Dr. Wang of the Brain Research Center, National Yang-Ming University, and the Neurological Institute, Taipei Veterans General Hospital, received consulting fees from Eli Lilly, Daichi-Sankyo, and Novartis.)
This article includes discussion of chronic daily headache, evolutive migraine, mixed headache, mixed tension-vascular headache, new daily persistent headache, rebound headache, and transformed migraine. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
Chronic daily headache is a highly prevalent problem affecting 3% to 5% of the population as determined by studies in many parts of the world. The syndrome consists mainly of chronic migraine and chronic tension-type headaches. Chronic daily headache produces intermittent disability and a great deal of economic and psychosocial burden with loss of time from work, from family responsibilities, and from recreational activities. Chronic daily headache often leads to the overuse of symptomatic headache and pain medications and then to an additional dimension of medication overuse or rebound-withdrawal headache. Much progress has been made in the understanding and management of this condition. This review summarizes the background and this progress, primarily focusing on chronic migraine and chronic tension-type headache.
• Chronic daily headache is a complex chronic problem with long-term relapses and remissions.
• The disease may have long-term psychosocial effects that drastically alter relations to family and job.
• There is no “cure.” However, many patients can have a good response to appropriate management.
• Management of chronic daily headache requires ongoing contact between patient and physician, and the understanding of this condition as a potentially chronic disease with pain and psychosocial morbidity. There must be ongoing vigilance to the factors noted above. Subjects often relapse into periods of frequent headache and may become discouraged or depressed.
• There is often occurrence of medication overuse headache as a complication of treatment of chronic daily headache, or relapse of medication overuse headache due to the original medication overuse headache inducing agent, or due to another symptomatic medication that has become a new medication overuse headache inducing agent.
• All these factors require the patient and physician to have a good understanding of the disease and its course and to work together to achieve the best outcome.
Historical note and terminology
History. Chronic daily headache has been recognized since ancient times. In the first century, Aretaeus of Cappodocia provided a classification of headache that included heterocrania (migraine), cephalalgia (a headache that lasted days), and cephalea, a very chronic headache (Critchley 1967). In the 17th century, Thomas Willis, who conducted research on circulation and was also a practicing physician, described a patient with severe chronic headaches refractory to treatment. For refractory headaches he mentioned the use of poultices of millipedes and wood lice and noted that trepanation was a consideration (Silberstein et al 2002). Liveing, in his book published in 1873, was aware of the problem of frequent headache and mentions valerian (from valerian root) as a treatment (Liveing 1873). Gowers mentioned bromide and India hemp as treatments for migraine.
Another facet of this history is posttraumatic chronic daily headache. As speed of travel has increased, head injury and posttraumatic headache have become more common, and the incidence of head injury has increased greatly. In 1879, the German neurologist, Rigler, noted an increase in posttraumatic headache after injury-related compensation became available and was concerned about chronic headache as a compensation neurosis (Couch 1995). In the 1880s Strumpell and Friedman debated on whether head injury-related headaches were of organic or psychiatric origin (Couch 1995). This debate continued through the 20th century and is still reflected in the second edition of the revised International Classification of Headache Disorders, published in 2004 (Headache Classification Committee of the International Headache Society 2004). The works of Guttmann and of Brenner and colleagues highlight the debate (Guttmann 1943; Brenner et al 1944). Later studies by Cartlidge and Shaw and Rimel and colleagues provide more data on posttraumatic headache and posttraumatic chronic daily headache (Cartlidge and Shaw 1981; Rimel et al 1981).
A third component of this problem is that of headache related to medication overuse. The first reports on this problem were by Peters and Horton in 1950 and by Friedman and Von Storch in 1955 (Peters and Horton 1950; Friedman and Von Storch 1955). These authors noted that subjects taking ergotamine frequently might develop an intractable headache that was improved by discontinuing use of this agent. Subsequent work by Andersson and by Tfelt-Hansen and Krabbe in Europe and by Kudrow in America between 1960 and 1980, further clarified the relation of overuse of ergotamine to increasingly chronic and refractory headache (Andersson 1975; Tfelt-Hansen and Krabbe 1981; Kudrow 1982).
In 1982, Mathew published a seminal work on chronic headache and suggested that chronic headache might develop from intermittent migraine (Mathew et al 1982). He proposed the term “transformed migraine” in 1987 to account for this more chronic headache (Mathew et al 1987). Other work by Saper and Jones and by Manzoni and colleagues supported this concept (Saper and Jones 1986; Manzoni et al 1987).
Classification. The classification of headache remained a chaotic area until the introduction of the International Classification of Headache Disorders in 1988 (ICHD-1) (Headache Classification Committee of the International Headache Society 1988). This was a major step forward in the development of headache medicine for both clinical practice and research. The initial version of ICHD-1 was based on a format in which every type of headache present for a patient at a particular point in time was classified individually. This “time biopsy” approach has great strengths, especially for research applications, but was difficult to apply in the longitudinal situation of an evolving headache syndrome. Evolution of a headache problem was treated by re-evaluating and reclassifying the headache at a future time. The ICHD-1 did not recognize the term chronic daily headache, but recognized only chronic tension-type headache and did not acknowledge migraine as a possible precursor of chronic headache.
