Lifestyle Modifications for Idiopathic Intracranial Hypertension

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 by Mary Labowsky, MD on July 31, 2023.


Summary

Idiopathic intracranial hypertension (IIH), also termed pseudotumor cerebri, is a health problem that is known to affect about 100,000 Americans. [1] In overweight women of child-bearing age it has a yearly incidence of 22.5 out of 100,000.[1] As rates of obesity rise, the incidence of IIH is expected to increase, thereby making this a very important medical condition to understand, diagnose, treat and manage. Although the pathophysiology of IIH is poorly understood, and formal guidelines for treatment are limited, there is evidence of significant success of symptom reversal with weight loss. As the need for more comprehensive and patient-centered medicine rises, lifestyle modification strategies may be used to further manage patients with IIH. In this article, we will discuss targeted lifestyle modification strategies, based on literature findings, and highlight the success and promise these methods bring.

Disease

IIH is a disorder predominantly seen in young, obese females of child-bearing age who present with increased intracranial pressure.[2] IIH is a diagnosis of exclusion. While the true cause of IIH is not fully understood, it has been linked to several risk factors. Clinical presentation is variable, but common symptoms include headache, vision loss, pulsatile tinnitus and back and neck pain.[2] On ocular examination, patients will present with papilledema, which is the primary finding in IIH.[2] Potential sequelae of poorly managed disease is progressive optic atrophy and blindness.[2]

Risk Factors[3]

  • Female Sex
  • Obesity or weight gain
  • Systemic Illnesses:
    • Obstructive Sleep Apnea (OSA)
    • Endocrine Disorders (Hypoparathyroidism, Addison’s Disease)
    • Anemia
    • Systemic Lupus Erythematosus
    • Coagulation Disorders
    • Uremia
  • Medications (including, but not limited to):
    • Tetracyclines
    • Vitamin A
    • Lithium
    • Steroid Withdrawal
    • Nalidixic acid

Pathophysiology

The pathogenesis of IIH is ill defined and the cause seems to be multifactorial. Theories and suggested mechanisms are constantly evolving as research progresses.

  • Cerebrospinal fluid (CSF) Hypersecretion: Original studies believed that CSF hypersecretion played a role in IIH. However, this was not supported by further studies.[2]
  • CSF Outflow Obstruction: Pressure gradient differences between the venous sinuses and subarachnoid space can result in increased CSF pressure in order to maintain CSF absorption rates.[2]
  • Venous Sinus Pressure: Although venous sinus stenosis can result in increased venous sinus pressure, in patients with IIH, the stenosis is more likely a consequence of the IIH, rather than an initial contributing mechanism.[2]
  • Cerebral Edema: Cerebral edema was originally thought to be another contributing factor, but further studies showed no evidence between cerebral edema and IIH.[2]
  • Obesity: A strong association exists between obesity and IIH. Initially, theories suggested that increased abdominal mass resulting in increased intrathoracic pressure led to increased venous pressures. Current studies focus on a possible obesity-related inflammatory component causing the disorder.[2]
  • Hormones: Current studies are seeking to understand the role of hormones in pathogenesis of IIH as the major demographic of patients affected are women of child-bearing age.[2]

Lifestyle Modification

As risk factors, symptoms and presentation of IIH may vary, individualized patient-centered care may be employed. Currently, formal guidelines for therapeutic approach are minimal. The main recommendation involves weight loss as the primary long-term method of treatment for IIH. Considering this, it is imperative to further identify lifestyle modifications that can be employed to reduce symptoms, recurrence and unfavorable outcomes of IIH. Targeted lifestyle modifications include: weight loss through nutrition and exercise and management of symptoms through relaxation methods.

Nutrition

Weight loss is strongly supported for IIH management. Previous studies focusing on weight loss provide promising results:

  • A retrospective study showed that a 6% weight loss in patients with IIH was associated with a cessation of marked papilledema, a clinical sign of increased intracranial pressure.[4]
  • Women with IIH in another prospective cohort study were placed on a low energy diet and intracranial pressures were observed. It was seen that women who followed the low energy diet for 3 months had a statistically significant reduction in intracranial pressure compared to the women who did not follow the low energy diet.[5]
  • A systematic review was conducted to assess the effectiveness of bariatric surgeries for treating IIH. It was seen that 60 out of 65 patients, across seventeen different publications, had symptomatic improvement after surgery, further indicating that decreasing weight can help decrease intracranial pressure.[6]
  • 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. Increasingly, research is revealing that GLP-1 agonists can prove to be effective therapeutic options for IIH by promoting weight loss in patients, and ultimately decreasing the increased intracranial pressures. Additionally, rodent studies have shown that GLP-1 agonists can decrease Na+ K+ ATPase activity, which leads to reduced CSF secretion at the choroid plexus.[7]


