Idiopathic Intracranial Hypertension: Symptoms are Common but the Disease is Rare


Jessica Walton described the staff at Queen Elizabeth Hospital in King’s Lynn as being in “a bit of a panic” when they thought that she was having a stroke. According to a recent article Cambridgeshire Live, Jessica had been diagnosed four years earlier with a rare disease known as intracranial hypertension (IH).

Jessica’s migraines began in 2015, but the cause of her headaches was unknown at the time. Hospital stays for many other symptoms kept Jessica from attending to her job as a mental health support worker. Bills began to pile up.

Jessica’s IH Symptoms

Jessica says the disease causes so much pain in her legs that her bones feel as if they are on fire. She can hardly lift her arms and her vision is severely affected.

The GoFundMe Account

Jessica’s unbearable pain, failed eyesight, and her other IH symptoms have prevented her from being able to work most days.

Only two days ago she discovered that her phone service had been turned off. Jessica is hoping that the GoFundMe account that she set up might bring in some funds to help her pay her overdue bills.

Access to Jessica’s GoFundMe campaign is available here.

About Idiopathic Intracranial Hypertension (IH)

The National Eye Institute describes IH as a condition that is caused by high pressure in the spaces surrounding the spinal cord and the brain.

The cerebrospinal fluid (CSF) that fills these spaces protects the brain from injury, eliminates waste and provides nourishment.

An estimated one hundred thousand U.S. residents  are known to have IH.

About IH Symptoms and Cause

IH affects vision in many ways. This includes blind spots, double vision, and temporary blindness; some patients may have permanent vision loss. Headaches are common as well as tinnitus (ringing in the ears) and shoulder or neck pain.

In chronic IH the CSF pressure may cause some swelling and some damage to the optic nerve. This condition is called papilledema.

Tetracycline and other drugs in its category are viewed as possibly causing IH. Other causes may be a blood clot in the patient’s brain, a brain tumor, or vitamin A overload.

Note that a diagnosis without any detectable cause is called idiopathic intracranial hypertension (IIH).

 Detecting IH

Risk factors are evaluated during a physical exam and review of the patient’s medical history. Doctors will also perform a neurological examination. A body mass index of 30 or higher is one of the major risk factors. A sudden weight gain of five to fifteen percent is another.

Various vision tests are performed, including a field of vision test to check the level of peripheral vision. Essential brain imaging checks for brain tumors or injury to the brain.

A spinal tap is used to measure CSF pressure. This procedure alone may result in a temporary reduction in CSF symptoms and pressure.

About Treatment for IH

Acetazolamide (Diamox) decreases CSF production. Diamox is an oral medication that is also used in weight loss therapy.

The drug’s side effects can be mitigated through dose adjustment or halting the drug completely. The side effects include nausea, fatigue, sensitivity in feet and hands, plus a metallic taste sensation usually after drinking carbonated soda.

Certain cases may require surgical procedures to alleviate pressure on the optic nerve.

Surgery called therapeutic shunting may also be required to clear excess CSF from brain cavities or ventricles to lower pressure.

Ongoing Clinical Trials

 The National Eye Institute’s IIH Treatment Trial is balancing the benefits of Diamox in 165 IH patients who have mild loss of vision against the known side effects of the drug.

The trial compares Diamox plus a weight loss program against an identical weight loss program and a placebo. The dosage was increased gradually to a maximum of four grams daily in an effort to avoid side effects.

The researchers found that over a six month period the drug plus weight loss program stabilized vision and in some cases restored patients’ vision. There will be a continuation of the trial for the next five years.

Information on IH clinical trials may be found at



Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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