NDPH (New Daily Persistent Headache)

New daily persistent headache (NDPH) is described as a headache that is daily and unremitting from or almost from onset. The pain is usually bilateral, pressing or tightening, and of mild to moderate intensity. [1] NDPH was first described in the literature by Vanast in 1986. [2]

Most patients who have NDPH know the exact date that their headache first began. It is one of the most treatment-refractory of all headache conditions. NDPH often begins in patients who have no significant headache problems in their past and can continue to persist for years to decades even with aggressive treatment. [3]

NDPH Diagnostic Criteria

The International Headache Society describes on NDPH diagnostic criteria on page 52 of their second edition of The International Classification of Headache Disorders: [1]

1. Headache for longer than 3 months.
2. Headache is daily and unremitting from onset or from less than 3 days from onset
3. At least two of the following pain characteristics:
   1. bilateral location
   2. pressing/tightening (non-pulsating) quality
   3. mild or moderate intensity
   4. not aggravated by routine physical activity such as walking or climbing stairs
4. Both of the following:
   1. no more than one of photophobia, phonophobia or mild nausea
   2. neither moderate or severe nausea nor vomiting
5. Not attributed to another disorder

There is believed to be two different subtypes of NDPH: 1) a self-limited form which will go away on it's own within a few months even without any treatment and 2) a refractory form which is near resistant to aggressive treatment. [3]

Clinical Features and Pathology

In 1986, Vanast identified 45 patients, 26 women and 19 men, with NDPH. [2] In 1993, Santoni and Santoni-Williams showed that 108 NDPH patients, 75 female and 29 male, at the Medical Center of the Universidad Central del Este in Santo Domingo, Dominican Republic, had evidence of systemic infection including Salmonella, Escherichia coil urinary tract infections, adenovirus, herpes zoster, toxoplasmosis, Epstein Barr virus, and pneuomonia. [4] In 2002, Li and Rozen identified 56 patients, 40 women and 16 men, with NDPH at the Jefferson Headache Clinic in Philadelphia, Pennsylvania. [5]

In 2003, Mack identified 40 children with NDPH, 27 female and 13 male, at the headache clinic at the Mayo Clinic in Rochester, Minnesota. [6] In 2004, Meineri et al. identified 18 NDPH patients, 11 female and 7 male, at the Headache Centre at S. Croce e Carle Hospital in Cuneo, Italy, and found association to herpes simple virus and cytomegalovirus. [7] Also in 2004, Takase et. al. identified 30 Japanese patients in Toyonaka Municipal Hospital with NDPH, 17 men and 13 women. [8] In 2006, Rozen et al. looked at 12 NDPH patients, 10 female and 2 male, at the Michigan Head-Pain and Neurological Institute (MHNI) in Ann Arbor, Michigan, and found that cervical spine joint hypermobility is a possible predisposing factor for the development of NDPH. [9] Rozen et al. 2007 looked at tumor necrosis factor-alpha (TNF-alpha), a proinflammatory cyotokine, in the CSF of 20 NDPH patients and found that in 19 of those patients TNF-alpha levels were elevated. This study by Rozen et al. 2007 postulates that NDPH is a disorder resulting from inflammation of the central nervous system (CNS). [10]

Based on examining the studies by Vanast 1986 [2], Li and Rozen 2002 [5], Meineri et al. 2004 [6], Takase et. al. 2004 [8], Rozen 2011 lists the clinical characteristics of new daily persistent headache which are: [3]

• Gender: female predominance noted in three out of four studies
• Age of onset: younger in women, many in their 20s and 30s
• Location: bilateral in most
• Intensity: moderate to severe in most patients
• Pain duration: constant without pain-free time
• Associated symptoms: migrainous features are common
• Recognized triggering event in less than 50%

Delayed onsent in sleep and not being able to fall asleep for alteast 30 minutes to several hours is common in NDPH patients. [11]

Infection, surgical procedures, and stressful life events are known triggers for the onsent of NDPH. Other known triggers include minor head trauma and high-altitude climbing. In the case of infections triggering NDPH, it is postulated that the nonspecific inflammatory response initiated by an infection, rather than the infections agent itself, may be the trigger. [5, 6, 11, 12]

How is NDPH diagnosed?

