Idiopathic Intracranial Hypertension - Symptoms & Causes

What is idiopathic intracranial hypertension?

Idiopathic intracranial hypertension (IIH), also known as benign intracranial hypertension or pseudotumour cerebri, is a condition characterised by increased pressure within the skull (intracranial pressure) without an obvious cause.

This pressure can lead to swelling of the optic nerve and, if left untreated, may result in vision loss. The condition is termed "idiopathic" because its exact cause is unknown.

Types of idiopathic intracranial hypertension

IIH is generally classified based on its cause and presentation:

  • Primary IIH (Idiopathic). This type has no identifiable cause and is the most common form.
  • Secondary IIH. This type occurs due to an underlying condition or factor, such as certain medications, hormonal imbalances, or other medical conditions.

What are the symptoms of idiopathic intracranial hypertension?

Symptoms of IIH can vary but often include:

  • Persistent and severe headaches, often described as throbbing and worsened by movement.
  • Blurred vision, double vision (diplopia), and temporary loss of vision (visual obscurations).
  • Papilloedema, or the swelling of the optic disc, visible during an eye examination.
  • Tinnitus, or a whooshing or pulsating sound in the ears.
  • Nausea and vomiting, often accompanying headaches and increased intracranial pressure.
  • Neck and shoulder pain.
  • Difficulty with balance and coordination.

What causes idiopathic intracranial hypertension?

The exact cause of IIH is unknown, but several factors are thought to contribute to its development:

  • There is a strong association between obesity and IIH, particularly in women of childbearing age.
  • Hormonal changes or imbalances, such as those related to the menstrual cycle, pregnancy, or polycystic ovary syndrome (PCOS), may play a role.
  • Certain medications, including oral contraceptives, antibiotics like tetracycline, and some types of steroids, can increase the risk of IIH.
  • High levels of vitamin A or retinoid therapy have been linked to the condition.
  • Conditions like sleep apnoea, kidney disease, and some autoimmune disorders can be associated with IIH.

If left untreated, IIH can lead to several serious complications:

  • Persistent increased intracranial pressure can damage the optic nerve, leading to irreversible vision loss.
  • Ongoing headaches that significantly impact quality of life.
  • Papilloedema, or the continued swelling of the optic nerve, causing ongoing visual disturbances.

How do you prevent idiopathic intracranial hypertension?

While there is no guaranteed way to prevent IIH, certain measures may reduce the risk:

  • Maintain a healthy weight. Weight management through a balanced diet and regular exercise can lower the risk of developing IIH, especially in individuals who are overweight or obese.
  • Monitor medications. Discuss with your healthcare provider the potential side effects of any medications you are taking, particularly those known to increase the risk of IIH.
  • Regular health check-ups. Routine medical examinations can help detect and manage any underlying conditions that might contribute to IIH.
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