In 1996, Silberstein, Lipton, and Sliwinski published a proposed classification of chronic (primary) headaches defined as those of greater than 4 hours duration and occurring 15 days/month or more (Silberstein-Lipton criteria). The term “chronic daily headache” was used for this classification (Silberstein 1996). This system looked at the longitudinal evolution of the headache syndrome and included the following entities:
(1) Transformed migraine: chronic headache evolving from intermittent migraine.
(2) Chronic tension-type headache: chronic headache evolving from intermittent tension-type headache.
(3) New daily persistent headache: a primary headache that is chronic from onset.
These 4 entities were all modified by the presence or absence of medication overuse.
The term “chronic daily headache” continues to reflect the entities described in the Silberstein-Lipton (S-L) publication and overlaps with the ICHD-2 criteria in that both criteria include all 4 entities and medication overuse headache. However, the S-L criteria group together 4 of the most common primary headache conditions into an overarching entity that constitutes the most persistent and difficult problem facing the physician dealing with headache patients. Another attempt to deal with the classification of these patients has been the introduction of the term “refractory headache” to identify the most difficult of the chronic daily headache patients. The term “refractory headache” will be discussed later in this article.
The conflict between the ICHD-1 and the S-L criteria is illustrated as follows. A patient with headaches occurring at least 15 days per month, which evolved from intermittent migraine, and who had a combination of headaches that could be classified as migraine at least several days per month and as tension-type headache on other days would be classified by the ICHD-1 as chronic tension-type headache with additional migraine. Using the S-L criteria, this same patient would be classified as transformed migraine. For a patient with chronic daily headache with only rare migraine, the ICHD-1 and S-L classifications would classify this subject as chronic tension-type headache with occasional migraine.
The revised version of the International Classification of Headache Disorders, third edition (ICHD-3) was published in 2013 (Headache Classification Committee of the International Headache Society 2013) and recognized chronic migraine, chronic tension-type headache, new daily persistent headache, and hemicrania continua as chronic forms of primary headache. This classification now embraces chronic migraine that can be developed from episodic migraine. The ICHD-3 classification is hierarchical with chronic migraine taking precedence over chronic tension-type headache when elements of both may be present. Initially, the diagnosis of chronic migraine by ICHD-2 required that there were 15 or more days per month meeting criteria for migraine. This was later revised to requiring 15 or more headache days per month, of which at least 8 were migraine (Headache Classification Committee 2006). In parallel to the diagnosis of chronic migraine, the ICHD-2 suggests that chronic tension-type headache may evolve from intermittent tension-type headache. This is discussed in greater detail by Manack and colleagues (Manack et al 2009).
The concept of new daily persistent headache characterizes those with chronic daily headache whose headache begins over a short period of time or may evolve rapidly from episodic tension-type headache, becoming chronic from that time onward. The headache of new daily persistent headache may manifest mild nausea, photophobia, or phonophobia (Headache Classification Committee of the International Headache Society 2004). Newer studies on new daily persistent headache summarized by Robbins and Crystal demonstrated that new daily persistent headache may manifest features of migraine in 50% of subjects (Robbins and Crystal 2010).
Hemicrania continua is manifested by a strictly unilateral headache that is always on the same side and is of variable intensity (Headache Classification Committee of the International Headache Society 2004). The intensity is usually moderate, but may be severe at times. There is usually a sensation of conjunctival irritation similar to a foreign object, such as dust on the conjunctiva, and there may be conjunctival injection or lacrimation. Ptosis or miosis has also been reported. Hemicrania continua does not have any of the neurologic or gastrointestinal symptoms associated with migraine. A diagnostic feature is that hemicrania continua responds to indomethacin with virtually complete relief. Chronic migraine and chronic tension-type headache may show some response to indomethacin but not to the dramatic degree seen with hemicrania continua. Hemicrania continua is unique in belonging to the group of so-called “indomethacin-responsive headaches,” which includes chronic paroxysmal hemicrania. The basis of the response to indomethacin is unknown.
Although the term “chronic daily headache” is not recognized by the ICHD-2, it remains a very useful concept designating the spectrum of primary chronic headaches. Commentaries by Manzoni (Manzoni 1995; Manzoni 2011) and by Midgette and Scher (Midgette and Scher 2009) have stressed that the concept of chronic daily headache is still a very useful one in the consideration of chronic primary headache syndromes. The evolutionary aspect of the headache syndromes that change and evolve over time continues to be a valid concept. As discussed below, patients may cycle between periods of chronic daily headache and intermittent primary headache. The longitudinal aspect of the chronic daily headache concept as opposed to the “time biopsy” approach helps to understand the spectrum of chronic primary headache.
