Idiopathic Intracranial Hypertension (IIH), historically known as Pseudotumor Cerebri, is caused by a buildup of cerebrospinal fluid (CSF) around the brain. Some people produce too much CSF, while others have inadequate blood flow which prevents drainage of CSF. IIH was discovered shortly after the development of the lumbar puncture in the late 1800s but was not formally recognized until the mid-20th century. It has been increasingly diagnosed in recent decades.
I was diagnosed with IIH after I lost vision in one eye, and was sent to the hospital for a lumbar puncture to measure the pressure of my CSF. I have congenital venous stenoses, which means that I was born with kinks in the veins leaving my skull, which is the overall cause of my IIH. Imaging such as MRIs and MRVs are used to rule out other conditions. Medication, weight loss, and occasionally surgery are often prescribed, however, more research must be done to determine the efficacy of these treatments.
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For those interested in learning more about the condition, here are five things you should know about IIH.
1. Women are much more likely to be diagnosed.
Women are much more likely than men to be diagnosed with IIH. Folks at higher weights are more frequently diagnosed with IIH, and recent weight gain, however slight, may be a factor.
This is not true for everyone, and the overall lack of research in medical literature limits our knowledge of the hormonal and weight components of the condition. Doxycycline, an antibiotic with general uses, is sometimes known to be connected to IIH when used for malaria prophylaxis.
2. Severe headaches are the main symptom.
The major symptom of IIH is severe headaches. Headaches are frequently more severe when lying down or changing positions.
Papilledema, or swelling of the optic nerves/discs, is also common. This can cause temporary, though sometimes permanent, double vision or blindness. A “whooshing” sound in the ears, or pulsatile tinnitus, may also occur.
3. A lumbar puncture is part of the diagnostic process.
Diagnosis of IIH is made through a series of tests and imaging to rule out other conditions, as well as a lumbar puncture with an abnormally high opening pressure. The imaging is generally painless, but lumbar punctures can be very uncomfortable.
Measuring the opening pressure is done right as the needle enters the space between the vertebrae in the lower back. If it is high, extra fluid is often drained during this procedure to temporarily reduce the patient’s headache.
4. Treatment usually looks the same for most.
Most people with IIH are given the same medication and told to lose weight if they are in heavier bodies, even though there is still much to learn about the condition.
A medication called acetazolamide is the standard of care for folks diagnosed with IIH, though other medications are sometimes effective when side effects are unmanageable.
Serial spinal taps aren’t often done due to the risk involved, possible adverse effects such as spinal headaches, and because CSF regenerates so quickly that the relief may be short-lived. Surgery to relieve pressure around the optic nerve may be performed to preserve vision in folks at risk for losing their sight. Current research is being done to determine the effectiveness of stent placement in blood vessels in the head to relieve symptoms of IIH.
5. The stigma associated with body size leads to missed diagnoses of many conditions.
Focusing on weight loss as a treatment option can cause extreme frustration, and possibly exacerbate disordered eating in folks with IIH. Since there is so much left to learn about the condition, treatment approaches must be multifaceted and take into account personal history. It is essential that medical providers emphasize health instead of weight loss with their patients. Folks in heavier bodies are very aware of their size due to societal expectations and are bombarded with weight loss recommendations at nearly every doctor’s appointment (even for yearly physicals when otherwise healthy). The stigma associated with body size leads to missed, or at least prolonged, diagnosis of many conditions. IIH is not your fault, no matter the size of your body.
National Organization for Rare Disorders https://rarediseases.org/rare-diseases/idiopathic-intracranial-hypertension/
“Not so benign intracranial hypertension” https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1125522/
“Idiopathic Intracranial Hypertension” https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908600/