Refractory headache: historical perspective, need, and purposes for an operational definition

The study of migraine has yielded many benefits for headache patients. Little research, however, has been performed on refractory migraine (RM) headache, a term often used interchangeably with intractable migraine. This may be a consequence of a lack of a well-accepted definition. In a survey performed by the Refractory Headache Special Interest Section (RHSIS) on the American Headache Society (AHS) in 2006, 58% of the members agreed that a definition for refractory headache should be added to the International Classification of Headache Disorders-2. A PubMed search identified 21 articles that defined refractory or intractable headache/migraine. Sixteen (76%) defined the term "refractory" and 5 (24%) defined the term "intractable." Many of these definitions did not address the need for an adequate trial of a preventive medicine, disability, and medication overuse. An operational definition will allow us to better characterize the disorder, address unmet medical needs, and identify the most effective treatments. RHSIS of the AHS has proposed a definition of RM. It is our hope that this definition will spur interest in this entity and will lead to further research in the area.     

Unusual headache syndromes in children

Headaches represent one of the most common reasons why children and adolescents seek medical attention and are the primary reason that they are referred to pediatric neurology practices. The most common headache syndromes diagnosed are migraine, tension-type, and chronic daily headache, and the bulk of recent medical literature regarding headache in children has focused on these clinical entities. Children are prone to have unusual headache syndromes, most of which fall under the category of "primary headache," most notably as manifestations of migraine with aura. Included within this group are basilar-type and hemiplegic migraine. The most intriguing subset included in the International Headache Society's classification system is the so-called "periodic syndromes of childhood that are precursors to migraine." These syndromes, quite peculiar to children, present a wide variety of episodic symptoms, including movement disorders, vomiting, ataxia, and vertigo, and may not include headache at all. This article provides an overview of some of the more unusual headache syndromes in children and adolescents.     

Prevalence of sleep disorders in childhood and adolescence with headache: a case-control study

Although a relationship between headache and sleep disturbances has been reported in adults, only few data have been available in children. Accordingly, we performed a survey to determine the prevalence of sleep disturbances in children with migraine and tension-type headache. A questionnaire of history and clinical data and of sleep disturbances was given to parents of 283 headache subjects (164 with migraine and 119 with tension-type headache). Results were compared to a normative group comparable for age and sex of 893 normal healthy subjects. Migraine subjects showed a higher prevalence of sleep disturbances during infancy as well as 3-month colic. In both headache groups, more parents had sleep disturbances and there was a higher occurrence of co-sleeping and napping. A high frequency of sleep disturbances involving sleep quality, night awakening, nocturnal symptoms and daytime sleepiness was reported in headache children. No statistical differences were found in the prevalence of sleep disturbances between migraine and tension-type headache. However, the migraine group tended to have "disturbed sleep" more often with increased prevalence of nocturnal symptoms such as sleep breathing disorders and parasomnias. Our results give further support to an association between sleep and migraine that may have a common intrinsic origin.     

How many different headaches do you have?

Patients with migraine attending a specialist clinic often have more than one type of headache. One hundred and two patients attending the City of London Migraine Clinic for the first time were asked: "What type(s) of headache do you think you have?" A separate diagnosis was made by the doctor, who was blinded to the self-diagnosis. On clinic diagnosis, 27 (26.5%) patients were found to have migraine plus an additional non-migraine headache. When compared with the self-diagnosis, 15 (56%) of these had correctly self-diagnosed two types of headache. Many migraineurs can distinguish migraine from non-migraine headaches when they have both.     

Current Aura Without Headache

Purpose of Review

This review evaluates and explains our current understanding of a rare subtype of migraine, typical aura without headache, also known as migraine aura without headache or acephalgic migraine.

Recent Findings

Typical aura without headache is a known entity within the spectrum of migraine. Its pathophysiology is suggested to be similar to classic migraines, with cortical spreading depression leading to aura formation but without an associated headache. No clinical trials have been performed to evaluate treatment options, but case reports suggest that most patients will respond to the traditional treatments for migraine with aura. Bilateral greater occipital nerve blocks may be helpful in aborting migraine with prolonged aura. Transcranial magnetic stimulation has shown efficacy in aborting attacks of migraine with aura but has not been specifically tested in isolated aura.

