Lifestyle Modifications for Idiopathic Intracranial Hypertension

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Article initiated by:
Andrew Go Lee, MD, Noor Laylani, MD, Pamela Davila-Siliezar, MD, Sujata Dalal, Swati Iyer
All contributors:
Nagham Al-Zubidi, MD, Noor Laylani, MD, Mary Labowsky, MD
Assigned editor:
Mary Labowsky, MD
Review:
Assigned status Up to Date
 by Mary Labowsky, MD on November 6, 2024.


Summary

Idiopathic intracranial hypertension (IIH), also termed "pseudotumor cerebri", has an annual incidence in the United States of 1.15 (per 100,000); in women, it has a yearly incidence of 1.97 out of 100,000 with an observed annual rise from 1997 to 2016.[1] As rates of obesity rise, the incidence of IIH is expected to increase.

Although the pathophysiology of IIH is poorly understood and formal guidelines for treatment are limited, standard management of IIH involves diuretic medications and weight loss.[2] In this article, we will discuss targeted lifestyle modification strategies for IIH and implications for IIH.

Disease

IIH is predominantly seen in young, obese females of child-bearing age who present with increased intracranial pressure.[3] IIH is a diagnosis of exclusion. Clinical presentation is variable, but common symptoms include headache, vision loss, pulsatile tinnitus and back and neck pain.[3] On ocular examination, patients will present with papilledema, which is the primary finding in IIH.[3] Potential sequelae of aggressive disease is progressive optic atrophy and blindness.[3]

Risk Factors[4]

  • 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 choroid plexus produces the cerebrospinal fluid (CSF) which turns over 3-4 times per day; the nervous system generally contains about 140 mL of CSF at a time. Arachnoid granulations clear the CSF, though the mechanisms of control are yet to be understood. Of increasing clinical and research interest is the outflow of CSF which occurs through the cribriform, basal, and meningeal lymphatics.[5] The pressure gradient between the venous sinuses and the subarachnoid space determines the rate of absorption of CSF.[3]

The pathogenesis of IIH is ill- defined and may be multifactorial. Below are some theories and suggested mechanisms:

  • Increased Cerebrospinal fluid (CSF) Production: Original studies believed that CSF hypersecretion played a role in IIH.[6] However, this was not supported by further studies.[3][7] Hydrocephalus or ventriculomegaly as seen in cases of choroid plexus papilloma may be expected, and these are not found in IIH.[3]
  • Decreased CSF Drainage across arachnoid granulations or lymphatics: Evidence exists that there may be delay clearance of CSF in patients with IIH.[7][3]
  • Increased Venous Sinus Pressure: Venous sinus stenoses are thought to be secondary to raised ICP.[3] However, it is being increasingly recognized to nevertheless contribute to IIH. In fact, venous sinus stenting has been found to lower the intracranial pressure and can be used as a option for treatment of medically-resistant IIH.[8]

Proposed contributors:

  • 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 (i.e. release of "adipokines" by adipose tissue, or other inflammatory/thrombophilic factors) may impact CSF dynamics causing the disorder.[3]
  • 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.[3]

Lifestyle Modification

No formal guidelines exist for therapeutic approach, though weight loss is generally recommended as one primary long-term method of treatment for IIH. Targeted lifestyle modifications include: weight loss through nutrition and exercise and management of symptoms through relaxation methods. However, a combination of diet, exercise, and behavior modification may only support a modest weight loss of 5-10%, which may or may not be sufficient to put the disease in remission.[9]

Nutrition

Weight loss is strongly supported for IIH management.[10] Patients with a BMI>40 are more likely to have severe papilledema, with risk of severe vision loss proportionate to increase in BMI.[11] Although prior studies have suggested a weight loss of 6% can lead to remission of papilledema,[12] more recent data suggests that 15-25% may be needed.[9][13]

  • Referral to a nutritionist may be advisable
  • Consider a calorie deficit of 500-1000 kcal/day to be effective in the short-term for modest weight loss[9]
  • 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. This allows people to feel increased satiety with lower calorie intake, leading to effective weight loss.
  • Restriction of vitamin A intake (tomatoes, carrots, sweet potatoes, leafy greens, fish and eggs) and tyramine (beer, wine, pickled foods, aged cheeses and meats) may prove beneficial.[14]
  • Advise patients that the goal of weight loss is to maintain the lower body mass index long-term

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.

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).[15]

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.

Other Interventions

Topamax. Topiramate is often the second-line diuretic in treatment of IIH, though it can often aid with weight loss via varying mechanisms.[16]

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.[13] 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. In patients with BMI>35, bariatric surgery should be considered as it has the best evidence for suitable weight loss.[9]

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.[17] 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.[18] Patients should be warned that cessation of GLP-1 therapy can lead to weight regain.

