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.     

Headache in juvenile myoclonic epilepsy

The objective of this study was to assess the prevalence of and risk factors for primary headaches in juvenile myoclonic epilepsy (JME). Headache was classified in 75 patients with JME using a questionnaire, and its prevalence was correlated with the literature on the general population and clinical data. Headache was present in 47 patients. Thirty-one had migraine [20 migraine without aura (MO), 11 migraine with aura (MA)]. Fourteen patients with migraine had tension-type headache (TTH) in addition. Sixteen had only TTH. Comparison with the general population revealed a significantly higher prevalence of migraine (RR 4.4), MO (3.6), MA (7.3) and TTH (3.4) in JME. Risk factors for migraine and MO were female gender and for MA family history of migraine in first-degree relatives. Migraine and MA were associated with fairly controlled generalized tonic clonic seizures, MO with absences. Together with its strong genetic background, JME appears to be an attractive homogenous subtype of epilepsy for genetic research on migraine.     

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.     

The clinical characteristics of new daily persistent headache

New daily persistent headache (NDPH) is a subtype of chronic daily headache. The literature on NDPH is scant and its true aetiology is unknown. A retrospective chart review was carried out from a computerized database at the Jefferson Headache Centre from August 1997 to May 2000 to identify patients with NDPH using the Silberstein et al. criteria. Forty women and 16 men were identified. Age of onset ranged from 12 to 78 years. The peak age of onset was the second and third decade in women and the fifth decade in men. Eighty-two per cent of patients were able to pinpoint the exact day their headache started. Onset occurred in relation to an infection or flu-like illness in 30%. A prior headache history was found in 38% of patients. A family history of headache was documented in 29%. The duration of daily headache ranged from 1.5 h to 24 h; 79% were continuous. Nausea occurred in 68% of patients, photophobia in 66%, phonophobia in 61%, and lightheadedness in 55%. Laboratory testing and neuroimaging in all patients was normal except for Epstein-Barr virus antibody titres, which were positive in 71% of seven patients tested, representing past infection. NDPH appears to be a female-predominant disorder, marked by a continuous daily headache with associated migrainous symptoms. Over 80% of patients could state the exact date their headache began. One-third of patients developed NDPH with a flu-like illness.     

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.     

The challenge of new daily persistent headache

New daily persistent headache is a rare chronic daily headache of long duration characterized by the abrupt onset of persistent headache that generally develops over less than 3 days and does not remit. While it was initially thought to be a benign, self-limiting disorder, further research has shown that a significant percentage of patients continue to suffer for many years, often experiencing pain that is refractory to treatment. This article reviews the symptoms, pathophysiology, diagnostic criteria, diagnostic testing, treatment, and prognosis.     

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.     

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.     

Chronic daily headache in a paediatric headache centre

The prevalence and the clinical features of chronic daily headache (CDH) were studied in 968 children and adolescents observed during a period of one year in the Headache Centre of the Anna Meyer Paediatric Hospital of Florence. Nine hundred and fortyfour patients (97.52%) had primary headache according to ICHD-II, 24 subjects had secondary headache and 56 patients had CDH (5.93% of primary headaches). The mean age of subjects with CDH was higher than general (13.5 vs. 11.5 years), with a female preponderance (69.6% vs. 30.4%). According to the ICHD-II, headaches were classified as chronic migraine in 10 patients (1.5.2 ICHD-II), chronic tension-type headache in 36 (2.3 ICHD-II), new daily persistent headache in 8 (4.8 ICHD-II) and 2 patients reported mixed pattern (chronic migraine+chronic tension type headache). Medication overuse was not implicated in our patients.     

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.     

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.     

Evidence that juvenile myoclonic epilepsy is a disorder of frontotemporal corticothalamic networks

The purpose of this study is to determine regions of cerebral cortex activated during the onset and propagation of dense array electroencephalographic (dEEG) epileptiform discharges in patients with juvenile myoclonic epilepsy (JME), through the use of 256 channel, dense array scalp EEG recordings. Ten patients (16–58 years old) with the clinical diagnosis of JME comprised the study group. In all cases the MRI and neurological exams were normal, while standard EEG recordings documented typical “generalized” 4–6 Hz epileptiform patterns. Outpatient dEEG recordings captured epileptiform discharges in each patient. Localization of onset and spread of discharges in relation to a standard MRI model was accomplished by applying dipole fits and a distributed linear inverse method of cortically constrained source analysis. All patients showed epileptiform discharges that localized to sources that included orbitofrontal/medial frontopolar cortex, while basal–medial temporal lobe sources were observed in 5/10 subjects. In many ways similar to discharges of typical absence, epileptiform patterns in JME are usually irregular and frequently include temporal lobe structures as the dominant contributors to the discharges. We find that epileptiform discharges in patients with JME are not “generalized” in the sense of bilaterally synchronous diffuse onset. Rather, discharges have both localized onsets and a restricted cortical network during propagation that includes regions of frontal and temporal cortex.     