Summary

Typical aura without headache occurs exclusively in 4% patients with migraine, and may take place at some point in 38% of patients with migraine with aura. Typical aura without headache commonly presents with visual aura without headache, brainstem aura without headache, and can also develop later in life, known as late-onset migraine accompaniment.

     

Psychogenic Headache

SYNOPSIS
An attempt has been made to identify what is commonly referred to as "psychogenic headache" and to differentiate between the most frequently encountered psychiatric disorders where headache is the main symptom. These disorders include anxiety reactions, conversion reactions and depressive reactions. While it is recognized that psychogenic factors often play a major part in migraine headaches and vascular headaches⁸ and possibly cluster headaches, this topic has not been discussed here as the literature on these types of headache is already extensive.This article attempts to review the causes, types, location and duration of psychogenic headaches and the types of premorbid personality encountered in the sufferer. It suggests how the diagnosis of psychogenic headache may be made and notes that effective treatment will depend upon establishing the correct diagnosis and include not only treatment of the symptom, i.e., the headache, but treatment of the underlying psychiatric disorder also.     

Effect of autogenic training on drug consumption in patients with primary headache: an 8-month follow-up study

OBJECTIVE: To examine the effects of Schultz-type autogenic training on headache-related drug consumption and headache frequency in patients with migraine, tension-type, or mixed (migraine plus tension-type) headache over an 8-month period. BACKGROUND: Behavioral treatments often are used alone or adjunctively for different types of headache. There are, however, only a few studies that have compared the efficacy and durability of the same treatment in different types of primary headache, and the effects of treatment on headache-related drug consumption rarely have been assessed even in these studies. METHODS: Twenty-five women with primary headache (11 with mixed headache, 8 with migraine, and 6 with tension-type headache) were evaluated via an open-label, self-controlled, 8-month, follow-up study design. After an initial 4 months of observation, patients began learning Schultz-type autogenic training as modified for patients with headache. They practiced autogenic training on a regular basis for 4 months. Based on data from headache diaries and daily medication records, headache frequencies and the amounts of analgesics, "migraine-specific" drugs (ergots and triptans), and anxiolytics taken by the patients were compared in the three subgroups over the 8-month period. Results.-From the first month of implementation of autogenic training, headache frequencies were significantly reduced in patients with tension-type and mixed headache. Significant reduction in frequency was achieved in patients with migraine only from the third month of autogenic training. Decreases in headache frequencies were accompanied by decreases in consumption of migraine drugs and analgesics resulting in significant correlations among these parameters. Reduction in consumption of anxiolytic drugs was more rapid and robust in patients with tension-type headache compared to patients with migraine, and this outcome failed to show any correlation with change in headache frequency. CONCLUSION: Schultz-type autogenic training is an effective therapeutic approach that may lead to a reduction in both headache frequency and the use of headache medication.     

Sinus CT scan findings in "sinus headache" migraineurs

OBJECTIVE: To evaluate the sinus CT scan findings in "sinus headache" migraineurs, and to compare the findings to nonmigraine "sinus headache" patients. BACKGROUND: The majority of patients presenting with "sinus headache" satisfy the International Headache Society (IHS) criteria for migraine headache. Few studies have correlated the rhinologic complaints and computed tomography (CT) findings in these patients. METHODS: Thirty-five patients with "sinus headache" were evaluated prospectively and referred for CT of the paranasal sinuses. The CT scans were assessed for sinus abnormality (recorded as a Lund-Mackay [L-M] score) and were analyzed for concha bullosa and septal deviation. The findings in the migraine cohort were compared with the nonmigraine "sinus headache" patients. FINDINGS: Twenty-six patients (74.3%) satisfied the IHS criteria for migraine. The mean CT scan L-M score did not differ significantly between the migraine (2.07) and nonmigraine cohort (2.66). Five of the migraine group had substantial sinus disease radiographically (with L-M scores of 5 or above). Concha bullosa of at least 1 middle turbinate was more common in the nonmigraine cohort. An analysis of the sidedness of the headaches, sinus disease, concha bullosa, and/or septal deviation is presented. CONCLUSIONS: The majority of "sinus headache" patients satisfy the IHS criteria for migraine. Surprisingly, these patients often have radiographic sinus disease. This raises the possibility of selection bias in otolaryngology patients, inaccurate diagnosis, or radiographic sinus disease and migraine as comorbid conditions. Positive migraine histories apparently do not obviate the need for a thorough ENT workup, possibly including CT scanning.     

