The Masquerading Headache:Chronic Daily Migraine, Positional Headache, POTS, or Spinal Fluid Leak? Why It Hurts When You Stand Up

Posted By SHL Librarian

Presented by: Ian Carroll, MD, MS
Assistant Professor of Anesthesiology
January 14, 2016

People who are suffering severe recurring headaches, neck pain, nausea, vomiting, dizziness, ringing in the ears, diffuse pain, fatigue, or brain fog may have a fixable condition that frequently goes unrecognized. The key to the diagnosis is recognizing that these symptoms get worse the longer the person is upright.

For some patients symptoms are rapidly progressive when someone becomes upright and rapidly relieved by lying down, but for others the postural symptoms are subtle. For these patients, headache, dizziness or other symptoms may only happen or be recognizably worse late in the day. In contrast, mornings for these patients after being recumbent all night are often better than the rest of the day. Doctors often recognize the first group, but fail to properly recognize the second pattern.

Ian Carroll, MD, thinks many can be helped if they find a doctor who recognizes the cause: a cerebrospinal fluid (CSF) leak.

Spinal fluid leaks occur when there is a tear or leak of the covering called the dura that surrounds the spinal cord and the brain. The dura functions like a watertight bag holding spinal fluid that bathes and protects the spinal cord and the brain. If the dura is pierced or torn, the fluid can leak. That can lower pressure around the brain, leading to “orthostatic” headaches that occur when someone stands up or sits up.

The telltale signs are headaches, neck pain, nausea, vomiting, dizziness, ringing in the ears (tinnitus), diffuse pain, fatigue, or brain fog that are worse when standing upright or late in the day (after patients have been upright for many hours). Patients can sometimes experience weird symptoms that make them worry they have a neurologic disease such as multiple sclerosis or visual disturbances, double vision, patchy numbness or tingling in the face or limbs, unusual smells (experienced as smelling odors that others don’t), and disturbances of taste like a persistent metallic taste in the mouth. Sometimes CSF leaks come with nausea, chronic fatigue and a racing pulse called tachycardia. The headaches have left some people without relief, trying to cope with an “invisible” disability that can ruin careers and rob them of a normal life, said Dr. Carroll, an assistant professor of anesthesiology, perioperative and pain medicine.

Disabling headaches from spinal fluid leaks have been in the news recently when the head coach of the Oakland-based Warriors professional basketball team, Steve Kerr, went on medical leave for several months while he recovered from this. Dr. Carroll said he hasn’t been involved in Kerr’s medical care, but he has seen plenty of other patients struggling with similar symptoms.

Spinal fluid leaks can often occur from whiplash injuries in a car accident or other physical trauma, but surprisingly many patients cannot identify a preceding trauma. Sometimes CSF leaks result from medical procedures like surgery or a spinal tap. Most doctors do not know that spinal taps can leak chronically or that leaks can present other than as a severe headache that is completely and rapidly relieved by lying down. Some cases occur spontaneously in people who have unusual connective tissue in their bodies and don’t know it, Dr. Carroll said.

People with CSF leaks are usually misdiagnosed. They’re told they have chronic migraines, chronic fatigue syndrome, fibromyalgia, or less known conditions like Chiari malformation, Tarlov cysts or POTS (postural orthostatic tachycardia syndrome).  A positive tilt table test and autonomic testing do not rule out a CSF leak as the underlying cause of the syndrome.

Dr. Carroll developed the hunch that some headaches were caused by spinal fluid leaks after his own family experience. After his young daughter had a spinal tap to evaluate some unrelated symptoms, she started getting headaches when she wasn’t lying down. “When she sat up, she screamed in pain,” he said. After the headaches persisted, she was treated for spinal fluid leaks and got better. That got his attention.

Then he read a New York Times column written by a Yale internist, Dr. Lisa Sanders, about a young woman who had endured 3 months of intractable headaches that started after she had whiplash. She later developed dizziness when upright, and  her pulse jumped from 74 when she was lying down to 130 when sitting up.

She was diagnosed with POTS, or postural tachycardia syndrome, a condition thought to be caused by blood vessels not constricting enough to keep blood flowing to the brain when a person stands up. Dr. Carroll suspects this patient and others were misdiagnosed and more likely had a CSF leak. He then began wondering if people at Stanford were being misdiagnosed as well. One of the reasons is that spinal fluid leaks may not show up on standard medical tests.

When spinal fluid pressure is extremely low inside the skull, it can show up on standard MRI tests. But many times the pressure drop may not be enough to cause MRI findings but are still enough to cause headaches, nausea and other symptoms, Dr. Carroll said. He has seen multiple headache patients whose MRI scans looked normal, but when they were given treatment to fix a spinal fluid leak, their headaches stopped.

Most physicians are trained to rely on MRI scans to determine if spinal fluid leaks are causing headaches, so they may miss this, Dr. Carroll said. “Most doctors think they know about this problem, but what they know is wrong,” he said.

Based on the patients he has seen, he said, “Most people are not extreme leakers. A lot of leaks may be more subtle.”

