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Idiopathic Intracranial Hypertension is a specific condition characterized by high pressure in the fluid around the brain. It is also known as pseudotumor cerebri which literally means false brain tumor, because it's symptoms mimic those of brain tumors.



Exogenous substances

Exogenous substances associated with idiopathic intracranial hypertension include:

  • non-steroidal anti-inflammatory drugs (NSAIDs), tetracycline, nitrofurantoin, isotretinoin, tamoxifen, nalidixic acid, lithium, and the starting or stopping of steroids
  • amiodarone,
  • antibiotics,
  • carbidopa,
  • levodopa,
  • chlordecone,
  • corticosteroids,
  • cyclosporine,
  • danazol,
  • growth hormone,
  • indomethacin

Systemic diseases

The following diseases have been associated with idiopathic intracranial hypertension:

  • anemia,
  • chronic respiratory insufficiency,
  • familial Mediterranean fever,
  • hypertension,
  • multiple sclerosis,
  • polyangiitis overlap syndrome,
  • psittacosis,
  • Reye syndrome,
  • sarcoidosis,
  • systemic lupus erythematosus…
  • Disorders of cerebral venous drainage


It is proven that venous compression by extra-vascular tumors or secondary thrombosis results in impaired absorption of the cerebrospinal fluid leading to pseudotumor cerebri. 

Endocrine disbalances

Pregnancy is occasionally associated with idiopathic intracranial hypertension.

Secondary Intracranial Hypertension

While pseudotumor cerebri or primary intracranial hypertension is idiopathic, secondary intracranial hypertension always has a clear cause. Possible causes include:

  • dural venous sinus thrombosis
  • kidney failure
  • leukemia
  • lupus
  • excess Vitamin A
  • growth hormone treatments
  • nasal fluticasone propionate

Diagnosis of pseudotumor cerebri

The diagnosis of intracranial hypertension is made by identifying the typical symptoms of the disease along with documentation of a high spinal fluid pressure.

Recommended blood tests are:

  • Complete blood count
  • Erythrocyte sedimentation rate
  • Serum iron and iron binding capacity
  • Anti-cardiolipin antibodies/lupus anticoagulant
  • Antinuclear antigen (ANA) profile

Cerebrospinal fluid studies

  • Opening pressure
  • White blood cell and differential counts
  • Red blood cell count
  • Total protein
  • Quantitative protein electrophoresis
  • Glucose
  • Aerobic bacterial culture and sensitivity


A CT scan, or MRI of the head and brain should be done although the result is usually normal. Sometimes the ventricles may appear smaller. The neurological examination is also normal except for the presence of swollen optic nerves called papilledema.

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