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Idiopathic Intracranial Hypertension

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Idiopathic Intracranial Hypertension

Idiopathic intracranial hypertension (IIH) sometimes also called pseudotumor cerebri (PTC), the more general term, is a neurological condition in which the pressure around the brain is elevated without the presence of a tumor or other cause on imaging. The more general term of pseudotumor cerebri, means “false brain tumor,” as the symptoms can mimic those of elevated intracranial pressure caused by a brain tumor and require careful diagnosis and management.

Naming convention note: “Idiopathic” means that the cause of a condition is unknown. So, in Idiopathic Intracranial Hypertension (IIH), “idiopathic” in the name suggests that the increased pressure inside the skull is happening without a clearly identifiable reason. However, in some cases the underlying cause is actually known—such as when the condition is medication-induced. For that reason, the older, broader term pseudotumor cerebri (PTC) remains more inclusive. Technically, IIH is a subtype of PTC, though the two terms are often used interchangeably in clinical settings. PTC is the older term, but it is still used—often to avoid alarming patients with the word “tumor” in a diagnosis that doesn’t actually involve one.

Causes and Risk Factors

The exact cause of IIH/PTC is unknown, but several factors are associated with increased risk:

  • Obesity, especially in women of childbearing age
  • Certain medications (such as tetracyclines or excessive vitamin A)
  • Hormonal changes
  • Blood clotting disorders that affect brain venous drainage
  • Narrowing of the venous outflow tracks that can affect brain venous drainage

Symptoms

Symptoms can be debilitating and may include:

  • Persistent, severe headaches that typically worsen when lying flat
  • Visual disturbances (episodic blurred vision, double vision, tunnel vision, or transient or permanent vision loss)
  • Pulsatile tinnitus (hearing a rhythmic sound in the ears)
  • Nausea and vomiting
  • Neck or shoulder pain

One of the most concerning complications is vision loss, which can become permanent if not treated.

Diagnosis

Diagnosis of idiopathic intracranial hypertension involves:

  • Ophthalmologic examination to check for swelling of the optic nerves (called papilledema if due to elevated pressure)
  • MRI or CT scan to rule out other causes
  • Lumbar puncture to measure cerebrospinal fluid (CSF) pressure and confirm elevated intracranial pressure
  • Visual field testing to monitor the extent of vision involvement
  • Optical coherence tomography to help assess and track the amount of swelling and damage to the optic nerves and retinae
  • Angiogram/venogram tests used to look for abnormalities in blood vessels

Treatment Options

The goal of treatment is to reduce intracranial pressure, relieve symptoms, and protect vision.

  • Weight loss: Even modest weight reduction can significantly improve symptoms.
  • Medications:
    • Acetazolamide (to reduce CSF production)
    • Topiramate (may also aid in weight loss and reduce pressure)
    • Steroids (typically only used in severe cases where vision is threatened)
  • Therapeutic lumbar punctures: Occasionally used for temporary relief.
  • Surgical interventions for more severe or refractory cases:

Prognosis

With early diagnosis and proper treatment, many patients experience significant improvement in symptoms and preservation of vision. Ongoing monitoring is essential to ensure pressure remains controlled and vision is stable.

When to Seek Medical Help

If you experience persistent headaches, vision changes, or pulsatile tinnitus, it’s essential to seek medical evaluation. Prompt diagnosis and management of IIH/PTC can help prevent permanent vision loss.

Contact Us

At Pacific Neuroscience Institute®, our team of experts specializes in diagnosing and treating IIH/PTC with individualized care plans. Contact us to schedule a consultation.