Another test, called a CT myelogram, is more likely to detect leaks and more commonly see the things that leak, such as aneurysms in the dura called meningeal diverticula or perineural cysts, Dr. Carroll said. But even that doesn’t show some leaks, in his experience.

Based on the patients he has seen, Dr. Carroll outlined three major causes of tears in the dura that cause spinal fluid leaks. A major cause, already widely known, can be any medical procedure that pierces the dura. That can be spinal surgery, a spinal tap or even the epidural anesthesia that some women get in childbirth. A headache that develops the day after any of these procedures is called a “post-dural puncture headache,” or PDPH, and is caused by a single leak. In most cases, a next-day headache after any of these procedures often goes away on its own as the dura heals and the leak stops. If it doesn’t stop, a treatment called a “blood patch” can be done that seals the leak in 90 percent of cases, Dr. Carroll said.

Two other causes of headaches from spinal fluid leaks are spontaneous, Dr. Carroll said. The first is a bone spur or any bony calcification that pierces the dura. People with degenerative changes in their spine can have bulging spinal discs that calcify and can then poke through the dura to cause a leak at a single site or multiple sites, he said. CT myelograms can detect bone spurs, but some patients with unexplained headaches may not get this test or their doctor doesn’t understand the connection between an “osteophyte” (bone spur) reported from the CT myelogram of the spine and the patient’s headache or neurologic symptoms.

A third cause is having unusual connective tissue that leaves the dura thinner and more susceptible to tearing or leaking, Dr. Carroll said. This kind of connective tissue is unusually stretchy. People who have it may be unusually flexible or appear “double-jointed” compared to most.

“If you have connective tissue that’s extra stretchy and flexible, the bag that holds your fluid in is thinner and more susceptible to having a tear or leak,” Dr. Carroll said. A small whiplash injury or fall could trigger the leak without being detected. “People are wondering why you’re having all these headaches and neck pains after a car accident. They don’t go looking down in your back,” he said.

Patients with Ehlers-Danlos, Marfan syndrome, neurofibromatosis, and adult polycystic kidney disease are all known to have weaker connective tissue and be more susceptible to having undiagnosed CSF leaks. Other tipoffs that may indicate unusual connective tissue are being very tall (above 6-foot-2), having scoliosis, having many bulging discs throughout the spine, or diffuse arthritis.

People who are so hyperflexible they can bend their bodies into difficult poses for gymnastics or ballet that others can’t do may have the type of connective tissue difference linked to spinal leaks. Having cataracts earlier in life than usual—by age 40 or 50, rather than 65—can also be a tipoff for connective tissue differences.

This kind of unusual connective tissue won’t show up on an MRI, so it may go undetected and lead doctors to miss spinal fluid leaks that can occur at multiple sites in the spinal dura, Dr. Carroll said. He explained that research suggests that 30 percent to 40 percent of patients with spontaneous CSF leaks have more than one leak when they are diagnosed. This seemingly unlikely occurrence can happen because the connective tissues in these patients are inherently weaker than normal.

When a patient has a spontaneous leak the conventional treatment for a spinal-leak headache­—a blood patch on the dura—has a roughly 30 percent chance of stopping the headache with the first patch, Dr. Carroll said. If further patches don’t work, other options to fix a leak include fibrin “glue” seals or surgery to close any defects in the dura.

He is working with other physicians at Stanford and elsewhere to examine more patients with unexplained headaches that fit the profile of spinal fluid leaks: daily headaches that worsen when standing up (or late in the day), as well as nausea and dizziness. In the past 6 months, he’s found 26 people who had unexplained or misdiagnosed headaches who were helped by blood patches or other treatment for spinal fluid leaks.

He wants to find more. He urges friends and family of people suffering from unexplained chronic headaches to contact him at Stanford if they show the tipoff symptoms. If people with headaches feel much better every day in the morning after sleeping all night lying down, that’s another tipoff, Dr. Carroll said. Patients can help establish the diagnosis themselves by simply spending 24 hours lying flat.  People who are leaking will feel that day is one the best days in a long time.

“Having lives derailed by intractable headaches is a tragedy, but it is a much greater tragedy when the person has an unrecognized CSF leak that could be fixed  easily if only recognized. We can help them be back at work, and live life again more fully,” he said.

About the speaker:
Ian Carroll received his MD from Columbia University and did his internship and residency at Stanford University School of Medicine. He also received an MS in clinical epidemiology from Stanford. He is board certified in pain medicine and anesthesia by the American Board of Anesthesiology, and in addiction medicine by the American Board of Addiction Medicine. He has received research grants from the NIH, the Foundation for Anesthesia Education and Research (FAER), and Stanford University. He also received a faculty award for teaching excellence in Stanford’s pain management division. He has previously lectured on neuropathic pain for the Stanford Health Library.

About the Stanford Pain Management Center
Clinicians and researchers at the Stanford Pain Management Center have made major advances in the understanding of chronic pain as a distinct disease that fundamentally alters the nervous system. The center’s work has earned it a designation as a Center of Excellence by the American Pain Society.

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About Dr. Carroll

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