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.     

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.     

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.     

New daily persistent headache

Primary new daily persistent headache is a rare disorder of children and adults defined by the onset of daily and unremitting headaches within 3 days of onset lasting 4 hours or more daily. There may be a link between a preceding flu-like or upper respiratory infection in about 15%, a stressful life event in 10%, or extracranial surgery in 10%. Migraine symptoms may be present in over 50%. The headache is generalized in most but may be unilateral in 11% and may be localized to any head region. The diagnosis is one of exclusion as many secondary etiologies can cause similar headaches. The pathophysiology of the primary type is unknown. There are no prospective placebo controlled trials of preventive treatment so prevention is empiric using the same medications for the phenotype of chronic migraine or tension-type headache. Most patients have persistent headaches, although about 15% will remit, and 8% will have a relapsing-remitting type.     

Management of Chronic Daily Headache: Challenges in Clinical Practice

Chronic daily headache (CHD) refers to a category of headache disorders that are characterized by headaches occurring on more than 15 days per month. This category is subdivided into long- and short-duration (>4 or <4 hours) CDH disorders based on the duration of individual headache attacks. Examples of long-duration CDH include transformed migraine (TM), chronic migraine (CM), new daily persistent headache (NDPH), acute medication overuse headache, and hemicrania continua (HC). The goal of this review is to enable clinicians to accurately diagnose and effectively manage patients with long-duration CDH. Patients with CDH often require an aggressive and comprehensive treatment approach that includes a combination of acute and preventive medications, as well as nondrug therapies.     

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.     

Epidemiology of chronic daily headache

Daily or near-daily headache is a widespread problem in clinical practice. The general term of chronic daily headache (CDH) encompasses those primary headaches presenting more than 15 days per month and lasting more than 4 hours per day. CDH includes transformed migraine (TM), chronic tension-type headache (CTTH), new daily persistent headache (NDPH), and hemicrania continua (HC). Around 40% of patients attending a specialized headache clinic meet CDH diagnostic criteria, of which 80% are women. In these clinics about 60% of patients suffer from TM, 20% from CTTH, and 20% meet NDPH criteria. Most, some 80%, overuse symptomatic medications. One should be very cautious on extrapolating these numbers to the general population. CDH prevalence in the general population seems to be around 4% to 5% (up to 8% to 9% for women). Regarding the prevalence of CDH subtypes, NDPH is rare (0.1%), whereas the prevalence of TM (1.5% to 2%) and CTTH (2.5% to 3%) is clearly higher. In contrast to data from specialized clinics, only around a quarter of CDH subjects in the general population overuse analgesics; the prevalence of CDH subjects with analgesic overuse being 1.1% to 1.9% of the general population. Most of these patients with analgesic overuse are TM patients.     

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.     

Classification of Daily and Near-Daily Headaches: Proposed Revisions to the IHS Criteria

SYNOPSIS
The International Headache Society (IHS) headache classification, while a major advance, does not adequately classify the daily and near-daily headache disorders known as chronic daily headache (CDH). We believe that chronic daily headache is a group of disorders which includes chronic tension-type headache (CTTH), transformed migraine (TM), new daily persistent headache (NDPH), and hemicrania continua (HC). We propose specific criteria for transformed migraine, new daily persistent headache, and hemicrania continua, and have modified the criteria for chronic tension-type headache.     

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.     

The differential diagnosis of chronic daily headaches: an algorithm-based approach

Chronic daily headaches (CDHs) refers to primary headaches that happen on at least 15 days per month, for 4 or more hours per day, for at least three consecutive months. The differential diagnosis of CDHs is challenging and should proceed in an orderly fashion. The approach begins with a search for "red flags" that suggest the possibility of a secondary headache. If secondary headaches that mimic CDHs are excluded, either on clinical grounds or through investigation, the next step is to classify the headaches based on the duration of attacks. If the attacks last less than 4 hours per day, a trigeminal autonomic cephalalgia (TAC) is likely. TACs include episodic and chronic cluster headache, episodic and chronic paroxysmal hemicrania, SUNCT, and hypnic headache. If the duration is > or =4 h, a CDH is likely and the differential diagnosis encompasses chronic migraine, chronic tension-type headache, new daily persistent headache and hemicrania continua. The clinical approach to diagnosing CDH is the scope of this review.     

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.     

New daily persistent headache in children and adults

New daily persistent headache (NDPH) is frequently seen in young patients with chronic daily headache. NDPH begins with a sudden onset, often associated with an infection or other physical stress. This headache syndrome is difficult to treat and may persist for years. This review discusses the epidemiology, comorbid symptoms, evaluation, and treatment of this disorder.     

Hemicrania Continua and Symptomatic Medication Overuse

SYNOPSIS
Hemicrania continua (HC) is a rare, strictly unilateral, non-paroxysmal headache disorder characterized by its absolute responsiveness to indomethacin. The pain is usually moderate in intensity and frequently associated with a superimposed"jabs and jolts" headache.
We report two cases of HC which presented as chronic daily headache (CDH) with abortive medication overuse.
CDH can be due to transformed migraine (TM), new daily persistent headache (NDPH), chronic tension-type headache, and HC. All can be unilateral, and all can be associated with medication overuse. Our two cases meet the criteria for HC based on indomethacin responsiveness. One meets the criteria for TM, the other NDPH. Is HC a distinct disorder, or a subset of these other disorders? CDH with medication overuse includes in its differential diagnosis HC.     