It is necessary to rule out other conditions of new onset headache before arriving at a diagnosis of NDPH. The evaluation of an NDPH patient should include a brain MRI with and without gadolinium, MRA (Magnetic Resonance Angiography), and MRV (Magnetic Resonance Venography) to rule out spontaneous cerebrospinal fluid (CSF) leak, and cerebral venous sinus thrombosis. If these imaging studies are negative a lumbar puncture (spinal tap) should be considered to help rule out infection and determine the CSF pressure to help rule out other conditions such as pseudotumor cerebri (idiopathic intracranial hypertension), which can also mimic NDPH. However, a lumbar puncture can lead to a postlumbar puncture headache. [3, 11]

Rozen 2011 [3] and Baron and Rothner 2010 [12] lists secondary mimics of new daily persistent headache which needs to be explored and ruled out before a diagnosis of NDPH is made. From personal research on the NDPH Support Group at MDJunction, it is possible for lyme disease and co-infections such as Borrelia, Bartonella, Babesia, and Erlichia to mimic NDPH. One such patient had a 24/7 headache start on July 14, 2008. She began treatment for lyme disease in March 2009 and her headache resolved in June 2009. [14, 15] Patients with a new onset of headache should hence be tested for Lyme disease and also be tested for Epstein-Barr virus and West Nile virus. [11]

The secondary mimics of NDPH include: [3, 11, 12, 14, 15]

• Low cerebrospinal fluid pressure
• High cerebrospinal fluid pressure
• Cerebral vein thrombosis
• Carotid or vertebral artery dissection
• Giant cell arteritis
• Meningitis
• Sphenoid sinusitis
• Cervical facet syndrome
• Intranasal contact: pain caused by contact of intranasal structures (e.g., nasal septum and nasal turbinate)
• Intracranial neoplasm or mass lesion
• Temporomandibular joint disease (TMJ)
• Congenital abnormalities (e.g., Chiari malformation)
• Metabolic disorders
• Endocrine disorders (e.g., Hashimoto's thyroiditis)
• Hormonal changes
• Toxins
• Infection (e.g., viral, Lyme disease, Epstein-Barr virus, West Nile virus)
• Posttraumatic headache

Routine blood work including thyroid studies, sedimentation rates, complete metabolic profile, and complete blood count should be checked. Electroencephalography (EEG) can certainly be considered as can electrocardiography (ECG). Other tests to consider include a 24 hour urine collection for catecholaimines, 24 hour blood pressure monitoring, a chest x-ray, and an MRI of the cervical spine. [12]

My Experience

For me, (this website's author) my headache started out extremely severve two days after my wisdom teeth removal. The pain was in the same spot, it felt like it was deep in the head, slightly to the right, and slightly in the back. The pain gradually improved the following couple of months, but remained and then I didn't notice any improvement.

I ended up seeing three different neurologists in the area. I started off with neurontin and was on it for about 2 months, it made me feel dizzy though after I worked out. Then I took vivactil for a few days, but had to stop because it gave me severe heartburn. I was on nortriptyline for 3 months and noticed quite an improvement. I did not have much of a headache during the day, but instead had a lot of head pain, and still, like always, noticed a constant throbbing whenver I lied on my bed or on the couch. I eventually got off of nortriptyline because it gave me more severe pain when I increased the dosage without any benefits. I have been on verapamil, but had a rash as a side effect. I also have taken occasionaly some ibproufin or darvocet, which helps, but only temporarily. I have also tried indocin and many herbs such as nettle leaf.

An excellent doctor for NDPH is Dr. Todd Rozen.

What is the treatment for NDPH?

NDPH can be very disabling because preventive and abortive medications often do not provide any pain relief. Some patients have had successful treatment with Neurontin (gabapentin) and Topamax (topiramate). NDPH patients often suffer from medication overuse (rebound) headaches as they often overuse medications to attempt to find pain relief. Currently no successful treatment plan has been specifically suggested for NDPH sufferers and most headache specialists will use the same medications as prescribed for chronic Migraine. These medications include the tricylic antidepressants (amitriptyline, nortriptyline), antieiletpics (topiramate, gabapentin, valproic acid), calcium channel blockers (flunarizine), and beta blockers (atenolol, propranolol). [3, 6, 11, 12, 13]

Some NDPH patients will see an an anesthesiologist/pain specialist and receive nerve blocks and facet blocks. Dr. Rozen has had several NDPH patients acheive pain relief from nerve blocks including greater occipital, auriculotemporal, and supraorbital/trochlear. More information on nerve blocks including my own negative experience with an occipital nerve block can be found on this website. I warn those to proceed with caution who suffer from NDPH before having a nerve block done as it could, as in my case, lead to even more pain and lasting nerve damage. [3] Botulinum toxin also can be used but it carries my same warning message as above.

