Clinical features, effectiveness of drug-based treatment, and prognosis of new daily persistent headache (NDPH): 30 cases in Japan

Although new daily persistent headache (NDPH) is considered to be one of the most refractory headaches to pharmacological treatment, the effectiveness of drug-based treatment, and the prognosis of NDPH have not been well studied. The purpose of this study is to evaluate the effectiveness of pharmacological treatment of NDPH. Seventeen men and 13 women who met the IHS diagnostic criteria for NDPH, were treated for five years from November 1997 to October 2002, and whose headache conditions were entirely available for reference as of October 2003 were investigated in Toyonaka Municipal Hospital. Mean age at onset 35.0 years (range 13-73 years). The onset of headache occurred in relation to a stressful life event in six (20%) patients and any precipitating events could not be identified in 24 (80%) patients. For treatment, muscle relaxants were first administered and if no effect was observed, tricyclic antidepressants, selective serotonin reuptake inhibitors, and antiepleptic drugs were subsequently administered. Results of the drug-based treatments in the 30 cases were 'very effective' for eight (27%) cases, 'moderately effective' for one (3%) case, 'mildly effective' for six (20%) cases, and 'not effective' for 15 (50%) cases. According to the survey on the phone for the patients who did not improve to the level of 'mildly effective' or better and did not come back to our institution as of October 2003, there were no cases found to have spontaneously improved to the level of 'mildly effective' or better. These results suggest that NDPH is highly recalcitrant to the treatments with poor prognosis and better treatments are needed.     

New Daily Persistent Headache: Historical Review and an Interview with Dr. Walter Vanast

New daily persistent headache (NDPH) is an idiopathic headache syndrome characterized by the abrupt onset of an unremitting, daily, continuous headache without an antecedent escalating headache pattern, and not attributable to other primary or secondary headache disorders. We review the history of NDPH in terms of its characterization and classification, and then interview Dr. Walter Vanast, the neurologist who initially described NDPH three decades ago, to gain his perspective now that there is more widespread recognition and interest in this syndrome.     

Juvenile myoclonic epilepsy presenting as a new daily persistent-like headache

New daily persistent headache (NDPH) is a recognized subtype of chronic daily headache with a unique presentation of a daily headache from onset typically in individuals with minimal or no prior headache history. Various secondary mimics of NDPH have now been documented but at present there has been no association made between primary epilepsy syndromes and new daily persistent-like headaches. A case patient is presented who developed a daily continuous headache from onset who 3 months after headache initiation had her first generalized tonic-clonic seizure. Further investigation into her history and her specific EEG pattern suggested a diagnosis of juvenile myoclonic epilepsy (JME). Her NDPH and seizures ceased with epilepsy treatment. Clinically relevant was that the headache was the primary persistent clinical symptom of her JME before the onset of generalized tonic-clonic seizures. The current case report adds another possible secondary cause of new daily persistent-like headaches to the medical literature and suggests another association between primary epilepsy syndromes and distinct headache syndromes.     

Post-infectious new daily persistent headache may respond to intravenous methylprednisolone

New daily persistent headache (NDPH) is a subtype of chronic daily headache (CDH) that starts acutely and continues as a daily headache from the onset. It is considered as one of the most treatment refractory of all headache syndromes. The pathophysiology is largely unknown. Viral infections, extracranial surgery, and stressful life events are considered as triggers for the onset of NDPH. A few patients may have the onset of their symptoms during an infection. Here we report nine patients with NDPH like headache. All of them had a history suggestive of extracranial infections a few weeks prior to the onset of headache. All patients received intravenous methyl prednisolone (IV MPS) for 5 days. Intravenous MPS was followed by Oral steroids for 2–3 weeks in six patients. The relief of headache started between the second and fifth days of infusion in all patients. The steady improvement in headache continued and seven patients experienced almost complete improvement within 2 weeks. Two other patients showed complete improvement between 6 and 8 weeks after initiation of IV MPS therapy. We conclude that NDPH-like headache may occur as a post infectious process following a recent infection. We also speculate on the possible mechanisms of headache in our patients.     

