CSF Leak Headache

CSF Leak and CSF Leak Headache

We were recently contacted by a person who wanted to raise awareness of a condition she has been suffering from. She was diagnosed as having a CSF Leak caused by a lumbar puncture. One of the disabling symptoms that she has been experiencing is a severe headache.

A CSF Leak can occur when a small hole or tear happens in the membranes around the brain or spinal cord, allowing fluid to leak out. It can happen spontaneously, without a known cause or can be caused by something else such as an epidural injection, a lumbar puncture or physical trauma.

Cancer Research UK / Wikimedia Commons

It is rare, but it is extremely painful and disabling. It does share symptoms with other headache disorders such as migraine or tension-type headache. Misdiagnosed patients are often treated for these headache disorders instead.

For many it is temporary and is generally fixed after the hole or tear seals naturally or after a patch is applied through surgery. For others however, the leak reoccurs and they continue to suffer from severe headache and other disabling symptoms.

The Scientific Part!

So, what is CSF?

Cerebrospinal Fluid (CSF) is a clear liquid that surrounds and cushions the brain and the spinal cord. It protects both from injury.

Blausen.com staff (2014). “Medical gallery of Blausen Medical 2014“. WikiJournal of Medicine 1 (2). DOI:10.15347/wjm/2014.010. ISSN 2002-4436.

What is a CSF Leak Headache?

Simply put, it’s a headache that occurs after the fluid leaks from around the brain or the spine due to a hole or tear, causing low Cerebrospinal Fluid pressure.

The International Classification of Headache Disorders 3rd edition describes a CSF Leak Headache as,

Headache attributed to low cerebrospinal fluid (CSF) pressure.

Orthostatic (relating to or caused by sitting upright) headache caused by low cerebrospinal fluid (CSF) pressure (either spontaneous or secondary), or CSF leakage, usually accompanied by neck pain, tinnitus, changes in hearing, photophobia and/or nausea. It remits after normalization of CSF pressure or successful sealing of the CSF leak.

Diagnostic criteria:

Any headache fulfilling criterion C.
Either or both of the following:
low cerebrospinal fluid (CSF) pressure (<60mm CSF)
evidence of CSF leakage on imaging
Headache has developed in temporal relation to the low CSF pressure or CSF leakage, or led to its discovery.
Not better accounted for by another ICHD-3 diagnosis.

How does it feel?

The headache is described as a dull, throbbing pain that varies in intensity from mild to severe and is positional, i.e., the headache gets worse when the person is sitting up and gets better when the person is lying down.

This is caused by what’s called ‘Intracranial Hypotension’ – when the pressure in the area is reduced. It usually happens within 15 minutes of sitting up and relieved after lying down within 15-30 minutes.

The Really Scientific Part!

The ICHD 3 describes 3 types of CSF Headache:

Post Dural Headache – Caused by Lumbar Puncture

- Headache occurring within five days of a lumbar puncture, caused by cerebrospinal fluid (CSF) leakage through the dural puncture. It is usually accompanied by neck stiffness and/or subjective hearing symptoms. It remits spontaneously within two weeks, or after sealing of the leak with autologous epidural lumbar patch.

Cerebrospinal fluid (CSF) fistula headache – Caused by a procedure or trauma

- Orthostatic headache occurring after a procedure or trauma causing a persistent cerebrospinal fluid (CSF) leakage resulting in low intracranial pressure. It remits after successful sealing of the CSF leak

Spontaneous Intracranial Hypotension – Spontaneous or unknown cause

- Orthostatic headache caused by low cerebrospinal fluid (CSF) pressure of spontaneous origin. It is usually accompanied by neck stiffness and subjective hearing symptoms. It remits after normalization of CSF pressure.

You can see that in each description the headache ‘remits’ after a blood patch or when CSF Pressure is back to normal.

What if it does not resolve?

Unfortunately, this is not always the case and for many people living with the symptoms of CSF leaks, life does not return to normal.

Some people suffer from continuous leaks due either to unsuccessful treatment or delayed diagnosis and suffer from long-term disability.

They can experience the following:

severe chronic pain
visual disturbance
seizures
nerve damage

Due to these and other disabling symptoms, sufferers may be unable to work, perform any kind of physical exercise or basic day-to-day duties we all do that people without these symptoms take for granted.

Symptoms of a CSF leak

The most common symptoms of a CSF leak in the spinal area (Spinal CSF Leak) are:

Positional headaches – sitting up makes them worse, lying down makes them better
Nausea and vomiting
Neck pain or stiffness
Change in hearing (muffled, ringing in the ears)
Sense of imbalance
Dizziness
Vertigo
Photophobia (sensitivity to light)
Phonophobia (sensitivity to sound)
Brain Fog
Pain between the shoulder blades

The most common symptoms of a CSF leak in the areas surrounding the brain (Cranial CSF Leak) are:

Drainage from the nose
Drainage from the ear
Change in hearing or ringing in the ears
Salty or metallic taste in the mouth
Sense of drainage down back of throat
Loss of sense of smell

As Migraineurs, many of us suffer from similar debilitating symptoms and practical day-to-day issues. This means we can empathise if not sympathise with those suffering a CSF Leak.

Diagnosis of a CSF Leak

The doctor will perform a physical examination of the person and look at their history, e.g., Did they recently have an epidural? Did they have a lumbar puncture recently? Was the patient in an accident or did they experience trauma?

If a Cranial CSF leak is suspected, the doctor will ask the person to lean forward to see if the discharge from the nose increases. If so, this is collected and then sent to the lab for tests to confirm that is cerebrospinal fluid.

The doctor might also order some scans such as:

Computed tomography (CT)
Magnetic resonance imaging (MRI)
Myelography (takes images of the spine, spinal cord and surrounding structures to look for abnormalities)
Cisternogram(to determine if there is abnormal CSF flow within the brain or spinal canal)
Intrathecal Fluorescein (visible dyes and other solutions injected into the area to try to pinpoint a leak)
Lumbar Puncture (also called a spinal tap)
Intracranial Pressure Monitoring (via a device inside the head to measure the pressure inside the skull)
Nasal Endoscopy (thin tube, camera and light inserted into the nasal cavity)
Beta-2-Transferrin Testing (a test for a specific protein only found in CS Fluid)

For further information on these tests see https://www.csfleak.info/what-is-a-cerebrospinal-fluid-csf-leak/diagnosis/

Diagnosis and treatment of spontaneous intracranial hypotension due to cerebrospinal fluid leakage”, Springer Plus doi:10.1186/s40064-016-3775-z Yake Zheng, Yajun Lian, Chuanjie Wu, Chen Chen, Haifeng Zhang, Peng Zhao

Treatment of a CSF Leak

Many CSF leaks heal on their own but treatment may include:

Bedrest
Oral and IV fluids
Oral and IV caffeine(strong coffee or tablets)

For spinal CSF leaks that do not respond to the above treatments the following surgical options can be tried:

Blood patch – done by injecting the person’s own blood into the spinal canal. The blood clot produced then “patches” the hole where the leak is occurring.

Epidural patching with fibrin glue.

If an epidural blood patch does not work, other surgical repairs may be necessary.

Sources of Information

The CSF Leak Association in the UK have some very good patient information fact sheets.

Here’s an example https://www.csfleak.info/download/1602/

Other excellent sources of information are

Spinal CSF Leak Foundation

Cedars Sinai

Information in this article was used with kind permission from the CSF Leak Association UK, Cedars Sinai and the Spinal CSF Leak Foundation in the US and the International Headache Society

The information above is for information only and is not intended to replace medical advice or diagnosis. Please see your doctor or pharmacist for medical information or advice.