Chronic daily headache: identification of factors associated with induction and transformation

BACKGROUND: Chronic daily headache (CDH) is one of the more frequently encountered headache syndromes at major tertiary care centers. The analysis of factors related to the transformation from episodic to chronic migraine (CM) and to the de novo development of new daily persistent headache (NDPH) remain poorly understood. OBJECTIVES: To identify somatic factors and lifestyle factors associated with the development of CM and NDPH. METHODS: We used a randomized case-control design to study the following groups: 1) CM with analgesic overuse (ARH), n = 399; 2) CM without analgesic overuse, n = 158; and 3) NDPH, n = 69. These groups were compared with two control groups: 1) episodic migraine, n = 100; and 2) chronic posttraumatic headache (CPTH); n = 65. Associated medical conditions were assessed. We investigated the case groups for any association with somatic or behavioral factors. Data were analyzed by the two-sided Fischer's exact test, with the odds ratio being calculated considering a 95% confidence interval using the approximation of Woolf. RESULTS: When the active groups were compared with the episodic migraine group, the following associations were found: 1) ARH: hypertension and daily consumption of caffeine; 2) CM: allergies, asthma, hypothyroidism, hypertension, and daily consumption of caffeine; and 3) NDPH: allergies, asthma, hypothyroidism, and consumption of alcohol more than three times per week. The following associations were found when comparing the active groups with CPTH: 1) ARH: asthma and hypertension; 2) CM: allergies, asthma, hypothyroidism, hypertension, and daily consumption of caffeine; and 3) NDPH: allergies, asthma, hypothyroidism, and consumption of alcohol more than three times per week. CONCLUSIONS: Several strong correlations were obtained between patients with specific types of CDH and certain somatic conditions or behaviors; some have not been previously described. Transformation of previously episodic headache or development of a NDPH thus may be related to certain medical conditions and behaviors beyond the frequently incriminated precipitant analgesic overuse. As similar results were obtained when CPTH was used as a control, the correlation is more complex than simple comorbidity.     

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.     

Unilateral photophobia or phonophobia in migraine compared with trigeminal autonomic cephalalgias

Our objective was to compare the presence of self-reported unilateral photophobia or phonophobia, or both, during headache attacks comparing patients with trigeminal autonomic cephalalgias (TACs)—including cluster headache, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and paroxysmal hemicrania—or hemicrania continua, and other headache types. We conducted a prospective study in patients attending a referral out-patient clinic over 5 months and those admitted for an intramuscular indomethacin test. Two hundred and six patients were included. In episodic migraine patients, two of 54 (4%) reported unilateral photophobia or phonophobia, or both. In chronic migraine patients, six of 48 (13%) complained of unilateral photophobia or phonophobia, or both, whereas none of the 24 patients with medication-overuse headache reported these unilateral symptoms, although these patients all had clinical symptoms suggesting the diagnosis of migraine. Only three of 22 patients (14%) suffering from new daily persistent headache (NDPH) experienced unilateral photophobia or phonophobia. In chronic cluster headache 10 of 21 patients (48%) had unilateral photophobia or phonophobia, or both, and this symptom appeared in four of five patients (80%) with episodic cluster headache. Unilateral photophobia or phonophobia, or both, were reported by six of 11 patients (55%) with hemicrania continua, five of nine (56%) with SUNCT, and four of six (67%) with chronic paroxysmal hemicrania. Unilateral phonophobia or photophobia, or both, are more frequent in TACs and hemicrania continua than in migraine and NDPH. The presence of these unilateral symptoms may be clinically useful in the differential diagnosis of primary headaches.     

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.     

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.     

Clinical features of unilateral headaches beyond migraine and cluster headache and their response to indomethacin

The majority of previous studies on unilateral headaches beyond migraine and cluster headache have focussed on certain disorders such as paroxysmal hemicrania, SUNCT and primary stabbing headache. We assessed headache characteristics, importance of neuroimaging and response to indomethacin in an unselected series of uncommon unilateral headaches. We investigated all consecutive patients presented with unilateral headaches not fulfilling ICHD-II criteria of migraine and cluster headache. Patients underwent cranial magnetic resonance imaging or computed tomography as well as an indo-test, i.e. oral indomethacin 75 mg b.i.d. for 3 days. Among 63 patients we diagnosed primary stabbing headache in 12 patients, (probable) paroxysmal hemicrania in 6 and tension-type headache in 3 patients. One patient each had probable SUNCT, new daily persistent headache and nasociliary neuralgia. Eight patients had a secondary headache and 31 could not be classified according to ICDH-II. Imaging revealed lesions causally related to the headache in 8 patients. Indo-test achieved full remission of headache in 13 of 51 patients. At follow-up 11 ± 3 months after the first visit 29% of the patients were headache-free for ≥3 months. In conclusion, almost half of the patients presented with unilateral headaches beyond migraine and cluster headache cannot be classified according to ICHD-II. Among classifiable headaches primary stabbing headache was the most common. Imaging should be considered to rule out secondary headaches. The course is favourable in one third of the patients.