Doxycycline, a tetracycline antibiotic and TNF-alpha inhibitor, has been successful in a few patients with NDPH who had previously failed at least five preventatives, infusion therapy, and inpatient treatments. [12]

Behavioral techniques and more alternative and natural therapies sometimes complement other forms of treatment. These forms of treatment include counseling, cognitive behavioral therapy, biofeedback, following a daily routine, avoidance of certain foods, supplements, acupuncture, yoga, physical therapy, massage, chiropractic care, craniosacral therapy, myofascial release, regular exercise, a proper diet, and regular sleep patterns. Three mg of melatonin taken 2 hours before bed can be used to help aid in sleep with more dosage needed up to 9 mg if difficulty falling asleep remains. Other supplements that could prove beneficial include coenzyme Q10, magnesium, calcium, butterbur, riboflavin, and fish oil. [12]

Additional information on my own treatment can be found along with additional information on headaches in more of a general sense on this website.

NDPH Support

If you have NDPH, I highly recommend visiting MDJunction and joining their New Daily Persistent Headache Support Group at http://www.mdjunction.com/ndph. The site has valuable information from people's own experiences with NDPH. The doctors people have seen, medications people have taken, and tests people have had in an attempt to become headache free are just some of the wisdom offered in the group.

A blog called Living with NDPH also provides you with a means to discuss and share with others who have NDPH http://lifewithndph.com/. Other people's NDPH stories are shared and discussed.

Refer to the references below for some additional information on NDPH.

References
1. The International Headache Society. The International Classification of Headache Disorders, 2nd edition. Written 2004.
2. WJ Vanast. New daily persistent headaches: definition of a benign syndrome. Headache 1986. 26. pages 317–320.
3. Todd D. Rozen. Chapter 44: New daily persistent headache. Handbook of Clinical Neurology. vol. 97 (3rd series) Headache. 2011. Elsevier B.V.
4. Juan R. Santoni and Carlos J. Santoni-Williams. Headache and Painful Lymphadenopathy in Extracranial or Systemic Infection: Etiology of New Daily Persistant Headaches. Internal Medicine. vol. 32. no. 7. July 1993.
5. D Li and TD Rozen. The clinical characteristics of new daily persistent headache. Cephalalgia. 2002. 22. pages 66-69.
6. Kenneth J. Mack. M.D. PhD. What Incites New Daily Persistent Headache in Children? Pediatric Neurology. 2004. vol. 31. no. 2. pages 122-125.
7. P. Meineri, E. Torre, E. Rota, and E. Grasso. New daily persistent headache: clinical and serological characteristics in a retrospective study. Neurol Sci. 2004. 25. (Suppl. 3). S281-S282.
8. Y Takase, M Nakano, C Tatsumi and T Matsuyama. Clinical features, effectiveness of drug-based treatment, and prognosis of new daily persistent headache (NDPH): 30 cases in Japan. Cephalalgia. 2004. 24. pages 955-959.
9. TD Rozen, JM Roth, and N Denenberg. Cervical spine joint hypermobility: a possible prediposing factor for new daily persistent headache. Cephalalgia. 2006. 26. pages 1182-1185.
10. Todd Rozen. and Sahar Z. Swidan. Elevation of CSF Tumor Necrosis Factor alpha Levels in New Daily Persistent Headache and Treatment Refractory Chronic Migraine. Headache. 2007. 47. pages 1050-1055.
11. Kenneth J. Mack. M.D. PhD. New Daily Persistent Headache in Children and Adults. Current Pain and Headache Reports. 2009. 13. pages 47-51.
12. Eric P. Baron and A. David Rothner. New Daily Persistent Headache in Children and Adolescents. Curr Neurol Neurosci Rep. 2010. 10. pages 127-132.
13. Teri Robert. New Daily Persistent Headache: The Basics. Accessed July, 2008. http://www.healthcentral.com/migraine/types-of-headaches-41643-5_3.html
14. Jensen16. http://www.mdjunction.com/forums/ndph-support-forums/general-support/2173122-ndph-can-be-symptom-of-lyme-disease. Accessed December 9, 2010.
15. Ramilin. http://www.mdjunction.com/mem/51289 Accessed December 9, 2010.