Prevalence of new daily persistent headache in the general population. The Akershus study of chronic headache

The aim of the present study was to investigate the prevalence of new daily persistent headache (NDPH) in the general population, and compare the clinical characteristics of NDPH and chronic tension-type headache (CTTH). This is a population-based cross-sectional study. A random sample of 30 000 persons aged 30–44 years was drawn from the population of Akershus County, Norway. A postal questionnaire was screened for chronic headache. Those ( n  = 633) with self-reported chronic headache within the last month and/or year were invited to an interview and examination by a neurological resident. A follow-up interview was conducted after 1.5–3 years. The headaches were diagnosed according to the International Classification of Headache Disorders, 2nd edn and relevant revisions. The response rate of the questionnaire was 71% and the participation rate of the interview was 74%. Four persons, three men and one woman, had NDPH. The overall 1-year prevalence of NDPH was 0.03%. The clinical characteristics of NDPH and CTTH were similar, except for the sudden onset in NDPH. Three of the four persons with NDPH had medication overuse. Follow-up disclosed that the symptomatology of NDPH is not unchangeable, since two persons had improvement of their NDPH. NDPH is rare and occurs in one of 3500 persons from the general population of 30–44-year-olds. It is often associated with medication overuse.     

The classification of chronic daily headache in adolescents--a comparison between the second edition of the international classification of headache disorders and alternative diagnostic criteria

OBJECTIVES: To compare the second edition of the International Classification of Headache Disorders (ICHD-2) and the Silberstein-Lipton (S-L) criteria in the classification of adolescents with chronic daily headache (CDH). METHODS: We reviewed the clinical records and the headache diaries of 170 adolescents (13 to 17 years) seen between 1998 and 2003 at a headache center. Relevant information was transferred to a standardized form that included operational criteria for the ICHD-2. CDH subtypes were classified according the criteria proposed by S-L into transformed migraine (TM) with (TM+) and without medication overuse (TM-), chronic tension-type headache (CTTH), new daily persistent headache (NDPH), and hemicrania continua (HC). RESULTS: From the 69 patients with TM- according the S-L criteria, most (71%) could be classified as chronic migraine (CM), while a minority of patients required a combination of diagnosis, mainly migraine and CTTH (14.4%). Of the patients with TM+, just 39.6% met the criteria for probable CM (PCM) with probable medication overuse (PMO). If instead of 15 migraine days per month, we considered 15 or more days of migraine or probable migraine, 84% of the subjects with TM- and 68.7% of those with TM+ could be classified. Of the 27 subjects classified as NDPH without medication overuse according to the S-L system, the majority (51.2%) were also classified as NDPH according the ICHD-2. Interestingly, three (11.1% of the subjects with NDPH without medication overuse) were classified as CM in the ICHD-2 because these patients had an abrupt onset of 15 or more days of migraine per month. All patients with NDPH with medication overuse according to the S-L criteria required a combination of diagnoses in the ICHD-2. All subjects with CTTH received a single diagnosis in both classification systems. CONCLUSIONS: (i) Among adolescents with TM, the majority (58.1%) could be classified as CM, according to the ICHD-2. These results were driven by TM without medication overuse. (ii) If the ICHD-2 criteria for CM are revised to require 15 days of migraine or probable migraine, the proportion of patients with TM- who meet the criteria for CM increases from 71% to 84%; for TM+, the proportion with probable chronic migraine and PMO increases from 30% to 68%. (iii) About half of the patients with NDPH according to the S-L criteria have too many migraine features to meet ICHD-2 criteria for NDPH.     

Chronic daily headache in children and adolescents: a clinic based study from India

