a mood stabilizer. It does have numerous side effects, however.
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