IHS criteria and gender: a study on migraine and tension-type headache in children and adolescents

The aim of this study was to investigate whether the IHS criteria for migraine and tension-type headache depend on gender. Among 409 children and adolescents with recurrent idiopathic headache seen at a university outpatient clinic, girls had significantly more often migraine with aura. Also, there was a trend towards a higher frequency of tension-type headache in girls. In migraine, aggravation of headache by physical activity and occurrence of aura symptoms were more common in females, whereas vomiting and phonophobia occurred more often in males. In tension-type headache, females more often reported mild intensity of headache. All other criteria were similar in both sexes. Age influenced the expression of some of the accompanying symptoms in the various types of migraine, but had only minimal influence on other diagnostic criteria of migraine and tension-type headache in females as well as in males. Our study suggests that the frequency of migraine (except that of migraine with aura) is similar among girls and boys, that tension-type headache may occur more often in girls, and that gender has some influence on the IHS criteria for migraine, but almost no influence on those of tension-type headache.     

Treating early versus treating mild: timing of migraine prescription medications among patients with diagnosed migraine

INTRODUCTION: Although research suggests that early treatment of migraine headache when the pain is mild results in better outcomes for patients, many patients delay taking their acute-migraine medication until their headaches are moderate or severe. Understanding when and why patients use their migraine medications is an important first step to improve migraine management. METHODS: A prospective observational study, conducted at a major national retail pharmacy chain with stores across the United States between April 2001 and November 2002, enrolled men and women between 18 and 55 years of age with a physician diagnosis of migraine with or without aura. Baseline data on 690 patients included patient demographics, migraine history, medication use, tendency to avoid or delay treatment of a migraine attack, and reasons for delaying treatment. Reasons for delaying treatment were assessed via a checklist of nine potential reasons. In the follow-up survey completed after treatment of the next migraine attack, patients reported the timing of medication use in relation to pain onset and the severity of the migraine headache at the time they took the medication. RESULTS: Despite the severity of their typical migraine attacks, approximately 49% of the respondents answered, "yes" to the question, "Do you often avoid or delay taking your migraine medications when you start to experience a migraine attack?" The two most common rationales for avoiding or delaying treatment were "wanting to wait and see if it is really a migraine attack" (69%) followed by "only want to take medications if it is a severe attack" (46%). In the follow-up survey, regardless of medication used, about 85% of patients did not treat their next migraine attack until the headache pain was moderate or severe, although 74% treated within 1 hour of pain onset. CONCLUSION: These results suggest that patients with migraine often delay their treatment until they have identified their attack as a migraine. In addition, while many patients treated their follow-up headache early, they did not treat when the pain was mild. This suggests that there is an opportunity for physicians to educate their migraine patients on how to differentiate migraine from other headache types and about when and how to use their acute-migraine medication.     

Acephalgic Migraine

SYNOPSIS
Acephalgic migraine is a term used interchangeably with the term migraine equivalents. These terms by definition refer to any migrainous phenomena that may occur in the absence of a migraine headache.¹ Perhaps 20% of migraineurs may experience acephalgic attacks of migraine at one time or another. The idea that various symptoms can occur in the absence of any headache has been noted for hundreds of years, but very little has been written about this condition recently. Some people do not believe the symptoms that are often classified as migraine equivalents are in actuality part of the migraine syndrome. Because there is as yet no specific test for migraine, there is no proof that these various symptoms are due to the same neurovascular dysfunction we know as migraine. The diagnosis of migraine is based only on the patient's history and the exclusion of other diagnoses. It is not unusual for a headache patient to see several headache "specialists" and be given different diagnoses.
Some prefer the term "migraine accompaniments" for neurological or visual symptoms occurring with or without a headache.² The aura of the classic migraine attack may linger into the painful phase and thus "accompany" rather than just precede the headache. At times, symptoms typical of the aura may occur and may not be followed by a headache (acephalgic migraine). The term "complicated migraine"should probably be reserved for those neural and/or visual symptoms that outlast the headache by at least 24 hours and should not be used when referring to symptoms of shorter duration which may accompany the headache or which occur in the absence of migraine headache. Although non-visual migraine equivalents are not nearly as common as visual symptoms, it is important to recognize the fact that migraine may account for almost any recurrent, transient, episodic organ dysfunction.     