Chronic Daily Headache (CDH) is uncommon in Indian children compared to their adult counterpart. This is a retrospective study looking at the headache phenomenology of CDH in Indian children and adolescents. The validity of the case definitions of subtypes of chronic primary headaches mentioned in the IHS 2004 classification have been evaluated. 22 children (age range 8-15 years; M : F-16 : 6) diagnosed as having primary CDH using a modified definition seen between 2002 and 2003 have been studied. CDH has been defined as daily or near daily headaches > 15d/month for > 6 weeks. The rationale for this modified definition has been discussed. Majority of children (15/22) had a more or loss specified time of onset of regular headache spells resembling New Daily Persistent Headache (NDPH) but did not fulfil totally the diagnostic criteria of NDPH as laid down by IHS 2004. In all cases headache phenomenology included a significant vascular component. Headache phenomenology closely resembled Chronic Tension Type Headache (CTTH) in 4 patients and Chronic Migraine in 3 patients. However, in no patient in these groups, a history of evolution from the episodic forms of the diseases could be elicited. Heightened level of anxiety mostly related to academic stress and achievement was noted in the majority (19/22). Only a minority of patients (3/22) had anxiety and depression related to interpersonal relationships in the family. Medication overuse was not implicated in any patient. CDH in children in India is very much different from CDH in adults with the vast majority of patients exhibiting overlapping features of migraine and tension-type headache. There is need for a modified diagnostic criteria and terminology for chronic primary headaches in children.     

New Daily Persistent Headache Caused by a Multinodular Goiter and Headaches Associated With Thyroid Disease

A 33‐year‐old female is presented with the first case to our knowledge of new daily persistent headache (NDPH) with a large right benign non‐toxic multinodular goiter causing carotid and vertebral compression with complete resolution of the headache immediately after thyroidectomy. Although this may be quite rare, hypothyroidism or hyperthyroidism causing NDPH, migraine, or an exacerbation of pre‐existing migraine is not. Clinicians should consider routinely obtaining serum thyroid‐stimulating hormone (TSH) and free T4 in patients with new onset frequent headaches or an exacerbation of prior primary headaches.     

Development of chronic daily headache: a clinical study

We studied the development of chronic daily headache in 258 headache practice patients, 50 men and 208 women. Chronic daily headache was defined as headaches occurring at least 5 days per week for at least 1 year. Twenty-two percent of the patients had daily headaches from the onset, and 78% initially experienced intermittent headaches. Of the patients with initially intermittent headaches, 19% experienced an abrupt transition into daily headaches and 81% a gradual one. The distribution of the age of daily headache onset was the same in the patients with daily headaches from the onset and in those with initially intermittent headaches but with abrupt transition into daily headaches. The distribution of the circumstances of daily headache onset was also the same in the groups. The most common circumstance of abrupt onset of daily headaches was head, neck, or back injury, in 61% caused by a motor vehicle accident. In the patients with initially intermittent headaches but with gradual transition into daily headaches, the transition took an average of 10.7 years.     

Cervical spine joint hypermobility: a possible predisposing factor for new daily persistent headache

The objective of this study was to suggest that joint hypermobility (specifically of the cervical spine) is a predisposing factor for the development of new daily persistent headache (NDPH). Twelve individuals (10 female, 2 male) with primary NDPH were evaluated by one of two physical therapists. Each patient was tested for active cervical range of motion and for the presence of excessive intersegmental vertebral motion in the cervical spine. All patients were screened utilizing the Beighton score, which determines degree of systemic hypermobility. Eleven of the 12 NDPH patients were found to have cervical spine joint hypermobility. Ten of the 12 NDPH patients had evidence of widespread joint hypermobility with the Beighton score. Based on our findings we suggest that joint hypermobility, specifically of the cervical spine, may be a predisposing factor for the development of NDPH.     

Chronic daily headache: correlation between the 2004 and the 1988 International Headache Society diagnostic criteria

