New Daily Persistent Headache
New Daily Persistent Headache (NDPH) is one of the more unusual headache types, in that the diagnosis hinges on the sufferer having a clear recall of the onset of symptoms. Not just a ‘rough idea’, but a ‘distinct and clearly-remembered’ onset of headaches.
There are other forms of chronic daily headache such as chronic migraine, chronic tension-type headache, hemicrania continua and medication overuse headache. Usually the differences between different headache types is distinguished by the presence or absence of associated symptoms, for example nausea and sensitivity to light or sound are typically migrainous whereas the absence of these is typically tension-type.
The clinical features of NDPH can adopt migrainous features of nausea, photophobia and phonophobia or aggravated by routine physical activity. Often the only feature to distinguish NDPH from these other chronic daily headaches is the ability to recall the exact onset.
NDPH was initially described in 19861 as a benign disorder that was self limiting. While this does happen, one case series by Li and Rozen2 revealed all subjects had endured at least 6 months of symptoms, with many having symptoms for over 5 years. In another study, only 15% had complete resolution of symptoms with the average time to remission being 21 months.
Very little is known of why NDPH starts. Some studies have linked it to viral illness, others suggest it may be relted to chronic central nervous system inflammation. Three studies point to a possible role of the upper cervical spine in NDPH. One study3 found hypermobile upper cervical spine joints to be common amongst sufferers in their cohort, whilst another noted onset after endotracheal intubation, with the necessary hyperextension of the cervical spine.4 A third study found successful management after treatment of the upper cervical spine.5
In our experience NDPH either responds very quickly to the Watson Headache ® Approach (within a few sessions we expect to see s significant decrease), or doesn’t respond at all. We are not waiting weeks or months to know if we can help.
Diagnosis & Symptoms
A 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.
A. Persistent headache fulfilling criteria B and C
B. Distinct and clearly-remembered onset, with pain becoming continuous and unremitting within 24 hours
C.Present for > 3 months
D.Not better accounted for by another ICHD-3 diagnosis
NDPH typically occurs in individuals without a prior history of headache. Patients who have previously had Migraine or Tension-Type Headache can still get NDPH, but it should be distinct in onset, as opposed to an escalation over time of the previous headache.
If the exact onset cannot be recalled then the headache is classified according to the presence or absence of additional features, as either Chronic migraine or Chronic Tension-Type Headache.
As with other primary headaches the cause of pain being secondary to other conditions must be excluded, such as acute headache due to traumatic injury to the head, and headache attributed to increased or decreased cerebrospinal fluid pressure.6
Two significant population based studies have been undertaken, looking at the prevalence of NDPH in the general population. The first of these in Spain used a working clinical criteria prior to NDPH being adopted into the ICHD-II and found a 1-year prevalence of 0.1%.7
The current incarnation of the ICHD – version III is more open in terms of definition and subsequent studies are likely to find a higher incidence and prevalence.
Amongst patients with chronic daily headache, there seems to be a significant difference in the proportion of NDPH when looking at adults compared to paediatric populations. In children and adolescents suffering chronic daily headache NDPH is diagnosed 13-35% of the time,9 compared to 1.7-10.8% in adults.8
The vast majority of sufferers have no prior history of headache, with one study reporting 25% having pre-existing headache (18% TTH, 7% Migraine).10
Several studies have attempted to delineate sub-types in NDPH.
In one series researchers 11 examined 71 patients with NDPH and found:
- 76.1% with persistent headaches
- 15% with remission (time to remission ranged from 4 months to 54 years, mean 21 months)
- 8% with relapsing-remitting type (range to first remission 3-24 months)
As of 2022 there are no randomised controlled clinical drug trials for NDPH. Given the scarcity of funding in headache and migraine, a condition that only affects 0.03% to 0.1% of the population is going to struggle to attract a lot of interest.
NDPH is perhaps the most intractable and least therapeutically rewarding form of headache. In general we classify the dominant phenotype, migraine or tension-type headache, and treat with preventives according to that sub-classification, as for patients with chronic daily headache.
Peter Goadsby, Christpopher Boes12
That is if it looks a bit like chronic migraine (has nausea, photophobia and phonophobia) then treatment for chronic migraine will commence. If it lacks these features then treatment for chronic tension-type headache will be the starting point.
Up to now (2019) there is no specific well-defined strategy for its treatment in the absence of double-blind controlled studies. Even aggressive treatments are usually ineffective or only partially effective. NDPH patients are therefore prone to overuse medication
Yamani and Olesen13
The typically amitriptyline, topiramate, sandomigran, beta-blockers (propranolol). Where these fail then ketamine infusion, onabotulinum toxin type A (Botox), intravenous (IV) lidocaine, IV methylprednisolone and greater occipital nerve blocks may be used13
Given the lack of efficacy of pharmaceutical approaches at present we couldn’t recommend other approaches more strongly. This is the perfect type of headache to adopt the ‘SEEDS’ approach and check your neck.