New daily persistent headache: clinical perspective

New daily persistent headache is a recognized form of primary chronic daily headache. It is unique in its presentation and course. The goal of this article is to discuss the clinical characteristics, triggering factors, possible underlying pathogenesis and treatment options for this unique headache disorder. At present prognosis for new daily persistent headache is considered poor with very few effective treatment options. A new treatment paradigm for new daily persistent headache based on triggering events will be suggested. The current International Classification of Headache Disorders 2 criteria for new daily persistent headache will also be discussed including its apparent inadequacies and revised criteria will be recommended.     

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.     

Chronic daily headache in a primary care population: prevalence and headache impact test scores

BACKGROUND: Population-based surveys estimate the prevalence of chronic daily headache (CDH) in the general community to be approximately 4%. The prevalence of CDH among patients seen in the primary care setting in the United States, however, is unknown. PURPOSE: To estimate the prevalence and associated burden of suffering of CDH in a primary care patient population. METHODS: Cross-sectional survey of a randomly selected sample of 1500 adult patients in an academic Family Medicine Center was done. Outcome measures include self-reported headache frequency and Headache Impact Test scores. RESULTS: Completed questionnaires were returned by 853 (57%) patients. The mean age of respondents was 49 years (SD = 16), with a range of 18 to 94 years. Two hundred ninety-six (58%) patients reported having had 1 or more severe headaches in the past month. Seventy-four (9%) patients reported a frequency of headache consistent with CDH, defined as the occurrence of headache 15 or more days in the past month. Twenty-four patients (32%) with CDH either believed that none of their doctors know that they experienced headaches or were not sure if their doctors were aware of their headaches, and 21 (28%) reported that they have not needed a doctor's care for their headaches. CONCLUSIONS: The prevalence of CDH is greater among a primary care patient population compared to the general community. A substantial proportion of patients with CDH do not bring their headaches to the attention of their health-care providers. In light of the advances in the development of effective medications for migraines and the growing body of evidence implicating medications as a contributing cause of CDH, it may be appropriate to encourage patients to inform their health-care providers about their headaches and to encourage providers to identify patients with frequent headaches.     

Chronic daily headache

Abstract Chronic daily headache relates to the daily or almost daily occurrence of headache in a nonparoxysmal pattern. In this review, I discuss the presentation, development, outcome, and treatment of chronic daily headache. In the context of the development of chronic daily headache, a headache continuum is presented along with its underlying pathophysiology. The treatment section covers rebound headache, analgesic and vasoconstrictor withdrawal, and the use of long-acting opioids in intractable patients. The review concludes with a discussion of hemicrania continua, an indomethacin-responsive headache syndrome.     

Chronic daily headache in children and adolescents presenting to tertiary headache clinics

BACKGROUND: Adults with chronic daily headache often describe a transformation from episodic migraine and partial retention of migrainous features. Although chronic daily headache has not been investigated as carefully in the pediatric population, one study showed a predominance of coexisting daily headache and episodic migraine, without a clear history of transformation. OBJECTIVE: To identify the clinical features of chronic daily headache in children and adolescents, to evaluate the efficacy of current headache classification criteria, and to compare the features of coexistent daily and episodic headaches so as to determine whether they represent separate syndromes or different stages in the "transformation" process. DESIGN: We surveyed 189 consecutive patients, 18 years of age or younger, who presented for initial evaluation of daily or near daily headache at one of 9 tertiary headache clinics. Data were collected in semistructured interviews employing a standard questionnaire and analyzed using Statistical Analysis Systems and Stata statistical software computer programs. RESULTS: Of the patients enrolled, 70% were female and 87% were white. Mean age was 13.0 +/- 3.1 years. Male gender was associated with a higher degree of reported disability. A family history of headache (typically migraine) was described in 79%. Use of nonsteroidal anti-inflammatory drugs 5 days per week or more was reported by 44% of patients. The International Headache Society (IHS) criteria failed to classify 64% of patients and criteria proposed by Silberstein et al failed to classify 31% of patients. Participating physicians misclassified patients according to criteria of the IHS and Silberstein et al in one third of cases. Nearly one quarter of patients reported two separate headache types with distinguishing characteristics. "Baseline" headache was present 27.3 +/- 4.1 days per month with a mean pain intensity of 5.9 +/- 2.1 on a 10-point scale. Superimposed episodic headache occurred 4.7 +/- 3.8 days per month with a mean pain intensity of 8.4 +/- 1.4, and was more often accompanied by other migrainous symptoms. After logistic regression to control for pain intensity, the only statistically significant difference between the two headache types was a lower prevalence of tension-type head pain with the superimposed headache. CONCLUSIONS: Our data suggest that rather than having two coexistent headache types, children and adolescents with chronic daily headache have a single syndrome that, in many cases, will paroxysmally worsen and gather migrainous features.