BACKGROUND: In a previous study, we compared the 1988 International Headache Society (IHS) criteria and the Silberstein-Lipton criteria (S-L) in a subspeciality clinic sample of 638 patients with chronic daily headache (CDH) assessed both clinically and with headache diaries. Both systems allowed for the classification of most patients with CDH. The 1988 IHS classification required multiple diagnoses and was more complex to apply. OBJECTIVES: The aim of this study was to revisit the same database, now comparing the prior classification systems with the new 2004 IHS classification. In contrast with the 1st edition, the 2nd edition includes criteria for chronic migraine (CM), new daily persistent headache (NDPH), and hemicrania continua (HC). METHODS: We reviewed the clinical records and the headache diaries of 638 patients seen between 1980 and 2001 at a headache center. All patients had primary CDH according to the S-L criteria. RESULTS: Using the S-L criteria as a reference, of the 158 patients with transformed migraine (TM) without medication overuse, just 9 (5.6%) met 2004 IHS criteria for CM. Most of the subjects were classified using combinations of migraine and CTTH diagnoses, much like the 1988 IHS classification. Similarly, using the new IHS system, just 41/399 (10.2%) subjects with TM with medication overuse were classified as probable CM with probable medication overuse. Most patients with NDPH without overuse were easily classified using the 2004 criteria (95.8%). Regarding NDPH with medication overuse, the diagnostic groups were much like results for the 1st edition. All patients with chronic tension-type headache (CTTH) and hemicrania continua (HC) according to the S-L system were easily classified using the 2004 IHS criteria. CONCLUSIONS: We conclude that the 2004 IHS criteria facilitate the classification of NDPH without medication overuse and HC. For subjects with TM according to the S-L system, the new IHS criteria are complex to use and require multiple diagnoses. Very few patients with TM in the S-L system could be classified with a single diagnosis in the 2004 IHS classification. In fact, CM was so rare that it would be virtually impossible to conduct clinical trials of this entity using the 2004 IHS criteria. Clinical trials of this entity should therefore be conducted using the S-L criteria. Finally, we propose that in the 3rd edition of the IHS classification, the diagnosis of NDPH be revised so as not to exclude migraine features.     

Presentation of Chronic Daily Headache: A Clinical Study

We studied the presentation of chronic daily headache in 258 patients from a private headache practice, 50 men and 208 women. Chronic daily headache was defined as headaches, occurring at least 5 days per week for at least 1 year.
Seventy-seven percent of the patients experienced the onset of headache before the age of 30. The daily headaches were present on awakening in the morning or came about in the course of the morning in 79% of the patients. In 53%, they were worst in the afternoon or evening. The headaches awoke the patients at night at least once per week in 36%. At least twice per week, they were associated with nausea in 35% of the patients and with vomiting in 9%. Common aggravating factors included light, physical activity, bending over, noise, stress or tension, and menstruation. Ninety-four percent of the patients experienced severe headaches in addition to the daily headaches. In 63%, the severe headaches occurred 10 days per month or less. The daily caffeine intake of the patients averaged 170 mg, and the daily analgesic intake, 1860 mg of aspirin equivalents.     

Elevation of CSF tumor necrosis factor alpha levels in new daily persistent headache and treatment refractory chronic migraine

OBJECTIVE: To determine if patients with new daily persistent headache (NDPH) have elevated levels of tumor necrosis factor alpha (TNF alpha) in the CSF. BACKGROUND: NDPH is considered one of the most treatment resistant of all headache syndromes. This reflects a lack of understanding of its pathogenesis. As a certain percentage of NDPH patients have their headaches start after an infection, the possibility of a persistent state of systemic or CNS inflammation comes into question. TNF alpha is a proinflammatory cytokine involved in brain immune and inflammatory activities, as well as in pain initiation. The goal of this study was to look at TNF alpha levels in the CSF of NDPH patients, to determine if CNS inflammation may play some role in the pathogenesis of this condition. METHODS: CSF TNF alpha levels were studied in 38 patients: 20 with NDPH and a control population of 16 patients with chronic migraine (CM), and 2 with post-traumatic headache (PT). RESULTS: CSF TNF alpha levels were elevated in 19 of 20 NDPH patients, 16 of 16 CM patients, and both PT patients. Serum TNF alpha levels were normal in most of the study subjects. CONCLUSION: An elevation of CSF TNF alpha levels was found in almost all NDPH patients and suggest a role for TNF alpha in the pathogenesis of this condition. Surprisingly, all CM and PT patients tested had elevated CSF TNF alpha levels. In most patients with elevated CSF levels, serum TNF alpha levels were normal. All of these syndromes may be manifestations of CNS inflammation. As most of the positive-tested patients showed minimal to no improvement during aggressive inpatient treatment, persistent elevation of CSF TNF alpha levels may be one of the causes of treatment refractory CDH.     

