CSF Leak: Signs, Symptoms and When to Seek Care

A headache, a runny nose, neck pain: Everybody gets them now and then. Most of the time these symptoms don’t indicate a serious disease, and they pass quickly on their own. But what if they don’t? How do you know when a headache is a sign of a cerebrospinal fluid (CSF) leak, a rare but debilitating disorder?

Here are answers to five key questions about this painful and sometimes dangerous condition, including telltale symptoms, when to see a doctor and what to expect if you need treatment.

What Is a CSF Leak?

There are two types of CSF leaks, and they cause different symptoms, said Wouter Schievink, MD, director of Microvascular Neurosurgery and the Cerebrospinal Fluid (CSF) Leak Program at Cedars-Sinai.

• A cranial CSF leak occurs when some of the fluid surrounding your brainseeps out from the lining around it, called the dura. A cranial CSF leaknormally causes a runny nose and carries a high risk of meningitis, aninflammation of the membrane around the brain and spinal cord, which can befatal and requires emergency attention.
• A spinal CSF leak happens in the spinal cord. During a spinal CSF lake, thevolume and pressure of fluid in the skull drops, causing the brain to slump,which can trigger severe headaches, vision and hearing disturbances, seizuresand other symptoms, such as neck pain and a heightened sense of smell.

How Common Is a CSF Leak?

Spontaneous CSF leaks (ones that are not triggered by a medical procedure, such as a lumbar puncture, or by a serious injury, such as a gunshot wound) are rare, occurring in roughly 5 in 100,000 people per year.

“Because doctors and patients alike aren’t used to seeing them, spontaneous CSF leaks are underdiagnosed,” said Schievink, who is one of the world’s foremost authorities on the condition.

Patients can suffer for a long time without knowing what’s wrong, and they may make repeated trips to the hospital or doctor’s office without getting clear answers or relief from their symptoms. Prompt medical attention can spare patients from disability and even save their life.

“The longer the diagnosis is delayed, the higher the risk of the leak continuing and having lasting effects,” said Schievink, who has successfully treated many patients who had lost hope after being told by other doctors that they didn’t know what was wrong or how to help.

When Should You Seek Medical Care?

Always pay attention to symptoms that are unusual for you. If your head hurts and the pain won’t go away, don't ignore it.

“Headaches caused by CSF leaks don’t feel like ordinary headaches. They’re typically more severe,” said Schievink.

One key characteristic of a CSF leak is that symptoms get much worse when you’re sitting or standing and better when you’re lying down. One particular combination of symptoms is also noteworthy: If you get bad headaches, neck pain and a clogged feeling in the ears all at once for a prolonged period of time, get checked out, especially if you also experience changes to your hearing, vision or other senses.

Who Is at Risk for a CSF Leak?

Risk factors include having a connective tissue disorder such as Marfan syndrome or Ehlers-Danlos syndrome, but a spontaneous CSF leak can happen to anyone. In many cases, the reason is simply unknown. The average age for a leak developing spontaneously is 42, but it can occur at any age, including in children. Women are about twice as likely as men to develop the condition.

What Happens If You’re Diagnosed?

In some cases, a CSF leak will heal with basic treatment, including strict bed rest, increased fluid intake and caffeine.

When that’s not enough, one successful approach is to use blood patches. This outpatient procedure uses the patient’s own blood to patch the leak. In more difficult cases, doctors can use a surgical sealant known as fibrin glue or perform surgical procedures. Relief can be quick and dramatic, and any continued discomfort is often more tolerable.

“Patients are often shocked to get the diagnosis, but they are also relieved because they finally know what’s wrong,” said Schievink. “If you suspect you may have a CSF leak, don’t delay and don’t despair. There is help here for you.”