Migraine-Related Symptoms in Childhood

Periodic disorders of childhood often represent precursors of migraine. As we advance our knowledge of migraine and its complicated phenotypic presentation in childhood, we have noted the similarities in overlapping symptoms of children presenting with childhood periodic syndromes. There is often a positive family history of migraine in children that present with periodic syndromes. There is a known transformation of periodic syndromes from childhood into adult migraine with and without aura phenotype. There has been an evolution of our understanding of what was initially referred to as periodic symptoms of childhood and more recently been called childhood periodic syndromes and is now evolving into the terminology of episodic symptoms that are associated with migraine. International classification of headache disorders has been instrumental in the evolution of the classification of headache and children adults. The most recent classification utilizes and replaces the periodic symptom terminology with episodic symptoms that will no longer be limited to just childhood. This article will address the evolution of our understanding of the diagnostic criteria, pathophysiology and management of the episodic syndromes that may be associated with migraine.     

Food allergy and headache

Headache is one of the ten most common reasons for visiting a physician. Although many studies have linked food allergy with headache, the subject has often been ignored or minimized in the literature. Now is the time to acknowledge this relationship, according to Dr Mansfield, who describes how diet modification and avoidance can bring relief to many patients with migraine or sinus headache.     

Genetic analysis of migraine headache: a review

Recent advances in genetic analysis of migraine headache are reviewed. Point mutations of P/Q -type Ca2+ channel alpha1 subunit(CACNA1A) gene and Na-K ATPase, alpha2 (ATP1A2) gene have been identified in the familial hemiplegic migraine (FHM-1 and FHM-2, respectively). Mutations in notch-3 gene cause the cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), which is an autosomal dominant inherited disorder often accompanying with migraine like headache. Serotonin (5-HT) related genes, dopamine D2 receptors (DRD2) gene, methylenetetrahydrofolate reductase (MTHFR) gene, and angiotensin converting enzyme (ACE) gene have been noticed as the susceptible genes for migraine pathogenesis. Genetic study of migraine is promising and will provide further understanding of the migraine pathophysiology. Discovery of the responsible or susceptible genes will open an avenue to develop new therapeutic strategy.     

Headache,migraine and risk of brain tumors in women: prospective cohort study

Background

While headache is a common symptom among brain tumors patients, often patients with common headache have concerns of being at risk for developing brain tumors. We aimed to disprove that migraine or headache in general is associated with increased risk of developing brain tumors.

Methods

Prospective study among 39,534 middle-aged women, free of any cancer, and who provided information on headache history at baseline. We followed participants for occurrence of medical record-confirmed brain tumors. We ran multivariable-adjusted Cox proportional hazards models to evaluate associations between any headache, migraine, and non-migraine headache with incident brain tumors. We further evaluated whether migraine frequency and updated headache information during follow-up could be linked with brain tumors.

Results

A total of 13,022 (32.9%) women reported headache, of which 5,731 were classified as non-migraine headache and 7,291 as migraine. During a mean follow-up of 15.8 years, 52 brain tumors were confirmed. The multivariable-adjusted hazard ratios (95% confidence interval) for brain tumors were 1.33 (0.76-2.34) for any headache, 1.18 (0.58-2.41) for migraine and 1.53 (0.75-3.12) for non-migraine headache. The association for any headache was further attenuated in time-varying analyses (1.15; 0.58-2.24). Those who experience migraine six times/year were also not at increased risk of brain tumor (0.67; 0.13-3.32).