New daily persistent headache in the paediatric population

We conducted a clinic-based study focusing on the clinical features of new-onset chronic daily headaches (CDH) in children and adolescents. The clinical records and headache diaries of 306 children and adolescents were reviewed, to identify 187 with CDH. Relevant information was transferred to a standardized form that included operational criteria for the diagnoses of the headaches. Since we were interested in describing the clinical features of these headaches, we followed the criteria A and B of the 2nd edn of the International Classification of Headache Disorders (ICHD-2) and refer to them as new daily persistent headaches (NDPH) regardless of the presence of migraine features (therefore, this is a modified version of the ICHD-2 criteria). From the 56 adolescents with NDPH, most (91.8%) did not overuse medications. Nearly half (48.1%) reported they could recall the month when their headaches started. NDPH was more common than chronic tension-type headache in both adolescents overusing and not overusing medication. Individuals with NDPH had headaches fulfilling criteria for migraine on an average of 18.5 days per month. On most days, they had migraine-associated symptoms (one of nausea, photophobia or phonophobia)). NDPH is common in children and adolescents with CDH. Most subjects do not overuse medication. Migraine features are common.     

Chronic daily headache: a time perspective

OBJECTIVE: To determine the development and outcome of chronic daily headache in 258 headache practice patients, consisting of 50 men and 208 women. Chronic daily headache was defined as headaches occurring on at least 5 days per week for at least 1 year. METHODS: Two hundred fifty-eight patients with headache were interviewed and evaluated. Ninety-one patients were contacted by telephone for follow-up. RESULTS: Twenty-two percent of the patients had daily headaches from the onset, and 78% initially experienced intermittent headaches. Of the patients with initially intermittent headaches, 19% experienced an abrupt transition into daily headaches and 81%, a gradual one. In the patients with gradual transition, the transition of the initial, intermittent headaches into daily headaches took an average of 10.7 years. The initial headaches were mild in 33% of the patients and severe in 67%. The severe headaches were associated with nausea and vomiting significantly more often than the mild ones. However, the daily headaches that these patients ultimately developed were the same, regardless of whether the initial headaches were mild or severe. The patients who gradually developed daily headaches from initially intermittent headaches were contacted to determine the outcome of their headaches. Of these patients, 33% continued to have daily headaches and 67% again experienced intermittent headaches. Of the latter group, 88% of the patients who now had migraine also had migraine initially.     

Focus on therapy: hemicrania continua and new daily persistent headache

Hemicrania continua (HC) and new daily-persistent headache (NDPH) represent the only two forms of chronic daily headache in Chap. IV “Other Primary Headaches” of the second edition of the International Classification of Headache Disorders. HC and NDPH are rare and poorly defined from a pathophysiological point of view; as a consequence, their management is largely empirical. Indeed, there is a lack of prospective, controlled trials in this field, and treatment effectiveness is basically inferred from the results of sparse open-label trials, retrospective case series, clinical experience and expert opinions. In this narrative review we have summarised the information collected from an extensive analysis of the literature on the treatment of HC and NDPH in order to provide the best available and up-to-date evidence for the management of these two rare forms of primary headache. Indomethacin is the mainstay of HC management. The reported effective dose of indomethacin ranges from 50 to 300 mg/day. Gabapentin 600–3,600 mg tid, topiramate 100 mg bid, and celecoxib 200–400 mg represent the most interesting alternative choices in the patients who do not tolerate indomethacin or who have contraindications to its use. NDPH is very difficult to treat and it responds poorly only to first-line options used for migraine or tension-type headache.     

Refining clinical features and therapeutic options of new daily persistent headache: a retrospective study of 63 patients in India

The aim of this retrospective study was to provide data on the clinical features and treatment outcomes of patients with NDPH (fulfilling Kung et al.'s criteria). A total of 63 patients were observed during a 5-yr period (2007-2012). More than one-third (35 %) patients had migrainous features; 65 % patients fulfilled the ICHD-II criteria. Both groups were similar in most clinical and epidemiological features. However, migrainous features were more common in patients with a prior history of episodic migraine (though statistically not significant). After a median follow-up of 9 months, 37 % patients showed "excellent" response (no or less than 1 headache per month). Another 30 % patients had "good" response (>50 % reduction in headache frequency or days per month). Excellent response was more in patients with a history of less than 6 months duration (statistically not significant). Patients with a recognized trigger showed better prognosis. Response was better in patients who received intravenous therapy of methyl prednisolone and sodium valproate. We suggest prospective and controlled studies to confirm our observations.     