Another major problem in classification is the relation of migraine and tension-type headache. Although the migraine syndrome has clear defining characteristics, tension-type headache is often defined by being a primary headache of variable duration that does not fit the criteria for migraine or the trigeminal autonomic cephalgias. The work of Raskin and of Ziegler and colleagues suggested that there was a continuum between tension-type headache and migraine (Raskin 1980; Ziegler et al 1982). The Spectrum Study in 2000 found that for subjects with tension-type headache and migraine, the tension-type headache would respond well to sumatriptan if the migraine component did (Lipton et al 2000). For those subjects with pure tension-type headache and no migraine component, there was no response to sumatriptan. They suggested that for the group with sumatriptan-responsive tension-type headache, there was a “spectrum headache” disorder in which the subjects manifest headaches that fulfill the criteria for migraine and for tension-type headache, and the combined headache disorder represents a spectrum ranging from tension to migraine headache. This concept of a spectrum disorder fits a large percentage of chronic daily headache subjects.
Table 1. Classification of Migraine Without Aura, Chronic Migraine, and Chronic Tension-type Headache by ICHD-3
1.1 Migraine without aura
A. At least 5 attacks,1 fulfilling criteria B - D
B. Headache attacks lasting 4 to 72 hours (untreated or unsuccessfully treated) 2;3;4
C. Headache has at least 2 of the following characteristics:
(1) unilateral location
D. During headache, at least 1 of the following:
(1) nausea and/or vomiting
E. Not better accounted for by another ICHD-3 diagnosis
1.5.1 Chronic migraine
A. Headache (tension-type-like and/or migraine-like) on ≥15 days per month for >3 months and fulfilling criteria B and C
B. Occurring in a patient who has had at least 5 attacks fulfilling criteria B - D for 1.1 Migraine without aura and/or criteria B and C for 1.2 Migraine with aura
C. On ≥8 days per month for >3 months, fulfilling any of the following 3:
(1) criteria C and D for 1.1 Migraine without aura
D. Not better accounted for by another ICHD-3 diagnosis
2.3 Chronic tension-type headache
A. Headache occurring on ≥15 days per month on average for >3 months (≥180 days per year), fulfilling criteria B - D
B. Lasting hours to days, or unremitting
C. At least 2 of the following 4 characteristics:
(1) bilateral location
D. Both of the following:
(1) no more than 1 of photophobia, phonophobia, or mild nausea
E. Not better accounted for by another ICHD-3 diagnosis
Medication overuse headache. The problem of medication overuse has been a major issue complicating the understanding of chronic daily headache syndromes since efforts to define and classify headache in this area were initiated (Diener and Silberstein 2006; Couch and Lenaerts 2007a; Lenaerts and Couch 2007). Medication overuse headache has been termed a worldwide epidemic and is estimated to affect approximately 1% of the population (Diener and Limmroth 2004), or 15% to 20% of the chronic daily headache population. In a study from China involving 5041 subjects (50% female) interviewed in a door-to-door survey, the prevalence of chronic daily headache was 1% with a 3:1 female:male ratio (Yu et al 2012). In this population, 60% of chronic daily headache subjects of both sexes manifested medicine overuse headache. Similar results were seen in a population study of 7417 subjects in Germany (Straube et al 2010).
A major problem of classification that arises is whether medication overuse headache is a separate entity or a part of the chronic daily headache syndromes as suggested by the S-L classification. The ICHD-2 classifies medication overuse headache as an organic headache that is superimposed on the syndromes of chronic daily headache whereas the S-L criteria consider medication overuse headache to be a part of chronic daily headache. For chronic migraine, medication overuse headache is present in 40% to 80% of patients in various series (Rapoport 1988; Diener and Limmroth 2004; Straube et al 2010; Yu et al 2012).
Refractory migraine. The concept of “refractory migraine” has been proposed, and a special interest group was brought together within the American Headache Society to study this possible entity. The term is not recognized by the ICHD-2.
The concept of refractory migraine deals with the extreme group of chronic daily headache patients who are usually significantly disabled by their headache problem and have responded poorly to preventative and symptomatic antimigraine therapies and to non-pharmacological therapies. An interest group within the American Headache Society has dealt with this entity for a number of years and has proposed the following criteria for consideration.
Table 3. Proposed Criteria for Refractory Migraine
(1) Patient has diagnosis of chronic migraine
(2) Patient has failed adequate trial of at least 2 of 4 drug classes
(3) Patient has modified lifestyle and eliminated (migraine) triggers
(4) Patient has failed abortive medications, including:
(5) There may be modifiers
(a) With or without medication overuse
Adapted from (Schulman et al 2008)
Robbins published a Refractory Migraine Scale and used it to evaluate 127 refractory migraine subjects over 10 years (Robbins 2012). He found that the patients did not achieve good results by usual standards, but a decrease of 30% in pain and disability was achieved in a majority of subjects over 10 years. He found that use of long-acting opioids, frequent triptan use, and ongoing use of butalbital were modestly effective, but patients had to be monitored for dose escalation. Botulinum toxin was helpful in 16% of subjects.
Refractory migraine is still an area of controversy, and its management is even more controversial. As indicated, this group appears to encompass the most severely affected chronic daily headache subjects. Whether this should be a separate classification will be a subject of debate in the future.
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