Conclusions

Results of this large, prospective cohort study in women do not provide evidence that headache in general or migraine in particular are associated with the occurrence of brain tumors. Our data should reassure patients with headache that brain tumor is not a long-term consequence of headache.     

Occipital Headaches and Neuroimaging in Children

Purpose of Review

The goal of this review is to outline the prevalence and significance of occipital headaches in children and how they relate to neuroimaging findings. We seek to evaluate the concern that occipital headaches in children are indicative of secondary headache pathology by reviewing the yield of neuroimaging in pediatric patients with occipital headache location.

Recent Findings

Occipital headaches are a common presentation of primary headache disorders in children, seen in 7–16% of children presenting for evaluation of headache and in up to 20% of children diagnosed with migraine in the emergency department. Review of recent literature confirms that in and of itself, occipital location of headache in a child with recurrent headache and a normal physical examination should not be regarded as worrisome. Headaches with associated signs on neurologic examination should be investigated for a secondary cause, regardless of headache location. Occipital headaches that do not meet criteria for a primary headache disorder should be evaluated for site-specific occipital headache conditions. Neuroimaging for recurrent headache in children who have normal neurological examinations has an overall low yield (0–4.1%) for actionable findings in recent studies. Importantly, an abnormal neurologic examination often predicts the presence of neuroimaging abnormalities.

Summary

In the absence of an atypical history or abnormalities on clinical examination, occipital headaches in children are no more likely to be associated with intracranial pathology than headaches in other locations. If the child’s headaches are otherwise consistent with migraine or another primary headache disorder, and the neurologic examination is normal, the yield of neuroimaging is low, and imaging can generally be deferred.

     

PEDIATRIC HEADACHE

Introduction: Treatments for headache in children have been selected on the results of a few good quality comparative studies, but also by inference from case studies, etiological studies, and studies on chronic pain in children, and headache in adults.
Methods: A literature search was carried out for all articles written in English from January 1999 to August 2004 detailing headache and migraine in children aged 0-18 years.
Results: Overall, the search revealed:

• 1.  

  Psychotherapy. Meta-analysis shows psychotherapy to be efficacious for tension-type headache and migraine.

       
  
  

Chiari-associated exertional,cough, and sneeze headache responsive to medical therapy

Benign exertional headache is coded as a separate entity within the International Headache Society's classification system, but the pathophysiological mechanisms underlying this clinical headache subtype are unknown and possibly are similar to those generating migraine. Coexistence of migraine and benign exertional headache in the same patient is not unusual, and antimigraine pharmacologic treatments are often effective in both headache types. Regardless, optimal management mandates that the clinician exclude any intracranial or systemic disease that could mimic "primary" exertional headache. The same holds for primary headaches induced by coughing or sneezing; congenital malformations or neoplasms, particularly within the posterior fossa, are not rare in these patients. The neurologic examination may not be sufficiently sensitive to detect the offending lesion. We describe a patient with migraine without aura and exertional secondary headache due to Chiari malformation type I whose headaches responded to treatment with propranolol and indomethacin.     

Migraine and tension-type headaches: the questionable validity of current classification systems.

Headache is the most common symptom patients present to their doctors. Current systems classify the most common recurring headaches as either migraine or tension-type. Review of the literature brings into question this traditional approach to headache classification. These two "types" of headache patterns appear, instead, to be different expressions of the same pathophysiological process, having overlapping symptomatic presentations with certain features emphasized to a greater or lesser extent. Additionally, the same therapies have been demonstrated to be effective for patients traditionally classified in either headache group. This article reviews the overlap of clinical symptoms, pathophysiology, and effective treatments for headaches traditionally diagnosed as migraine and tension-type. An alternative continuum classification model is suggested.     

Clinical manifestations of migraine

Migraine is a disorder with many clinical manifestations that vary from person to person and often from headache to headache in the same person. Several body organ systems are involved in a migraine attack. It is a condition that is underdiagnosed by physicians and misidentified by persons suffering with headaches. Many individuals with migraine self-diagnose as having sinus headaches or tension headaches. This article discusses the many symptoms of migraine and will help the busy practitioner to better recognize and diagnose this common disorder.