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Idiopathic intracranial hypertension (IIH)* , formerly known as pseudotumor cerebri, * is a condition that affects the brain. Pseudotumor cerebri literally translates to "false brain tumor." This term was used because symptoms of IIH resemble those of brain tumors depsite no tumor being present. Symptoms of IIH may include severe headache, nausea and vomiting, altered vision, and pulsating sounds within the head. A person with IIH may also have symptoms such as a stiff neck, back or arm pain, eye pain, and memory problems. If the condition remains untreated, permanent visual loss or blindness may develop.
While the exact underlying cause of IIH is not known (idiopathic), it is likely due to high fluid pressure within the skull that is due to buildup, or poor absorption, of cerebrospinal fluid (CSF). IIH most often (but not always) occurs in young, overweight, females. When symptoms of intracranial pressure have an identifiable cause such as another underlying disease or medication, the condition is instead referred to as secondary IH.
Treatment for IIH may include medications called carbonic anhydrase inhibitors to control the production of CSF. In some cases, weight loss may also be recommended. However, medications and/or weight loss are not effective in all cases. In severe cases that do not respond to medication and weight loss, surgery may be needed to relieve symptoms and/or preserve vision.
Source: GARD Last updated on 05-01-20
80%-99% of people have these symptoms.
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| Nephrogenic diabetes insipidus |
| Increased intracranial pressure |
30%-79% of people have these symptoms.
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| Papilledema |
| Abnormality of the pulmonary artery |
| Obesity |
| Impaired Ig class switch recombination |
| Allergy |
5%-29% of people have these symptoms.
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1%-4% of people have these symptoms.
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| Lethargy |
| Pulsatile tinnitus |
| Migraine |
| Elevated 8(9)-cholestenol |
| Abnormal scrotal rugation |
| Broad face |
| Temporal cortical atrophy |
| Back pain |
| Depressivity |
| Olfactory auras |
| Vertigo |
An unknown % of people have these symptoms.
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| Limb apraxia |
| Hypertension |
| Autosomal recessive inheritance |
The outlook (prognosis) associated with idiopathic intracranial hypertension (IIH) is quite variable and difficult to predict in each person. In some cases, it goes away on its own within months. However, symptoms may return. It has been reported that regaining weight that was previously lost has been associated with symptoms returning in some people. Some individuals with IIH experience progressive worsening of symptoms, leading to permanent vision loss. Symptoms that cause pain and loss of visual function cause much of the burden associated with IIH.
IIH is not known to be associated with any specific mortality risk, but surgical procedures used to treat severe cases may affect quality of life or lead to a shortened lifespan due to complications.
Last updated on 05-01-20
Management options for idiopathic intracranial hypertension (IIH) can vary from person to person and may involve regular eye exams to monitor vision changes, medications, weight loss, and in some cases, surgery.
Medications that may be used to reduce CSF build-up and relieve intracranial pressure include acetazolamide and furosemide. Weight loss through dieting or weight loss surgery may also be recommended for people with IIH who are overweight or obese, although this does not lead to improvement of symptoms in all cases. If the above management options are not successful and symptoms are severe or permanent vision loss is possible, surgery may be needed. Types of surgery that may be needed may include a procedure to remove pressure on the optic nerve (optic nerve sheath fenestration), and CSF shunting, which involves surgically inserting a tube to drain cerebrospinal fluid.
Last updated on 05-01-20
Mollan SP, et al. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry 2018;89:1088–1100
Last updated on 04-27-20
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