New Daily Persistent Headache - The Basics
In the last several years, New Daily Persistent Headache (NDPH) has come to be recognized as a distinct primary headache syndrome. Primary headache disorders are those for which there is no underlying secondary cause that can be identified. As with Migraine disease and some other headache disorders, there are several secondary conditions that can mimic NDPH, so they must be ruled out before a diagnosis of NDPH can be confirmed. Two conditions in particular that must be ruled out are spontaneous cerebrospinal fluid (CSF) leak and cerebral venous sinus thrombosis. Headache from a spontaneous CSF leak is usually affected by body position, but the longer it continues, the less apparent that becomes. Therefore, patients may not think to mention that their headache was, at one point, affected by body position, and that maybe missed.
What is new daily persistent headache?
The best way to define NDPH is to excerpt that section of the International Headache Society's International Classification of Headache Disorders:
4.8 New daily-persistent headache (NDPH)
Previously used terms: De novo chronic headache; chronic headache with acute onset
Persistent headache, daily from its onset, which is clearly remembered. The pain lacks characteristic features, and may be migraine-like or tension-type-like, or have elements of both.
Persistent headache fulfilling criteria B and C
Distinct and clearly remembered onset, with pain becoming continuous and unremitting within 24 hours.
Present for more than three months.
Not better accounted for by another ICHD-3 diagnosis.
4.10 New daily persistent headache (NDPH) is unique in that headache is daily from onset, and very soon unremitting, typically occurring in individuals without a prior headache history. Patients with this disorder invariably recall and can accurately describe such an onset; if they cannot do so, another diagnosis should be made. Nevertheless, patients with prior headache (1. Migraine or 2. Tension-type headache) are not excluded from this diagnosis, but they should not describe increasing headache frequency prior to its onset. Similarly, patients with prior headache should not describe exacerbation followed by medication overuse.
4.10 New daily persistent headache (NDPH) may have features suggestive of either 1. Migraine or 2. Tension-type headache. Even though criteria for 1.3 Chronic migraine and/or 2.3 Chronic tension-type headache may also be fulfilled, the default diagnosis is 4.10 New daily persistent headache (NDPH) whenever the criteria for this disorder are met. In contrast, when the criteria for both 4.10 New daily persistent headache (NDPH) and 3.4 Hemicrania continua are met, then the latter is the default diagnosis.
Abortive drug use may exceed the limits defined as causative of 8.2 Medication-overuse headache (qv). In such cases, the diagnosis of 4.10 New daily persistent headache cannot be made unless the onset of daily headache clearly predates the medication overuse. When this is so, both diagnoses, 4.10 New daily persistent headache (NDPH) and 8.2 Medication-overuse headache, should be given.
In all cases, other secondary headaches such as 7.1 Headache attributed to increased cerebrospinal fluid pressure, 7.2 Headache attributed to low cerebrospinal fluid pressure and 5.1 Acute headache attributed to traumatic injury to the head should be ruled out by appropriate investigations.
4.10 New daily persistent headache (NDPH) has two subforms: a self-limiting subform that typically resolves within several months without therapy, and a refractory form that is resistant to aggressive treatment regimens. These are not separately coded.
In 2002, Li and Rozen conducted the largest study of NDPH to date based on 56 patients from the Jefferson Headache Center in Philadelphia. Some interesting points from the study included:
82% of patients were able to pinpoint the exact day their headache started.
In 30% of the patients, the onset of the headache occurred in correlation with an infection or flu-like illness.
38% of the patients had a prior personal history of headache.
29% of the patients had a family history of headache.
68% reported nausea.
66% reported photophobia.
61% reported phonophoiba.
55% reported lightheadedness.
Imaging and laboratory testing was unremarkable except for an unusually high number of patients who tested positive for a past Epstein-Barr virus infection.
How is NDPH diagnosed?
As mentioned above, other conditions must be ruled out before arriving at a diagnosis of NDPH. Goadsby et al recommend that evaluation of an NDPH patient should include MRI with and without enhancement and MRA (Magnetic Resonance Angiography). These are done to rule out other conditions such as the spontaneous cerebrospinal fluid (CSF) leak and cerebral venous sinus thrombosis discussed earlier. If these tests are negative, Goadsby et al recommend considering a lumbar puncture (spinal tap) to rule out infection as well as conditions related to CSF pressure such as pseudotumor cerebri, which can also mimic NDPH.
What is the treatment for NDPH?
Many doctors consider NDPH to be the most treatment refractory (not responsive to treatment) of headache disorders. Unfortunately, NDPH can be very disabling because it often does not respond to preventive or abortive medications. Some cases have shown successful preventive treatment with Neurontin (gababentin) and Topamax (topiramate). Otherwise, since no successful treatment regimens have been devised specifically for NDPH, most specialists work with the same medications prescribed for chronic Migraine.
New daily persistent headache is now classified as a primary headache disorder. As you look at the symptoms, you'll find that some of them are characteristic of tension-type headache; others are more characteristic of Migraine disease. NDPH is unique, however, in that many patients can tell you the exact date when their headache began. It is characterized by continuous daily head pain, varying in intensity, and sometimes accompanied by some Migrainous symptoms. It's important that NDPH be diagnosed carefully and correctly after ruling out other conditions that can present the same symptoms. Unfortunately, at this time, there are no treatments specifically outlined for NDPH.
Headache Classification Committee of the International Headache Society. "The International Classification of Headache Disorders, 3rd edition (beta version)." Cephalalgia. July 2013 vol. 33 no. 9 629-808 10.1177/0333102413485658
Goadsby, Peter J., MD, PhD, DSc, FRACP, FRCP; Silberstein, Stephen D., MD, FACP; Dodick, David W., MD, FRCPD, FACP. "Chronic Daily Headache for Clinicians." Hamilton, Ontario: BC Decker. 2005.
Li, D & Rozen, TD (2002). "The clinical characteristics of new daily persistent headache." Cephalalgia 22 (1), 66-69. doi: 10.1046/j.1468-2982.2002.00326.x.