Repetitive intravenous prochlorperazine treatment of patients with refractory chronic daily headache

OBJECTIVES: To investigate the efficacy and long-term outcome of intravenous prochlorperazine for the treatment of refractory chronic daily headache. BACKGROUND: Unlike dihydroergotamine, the treatment results of intravenous neuroleptics as first-line agents for refractory chronic daily headache have rarely been reported. METHODS: We retrospectively analyzed the data of inpatients with refractory chronic daily headache who received intravenous repetitive prochlorperazine treatment from November 1996 to March 1999. A semistructured telephone follow-up interview was done in September 1999. RESULTS: A total of 135 patients (44 men, 91 women) were recruited, including 95 (70%) with analgesic overuse. After intravenous prochlorperazine treatment, 121 (90%) achieved a 50% or greater reduction of headache intensity, including 85 (63%) who became headache-free. The mean hospital stay was 6.2 +/- 2.7 days, and mean total prochlorperazine used was 98 +/- 48 mg. Acute extrapyramidal symptoms occurred in 21 patients (16%). One hundred twenty-four patients (92%) were successfully followed up, with a mean duration of 14.3 +/- 7.5 months. Compared with pretreatment status, 93 patients (75%) considered their headache intensity decreased, and 86 patients (69%) considered their headache frequency decreased, although 40 (32%) still had a daily headache. Of the 87 patients with analgesic overuse who could be followed, 61 (70%) no longer overused analgesics. Poor response to prochlorperazine treatment (relative risk, 1.8) and presence of major depression (relative risk, 1.8) were predictors of persistent chronic daily headache at follow-up. CONCLUSIONS: Prochlorperazine was effective and safe in the treatment of patients with refractory chronic daily headache with or without analgesic overuse. Compared with dihydroergotamine, prochlorperazine seemed less effective at achieving "freedom from headache" during hospitalization, but had a similar outcome at follow-up.     

New Daily–Persistent Headache Versus Tension-type Headache

New daily–persistent headache (NDPH) and chronic tension-type headache (CTTH) are two forms of primary chronic daily headache of long duration that often are similar in their headache manifestations. NDPH distinguishes itself from CTTH and the other forms of chronic daily headache by its continuous head pain from onset. However, despite formalized criteria that specify NDPH must resemble the acute onset of a headache identical to that of CTTH, NDPH commonly has migraine features. Here, we review the available literature on NDPH and compare its clinical features, epidemiology, prognosis, inciting factors, and treatment to CTTH.     

Chronic Daily Headache: Long-Term Prognosis Following Inpatient Treatment With Repetitive IV DHE

We retrospectively studied the long-term (2-year) outcome of 50 consecutive patients admitted to our inpatient headache program because of chronic daily headache (CDH) associated with the overuse of analgesics, ergotamine, or both. They had been detoxified, given repetitive intravenous dihydroergotamine (IV DHE) and prophylactic medications as part of the program, and had become headache-free on this regimen. At the time of admission, 37 of the 50 patients had transformed migraine (TM), 12 had new daily persistent headache (NDPH), and 1 had chronic tension-type headache; 29 of the patients with TM, 7 of those with NDPH, and the single patient with chronic tension-type headache had coexistent migraine. Substances abused, alone or in combination, included: caffeine in 39 patients (av. 441 mg/d), acetaminophen in 32 (av. 2187 mg/d), aspirin in 24 (av. 1807 mg/d), ibuprofen in 9 (av. 1156 mg/d), narcotics in 7 (av. 10.1 mg morphine equivalents/d) and ergotamine in 11 (av. 2.3 mg/d). Twenty patients were using preventive medication at the time of admission. Follow-up evaluations were performed at 3, 6, 12, and 24 months after discharge. Forty-three patients were analyzed at 3 months. Of these, 44% had an excellent or good result and 28% a fair result; 3 were overusing analgesics. At 24 months, 39 patients were analyzed: 59% had a good or excellent result and 28% a fair result; 5 were overusing analgesics, 4 of whom were doing poorly.(ABSTRACT TRUNCATED AT 250 WORDS)