Intracranial Hyper/Hypotension

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Intracranial Hypertension (IH) previously known as Pseudotumor Cerebri, is a rare neurological disorder that impacts around 1 in 100,000 people. This occurs when there is too much cerebrospinal fluid (CSF) in the skull which puts pressure on the brain and optic nerve. There are a variety of reasons this can occur but in most cases it's idiopathic (unknown origin). 

Symptoms:

  • Headaches

  • Visual Disturbances

  • Tinnitus

  • Vertigo/Dizziness

  • Nausea

  • Memory/Cognitive Issues

Diagnosis is usually through a Lumbar Puncture (LP) or Invasive Instracrannial Pressure Monitor. Further tests such as CT and MRI are performed to check for potential causes of the high intracranial pressure and check for swelling of the optic nerves and issues such as Empty Sella. Test with an Ophthalmologist is also required to check for any damage to the optic nerve or issues like Papilledema (swelling in the optic nerve)

 

Treatment usually begins with medications such as Diamox or Topiramate. If medication and some doctors may perform further LP's to reduce pressure. Surgical intervention is sometimes required with either a shunt or stent. A shunt is a device that's implanted in either around the spine or brain with tubing leading to other areas for the excess CSF to drain to. Some research as shown upwards of 90% of patients with IH have narrowing of the veins under the skull. Surgical stenting can be used as another treatment option for narrow veins.

There are some reports of links between IH and EDS. The lining (dura) around the spinal cord in patients with EDS can be weak which increases the risk of developing cerebrospinal fluid leaks from lumbar punctures. Care needs to be taken with such a test to allow the area punctured by the needle to heal. 

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Spontaneous CSF leak can also occur in patients with EDS which can result in Intracranial HYPOtension. This is a condition where there isn't enough CSF in the skull. One of the most common symptoms is a headache that gets worse when upright (as more CSF drains away). This headache often gets worse as the day progresses and the person spends more time upright. Its also often improved when lying flat.

Other potential symptoms:

  • Neck pain

  • Nausea/vomiting

  • Impaired Balance

  • Brain Fog

  • Fatigue

  • Coathanger Pain ​

Due to the potential weak dura, people with EDS can be at risk after a procedure/surgery that pierces the dura or even spontaneous leaks. Care needs to be taken to decrease these risks with surgery/procedures. Leaks can be large and cause build-ups of CSF (Pseudomeningocele) that can be seen in MRI's. Other times they can be slow and not able to be visualised on an MRI. Other tests may be needed to identify the area of the leak. Leaks can also cause cerebellar ptosis/sag which can be seen via an MRI. This is different from Chiari Malformation

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