Idiopathic intracranial hypertension (IIH) 

| NEURO-OPHTHALMOLOGY

WHAT IS IIH?

Idiopathic intracranial hypertension (IIH), also known as Pseudotumor Cerebri (PTC), is increased pressure in your skull. It occurs because of cerebrospinal fluid (CSF) buildup around your brain. When CSF builds up in your skull, it can put pressure on your optic nerve. This can cause symptoms of headache, ringing in your ears (tinnitus), vision loss when standing, and vision loss. This condition can occur in children and adults, but most commonly occurs in women of childbearing age who are obese.

Idiopathic intracranial hypertension (IIH), also known as Pseudotumor Cerebri (PTC), is increased pressure in your skull. It occurs because of cerebrospinal fluid (CSF) buildup around your brain. When CSF builds up in your skull, it can put pressure on your optic nerve. This can cause symptoms of headache, ringing in your ears (tinnitus), vision loss when standing, and vision loss. This condition can occur in children and adults, but most commonly occurs in women of childbearing age who are obese.

WHAT ARE THE RISK FACTORS FOR IIH?

  • Obesity: body mass index (BMI) above 30

  • Chronic kidney disease

  • Conditions that affect hormones such as Cushing syndrome, hypo/hyper-thyroid disease, underactive parathyroid glands, Addison’s disease

  • Iron deficiency anemia

  • Lupus

  • Polycythemia vera

  • Blood-clotting disorders

  • Sleep apnea

  • Medications such as birth control, growth hormone, tetracycline antibiotics (ex. Doxycycline or Minocycline), too much vitamin A or retinol use)


WHAT ARE THE RISK FACTORS
FOR IIH?

WHAT ARE THE SYMPTOMS OF IIH?

  • Severe headache that may start behind the eyes

  • A whooshing sound in your head that pulsates with your heartbeat (pulsatile tinnitus)

  • Dizziness, nausea, vomiting

  • Vision loss

  • Brief episodes of vision loss, lasting for a few seconds when changing positions (ex. When standing up)

  • Double vision

  • Neck, shoulder, and/or back pain

If IIH (idiopathic intracranial hypertension) is suspected, an ophthalmologist will check for optic nerve swelling (papilledema) and assess for blind spots with a visual field test. An OCT (optical coherence tomography) will also be done to measure your optic nerve and retina. If needed, brain imaging like MRI and MRV may be conducted. Additionally, a spinal tap might be performed to measure the pressure inside your skull and test the spinal fluid.

HOW IS IIH DIAGNOSED?

WHAT IS THE TREATMENT OF IIH?

The goal of treatment for IIH is to alleviate symptoms and prevent vision loss. A 5%-10% weight loss, often achieved with a low-sodium diet and possibly with the help of a dietitian, can reduce symptoms and medication needs.

Medications like acetazolamide (Diamox) and topiramate (Topamax) decrease CSF production, though they may cause side effects such as stomach upset, fatigue, and tingling.

A diuretic might be added to enhance urine output and manage fluid retention. Migraine medications are also used to tackle severe headaches associated with IIH.

In severe cases, surgery might be necessary. Optic nerve sheath fenestration, which involves making a window in the optic nerve sheath to let spinal fluid escape, can stabilize or improve vision, though results can vary. 

Another surgical option is a spinal fluid shunt, which drains excess CSF from the brain or spine to the abdomen, but may require maintenance or repairs over time.


WHAT IS THE TREATMENT OF IIH?

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