To lose weight effectively, and safely, patients may be counseled on the following:

Recommendations can be made to patients to meet with a nutritionist to come up with a feasible dietary plan that will allow for sustainable weight loss. These often entail 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. This allows people to feel increased satiety with lower calorie intake, leading to effective weight loss.

Additionally, limited data exists regarding dietary modification in the treatment of IIH, but restriction of vitamin A intake and tyramine ingestion may prove beneficial. Patients can be counseled on avoiding foods rich in vitamin A such as tomatoes, carrots, sweet potatoes, leafy greens, fish and eggs. Awareness may be made to patients of possible excess intake of vitamin A through nutritional supplements. Additionally, items like beer, wine, pickled foods, aged cheeses and meats are high in tyramine and may be avoided.[8]

Exercise

Increased physical activity through various mediums (resistance training, light to moderate aerobic activity) can provide promising results for weight loss.

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. She further decreased her BMI to 21.8 after a combined regimen of a balanced diet and moderate physical exercise (3 hours/week).[9]

However, increased exertion can increase intracranial pressure and lead to worsening of symptoms. 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.

Additional lifestyle modifications for symptom management

For symptom management, specifically headache management, minor lifestyle changes can still have significant impact. Changes can include limiting caffeine intake, ensuring adequate hydration and sleep hygiene, and focusing on stress management techniques, such as mindfulness, yoga, and cognitive behavioral therapy.[10]

Conclusion

As rates of obesity rise, the incidence of IIH is expected to increase as well. With the growing patient population, modification in treatment options and guidelines may be considered. Considering that IIH is a disease that is usually secondary to obesity lends itself to management through lifestyle modifications. 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. As a physician, it is imperative that we serve our patients with comprehensive, individualized care to treat and manage IIH. Providing them with these lifestyle modifications is an additional tool that may allow for this individualized care.


References

  1. 1.0 1.1 Friedman DI, McDermott MP, Kieburtz K, et al. The idiopathic intracranial hypertension treatment trial: design considerations and methods. J Neuroophthalmol. 2014;34(2):107-117. doi:10.1097/WNO.0000000000000114
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 2.9 Markey KA, Mollan SP, Jensen RH, Sinclair AJ. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol. 2016;15(1):78-91. doi:10.1016/S1474-4422(15)00298-7
  3. Chen J, Wall M. Epidemiology and risk factors for idiopathic intracranial hypertension. Int Ophthalmol Clin. 2014;54(1):1-11. doi:10.1097/IIO.0b013e3182aabf11
  4. Johnson LN, Krohel GB, Madsen RW, March GA Jr. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology. 1998;105(12):2313-2317. doi:10.1016/S0161-6420(98)91234-9
  5. Sinclair AJ, Burdon MA, Nightingale PG, et al. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: prospective cohort study. BMJ. 2010;341:c2701. Published 2010 Jul 7. doi:10.1136/bmj.c2701
  6. Handley JD, Baruah BP, Williams DM, Horner M, Barry J, Stephens JW. Bariatric surgery as a treatment for idiopathic intracranial hypertension: a systematic review. Surg Obes Relat Dis. 2015;11(6):1396-1403. doi:10.1016/j.soard.2015.08.497
  7. Mollan SP, Tahrani AA, Sinclair AJ. The Potentially Modifiable Risk Factor in Idiopathic Intracranial Hypertension: Body Weight. Neurol Clin Pract. 2021;11(4):e504-e507. doi:10.1212/CPJ.0000000000001063
  8. Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuroophthalmol. 2004;24(2):138-145. doi:10.1097/00041327-200406000-00009
  9. Raggi A, Grazzi L, Bianchi Marzoli S, et al. The importance of specific rehabilitation for an obese patient with idiopathic intracranial hypertension: a case report. Int J Rehabil Res. 2018;41(2):183-185. doi:10.1097/MRR.0000000000000277
  10. Mollan SP, Davies B, Silver NC, et al. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018;89(10):1088-1100. doi:10.1136/jnnp-2017-317440
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