Additional lifestyle modifications for symptom management

For headache management, minor lifestyle changes can still have significant impact:[19]

  • Limiting caffeine intake
  • Adequate hydration
  • Sleep hygiene
  • Stress management techniques: mindfulness, yoga, and cognitive behavioral therapy
  • Screening for Obstructive sleep apnea and consideration of sleep studies

Conclusion

As rates of obesity rise, the incidence of IIH is expected to increase as well. Considering that IIH is a disease that is often related, at least in part, 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. Bariatric surgery should be considered in patients with BMI>35. Weight loss will also reduce burden of obesity-related comorbidities.

References

  1. ↑ Ghaffari-Rafi A, Mehdizadeh R, Ko AWK, Ghaffari-Rafi S, Leon-Rojas J. Idiopathic Intracranial Hypertension in the United States: Demographic and Socioeconomic Disparities. Front Neurol. 2020 Sep 8;11:869. doi: 10.3389/fneur.2020.00869. PMID: 33013623; PMCID: PMC7506031.
  2. ↑ Subramaniam S, Fletcher WA. Obesity and Weight Loss in Idiopathic Intracranial Hypertension: A Narrative Review. J Neuroophthalmol. 2017 Jun;37(2):197-205. doi: 10.1097/WNO.0000000000000448. PMID: 27636748.
  3. ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 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
  4. ↑ 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
  5. ↑ Ahn, J. H. , Cho, H. , Kim, J. H. , Kim, S. H. , Ham, J. S. , Park, I. , … Koh, G. Y. (2019). Meningeal lymphatic vessels at the skull base drain cerebrospinal fluid. Nature, 572, 62–66. 10.1038/s41586-019-1419-5
  6. ↑ Donaldson JO. Cerebrospinal fluid hypersecretion in pseudotumor cerebri. Trans Am Neurol Assoc 1979; 104: 196–98.
  7. ↑ 7.0 7.1 Malm J, Kristensen B, Markgren P, Ekstedt J. CSF hydrodynamics in idiopathic intracranial hypertension: a long-term study. Neurology 1992; 42: 851–58.
  8. ↑ Dinkin MJ, Patsalides A. Idiopathic Intracranial Venous Hypertension: Toward a Better Understanding of Venous Stenosis and the Role of Stenting in Idiopathic Intracranial Hypertension. J Neuroophthalmol. 2023 Dec 1;43(4):451-463. doi: 10.1097/WNO.0000000000001898. Epub 2023 Jul 5. PMID: 37410913.
  9. ↑ 9.0 9.1 9.2 9.3 Abbott S, Chan F, Tahrani AA, Wong SH, Campbell FEJ, Parmar C, Pournaras DJ, Denton A, Sinclair AJ, Mollan SP. Weight Management Interventions for Adults With Idiopathic Intracranial Hypertension: A Systematic Review and Practice Recommendations. Neurology. 2023 Nov 21;101(21):e2138-e2150. doi: 10.1212/WNL.0000000000207866. Epub 2023 Oct 9. PMID: 37813577; PMCID: PMC10663033.
  10. ↑ Ko MW, Chang SC, Ridha MA, et al. Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study. Neurology. 2011;76(18):1564-1567. doi:10.1212/WNL.0b013e3182190f51
  11. ↑ Szewka AJ, Bruce BB, Newman NJ, Biousse V. Idiopathic intracranial hypertension: relation between obesity and visual outcomes. J Neuroophthalmol. 2013;33(1):4-8. doi:10.1097/WNO.0b013e31823f852d
  12. ↑ 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
  13. ↑ 13.0 13.1 Mollan SP, Mitchell JL, Yiangou A, Ottridge RS, Alimajstorovic Z, Cartwright DM, Hickman SJ, Markey KA, Singhal R, Tahrani AA, Frew E, Brock K, Sinclair AJ. Association of Amount of Weight Lost After Bariatric Surgery With Intracranial Pressure in Women With Idiopathic Intracranial Hypertension. Neurology. 2022 Sep 13;99(11):e1090-e1099. doi: 10.1212/WNL.0000000000200839. Epub 2022 Jul 5. PMID: 35790425; PMCID: PMC9536743.
  14. ↑ Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuroophthalmol. 2004;24(2):138-145. doi:10.1097/00041327-200406000-00009
  15. ↑ 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
  16. ↑ Goyal A, Zarroli K. Should topiramate be initial therapy in the management of idiopathic intracranial hypertension?: A literature review. Medicine (Baltimore). 2023 Oct 20;102(42):e35545. doi: 10.1097/MD.0000000000035545. PMID: 37861536; PMCID: PMC10589511.
  17. ↑ 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
  18. ↑ Mitchell JL, Lyons HS, Walker JK, Yiangou A, Grech O, Alimajstorovic Z, Greig NH, Li Y, Tsermoulas G, Brock K, Mollan SP, Sinclair AJ. The effect of GLP-1RA exenatide on idiopathic intracranial hypertension: a randomized clinical trial. Brain. 2023 May 2;146(5):1821-1830. doi: 10.1093/brain/awad003. PMID: 36907221; PMCID: PMC10151178.
  19. ↑ 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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