SEEDS stands for:
S – Sleep: Make sure you are getting enough and at regular times. This is one of the most important factors along with the next to try and change
E – Eating: Never is there a better time to turn to fresh whole foods and a healthy diet than when you are confronted with daily persistent headache. There are 21 chemical risk factors that have been identified as potentially linked to migraine, and while they may not be responsible for ‘starting the fire’ they may provide an underlying level of irritation to the nervous system that may help perpetuate symptoms. These include: Refined sugar, alcohol, aspartate (artificial sweetener), caffeine, free glutamate (MSG), nitrates, salt, sulphites, capsaicin, casein, corn, garlic, gluten, glycoalkaloids, histamine, nitrates, omega 6, onion, phenylethylamine, salicylates, tannins, tyramine.
E – Exercise: Research supports use of exercise at a moderate intensity to help reduce intensity of headaches in chronic headache. Exercise at a level where you can just ‘walk and talk’ will give your body’s serotonin levels a boost – important for mood control and also dampening the response of pain transmission in the trigemino-cervical nucleus – the source of headache symptoms.
D – Dehydration: Possibly the easiest intervention that anyone can make is to make sure they are remaining adequately hydrated. There is a small amount of research14 supporting an increase of 1.5L per day in adults improving quality of life after 3 months.
S – Stress: Consistently reported as the most common trigger, clearly stress plays a role in starting headaches. Adopting mindfulness may have an impact on inflammation in the body15 , and people who meditation show improved cortical and stress processing than migraine patients and healthy controls16
The exact underlying pathophysiology of NDPH is unclear as very little research has been done to elucidate it.
It is likely that like migraine, NDPH represents the intersection of multiple stressors, which vary from person to person. Like a large fire burning out of control, the precipitating events often become irrelevant. It is amongst the common features that we often find solutions. The presence of headpain immediately implicates the trigeminal nucleus, and the association of neck pain with presentation must raise the possibility of a neck based generator for symptoms, and as mentioned in the introduction there are good reasons to start with the neck.
For others, some clues may be drawn from the precipitating or triggering events, but in 53% of cases that is not able to be recognised. For those that can recall a precipitating event, the most common were17 :
- 22% After an infection and flu-like illness
- 12% After surgical procedures involving intubation (breathing tube used when having general anaesthetic involving the neck being hyperextended)
- 9% Triggered by stressful life events.
If this were the case one might expect a much more significant impact than 9% with regards to onset for stressful life events, which seems substantially lower than migraine (stress cited as a ‘very frequent trigger’ 33% of the time).19
The most important thing to note here is that typically, NDPH occurs in people with no significant prior history of headache, and often for no obvious reason. It is imperative that every new presentation of headache seeks medical evaluation early in their presentation.
Severe intracranial pathologies such as sub-arachnoid haemorrhage, cervical artery dissection and intra-cranial space occupying lesions can all present as a constant headache early in their genesis. While they are rare it is essential to have them excluded as a possible cause of your symptoms.
This list is indicative rather than exhaustive, and the importance of medical investigation after the initial onset cannot be stressed enough, as though these other causes are rare the can be catastrophic if not found early.
Low CSF volume headache:
A history of lumbar puncture or epidural injection, Valsalva events (lifting, straining, coughing, clearing the eustachian tubes in an aeroplane, multiple orgasms) or can be spontaneous. These patients present with a very typical postural driver, in that they are generally better if not completely devoid of headache after laying down or reclining, but the headache rapidly returns on resuming an upright posture and can become rapidly or steadily worse. In contrast NDPH does not vary significantly from laying down to standing.
Raised CSF pressure headache:
Clinical findings typically involved with raised CSF pressure involve intracranial space occupying masses, encephalitis, hydrocephalus, idiopathic intracranial hypertension or haemorrhage. These patients present with a very typical postural driver which is the exact opposite of low pressure presentations. They are generally better if not completely devoid of headache after standing up or sitting upright, but the headache rapidly returns on reclining or laying down. In contrast NDPH does not vary significantly from laying down to standing.
A history of trauma to the head or neck in close temporal relationship to the onset of new headache symptoms would see daily persistent symptoms classified as post-traumatic headache.
Chronic Daily Headache (Chronic Tension-Type Headache, Chronic Migraine, Medication Overuse Headache):
As indicated earlier these types cannot be distinguished comfortably based on clinical presentation, as there is significant overlap. The key difference is in development to chronic daily symptoms. Medication overuse excludes itself as its diagnosis is contingent on a pre-existing headache that increases in frequency in response to a high frequency of key medications (read more about MOH-link). Similarly, chronic tension-type headache and migraine begin as episodic cases, and gradually become more frequent and persistent. NDPH begins without a pre-exisiting headache and rapidly (within 3-days) becomes daily.
The nerve supply to the meninges – the connective tissue ‘skin’ that covers the brain is supplied by the trigeminal nerve. Irritation of the meninges is thought to be the pathophysiological cause of migraine, though the source of irritation is yet to be found. Viral meningitis will cause inflammation and irritation leading to post meningitis or chronic meningitis related headaches.
11% of NDPH can be unilateral, so distinguishing it from HC can be challenging. HC typically responds to Indomethacin and can be a key way of distinguishing the two disorders.
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