Overview

IdiopathicThe term idiopathic is used when there is no detectable reason for something. intracranial hypertension (IIH) is a disorder of elevated pressure within the brain without an identifiable cause. This increased pressure can lead to permanent damage to the brain and eyesight if left untreated. The term idiopathic is used when there is no detectable reason for increased pressure within the brain. However, as researchers continue to investigate potential causes, the term “idiopathic” may become a term of the past.¹

IIH is most commonly seen in overweight women in their reproductive years, usually in the 3rd and 4th decades of life. In the United States, approximately 19 per 100,000 women in this group are diagnosed with IIH.² With increasing rates of obesity in the US, defined as a body mass indexA value derived from the mass (weight) and height of a person. A healthy range is between 18.5-24.9, overweight is defined as 25-29.9 and obese… (BMIA value derived from the mass (weight) and height of a person. A healthy range is between 18.5-24.9, overweight is defined as 25-29.9 and obese…) of 30 or greater, the number of people affected by this syndrome is projected to increase.

The most common signs and symptoms of IIH are: headaches, dizziness, nausea, vision loss, and double vision.³ PapilledemaSwelling of the optic nerve that carries visual signals from the eye to the brain. – swelling of the optic nerve that carries visual signals from the eye to the brain – is a common finding during a visual exam in one with IIH. As swelling of the optic nerve persists, vision worsens and can progress to blindness which can be permanent.

Signs & Symptoms

Other signs and symptoms of IIH may include: radicular pain, sixth nerve palsy, and pulse synchronous tinnitus.³ Radicular pain is hypothesized to be an effect of increased cerebrospinal fluidFluid that is made by specialized cells in the ventricles of the brain. pressure, compressing nerves in the arms and legs causing the sensation of pins and needles. Sixth nerve palsy is the weakening of the lateral rectus muscle of the eye and can lead to double vision. Pulse synchronous tinnitus is the humming, whooshing, whistling, or marching noise that can be heard in one or both ears that is in sync with their own pulse.

Diagnosis

The diagnosis of IIH requires a detailed neurological history, complete physical, a visual exam (conventionally with an ophthalmologistA specialist in the branch of medicine concerned with the study and treatment of disorders and diseases of the eye.), lumbar punctureA procedure where a needle is placed in the lower part of the spine (the lumbar spine) to access cerebrospinal fluid., and imaging studies to diagnose this condition. If signs and symptoms are related to increased pressure within the brain, a lumbar puncture yields normal cerebrospinal fluid results and elevated opening pressure, and imaging studies of the brain do not show an identifiable reason for increased intracranial pressure, then IIH is suspected.

Diseases that are associated with increased pressure within the brain include: polycystic ovarian syndromeA hormonal disorder, seen in women, that is not well understood (PCOSA hormonal disorder, seen in women, that is not well understood), hypercoagulable state, obstructive sleep apnea, and systemic arterial hypertension.⁵,⁶ To learn about how these medical conditions increase intracranial pressure and how other conditions are linked to IIH, click here.

Medications that have shown to increase intracranial pressure are: anabolic steroids, withdrawal from chronic corticosteroids, vitamin A and retinoids, hormones, and certain antibiotics and can be a potential cause of IIH.³,⁷,⁸

There are a subset of people who do not fit the IIH criteria as stated above. These patients may not be overweight, may have normal opening pressure on lumbar punctureA procedure where a needle is placed in the lower part of the spine (the lumbar spine) to access cerebrospinal fluid., and may have medical conditions that can obscure signs and symptoms that are typical of IIH but in fact have elevated intracranial pressure due to venous congestion, venous stenosis or narrowing, and venous thrombosis.¹

As women’s health has proven an essential topic of medical interest, the discoveries made will help diagnose, treat, and prevent diseases that are female-related. Current medical research is studying how genetics, hormones, medications, and venous pressure contribute to this debilitating syndrome in hopes of a curative therapy.

Now that you have a better understanding of idiopathicThe term idiopathic is used when there is no detectable reason for something. intracranial hypertension, click here to learn more symptom relief, how to advocate for yourself, and how to treat IIH.

References

Fargen, K. (2019). IdiopathicThe term idiopathic is used when there is no detectable reason for something. intracranial hypertension is not idiopathic: proposal for a new nomenclature and patient classification. Journal of NeuroInterventional Surgery. November 27, 2019;12(2). doi:10.1136/neurintsurg-2019-015498
Wall M, Kupersmith MJ, Kieburtz KD, et al. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol. June 1, 2014;71(6):693-701. doi:10.1001/jamaneurol.2014.133
Ball A, Clarke C. Idiopathic intracranial hypertension. The Lancet Neurology. May 2006;5(5):433-442.
Friedman D, Liu G, Digre K. Revised diagnostic criteria for the pseudocerebri syndrome in adults and children. Neurology. August 21, 2013;81(13):1159-1165.
Markey K, Uldall M, Botfield H, et al. Idiopathic intracranial hypertension, hormones, and 11β-hydroxysteroid dehydrogenases. Journal of pain research. April 19, 2016;9:223–232. https://doi.org/10.2147/JPR.S80824
Glueck, C. J., Aregawi, D., Goldenberg, N., Golnik, K. C., Sieve, L., & Wang, P. (2005). Idiopathic intracranial hypertension, polycystic-ovary syndrome, and thrombophilia. Journal of Laboratory and Clinical Medicine, 145(2), 72–82. doi:10.1016/j.lab.2004.09.011
Libien J, Kupersmith MJ, Blaner W, et al. Role of vitamin A metabolism in IIH: Results from the idiopathic intracranial hypertension treatment trial. J Neurol Sci. January 15, 2017;372:78-84. doi:10.1016/j.jns.2016.11.014
Fuerst J, Wann R, Kline L. Vitamin A as a Cause of Pseudotumor Cerebri Syndrome: Not to Be Forgotten. Journal of Neuro-Ophthalmology. September 2021;41(3):403-406.

Edith Ruiz, PA-C

Edith Ruiz, PA-C is a certified physician assistant with over 14 years of clinical experience, including the care of neurological surgery and bariatric surgery patients. Additionally, she has several years of experience as a medical writer for healthcare companies to help connect patients to medical information.

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What is IIH?

Overview

Signs & Symptoms

Diagnosis

The criteria required to diagnose IIH are:

Patient stories

IIH Practitioner Series

What is IIH?

Overview

Signs & Symptoms

Diagnosis

The criteria required to diagnose IIH are:

Patient stories

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