One day, on a particular remembered day, out of the blue, a headache starts. Then, the headache stays, and nothing seems to make it go away. After going to the doctor, maybe many doctors, no cause is found. This is how New Daily Persistent Headache (NDPH) begins. Sometimes there has been a cold or brief viral illness before the headache starts, and sometimes there is a seemingly unrelated stressful life event, but often there is no logical reason for the headache to begin so suddenly, or for it to stay.
Frequently, the person can remember the exact day the NDPH started. Often, there is no previous history of headaches. About 50% of those with NDPH have migraine features such as light and noise sensitivity, or nausea. Or, the headache may be more similar to a tension-type headache, mild, two-sided, and not pounding. There is no difference in the likelihood of improvement between those with NDPH with migraine features compared to those with tension-type NDPH. The diagnosis of NDPH requires that the headache persist for 3 months without stopping, and it must not be caused by another medical problem.
NDPH can occur at any age from childhood on, even into the 70s. The average age of onset is about 35. It is two to three times more common in women than men. Almost half of those with NDPH have a close relative with frequent headaches. Up to 30% of individuals have had a cold or flu-like illness before the start of the headache, about 10% recall a stressful life event beforehand, and about the same number had some surgery involving the head (but not brain surgery). That leaves at least 50% of individuals recalling no triggering event. Many headaches that are not NDPH have a similar abrupt onset, and sometimes sudden headaches can be dangerous. An onset of headache like a thunderclap in a storm can signal bleeding in the brain, a leak in cerebrospinal fluid, an infection, cancer, or unidentified trauma causing damage to the brain tissue. Uncommonly, untreated sinusitis or even severe high blood pressure can cause a continuous headache. However, if a headache continues for 3 months, with a completely normal examination, the likelihood of abnormal testing is low.
Blood testing should be performed with the new onset of continuous headache looking for anemia or an increase or decrease in white blood cells or platelets, the blood cells that help blood to clot. Further blood testing should make sure that the body’s chemistry is normal, and that the kidneys and liver are functioning as they should. Imaging of the head, such as a CAT scan (CT), magnetic resonance imaging (MRI), MRI of the arteries (MRA), and MRI of the veins (MRV) are ordered to make sure nothing is missed. A measure of spinal fluid pressure may be performed by placing a small needle in the back, a lumbar puncture, or spinal tap. The neck may be examined to make sure there is no compression of the nerves resulting in an ongoing headache. The blood vessels that supply the brain may also be checked to make sure there is no interruption in flow. In individuals older than 50, a blood erythrocyte rate (ESR) is drawn, and there may be a biopsy of the arteries at the temples if the ESR is elevated. The tests chosen depend on the person, the history of the headaches, as well as the examination.
When evaluating NDPH, it is important to make sure that the daily headache is not the result of medication overuse, which can also result in daily headache. If a person is using pain medications of any kind, including over the counter pills, more than 2 days per week, the diagnosis of NDPH cannot be made until this overuse of medication stops. Still, it is hard to figure out how medication overuse would start daily, continuous headache on a particular day. Although we recommend keeping as-needed medication use to less than 3 days per week, it is not clear that this intervention helps with NDPH.
People with NDPH want to know what will work to treat it, and when it will stop. Unfortunately, there is no definite answer to either question. There are two types of NDPH, the kind that goes away, and another sort that remains ongoing with or without treatment for a longer period of time, without a predictable end point. Outcome predictions vary. Some evaluations suggest that most people with NDPH will be headache free in 3 years. However, one series of 79 patients with NDPH reported that 1249 Headache: The Journal of Head and Face Pain VC 2016 American Headache Society Published by JohnWiley & Sons, Inc. doi: 10.1111/head.12831 around 75% of them continued to have daily headache at 5 years, and in some the headaches do persist indefinitely.
NDPH does not have known treatment or medicine that cures it. In the previous description of 79 NDPH patients, 10 patients had their headaches go away while taking a preventive medication. Six of these lucky patients had their NDPH go away while on nortriptyline, and 4 of the 10 were taking topiramate. Perhaps the reasonable course is a combination of living a healthy lifestyle with exercise, good diet, and regular sleep, coupled with preventive medicines typically used for other headache disorders, including blood pressure medications such as propranolol or candesartan, antidepressants that target pain receptors such as amitriptyline, nortriptyline, or venlafaxine, or antiseizure medications such as topiramate, or use of OnabotulinumtoxinA (Botox). Biofeedback, behavioral treatment, and relaxation therapy may also be of benefit.
The most challenging problems with NDPH come from getting to the diagnosis, and even with the diagnosis, the absence of a good proven treatment. The best results are likely to come from a blended approach. Most people who have NDPH are able to lead a normal life, with eventual improvement or effective management of their daily headache.
Deborah Tepper, MD
Beth Israel Deaconess Medical Center
Sandwich, MA, USA