Headache and neck.

Cervicogenic headache (CEH) is currently identified with different diagnostic criteria. The latest one is the International classification of headache disorders (ICHD), 3rd edition (2018). At the present time, there are not enough published articles with reliable sensitivity and specificity that may support a classification for clinical and research purposes. Current literature suggests improvement to the classification(s). The ICHD criteria should be modified to reach an optimal sensitive and specific level to identify CEH as a secondary headache. The B, C1, and C2 criteria should be implemented with proposed suggestions. The C3 criterion should be upgraded. Criteria such as mechanical precipitation of pain by digital pressure on neck trigger points and specific movements, strictly unilateral pain without side-shift, diffuse unilateral shoulder and arm pain, pain starting posteriorly and spreading anteriorly should be integral part of the classification.

Familial hemiplegic migraine type 2 due to a novel missense mutation in ATP1A2.

BACKGROUND: The mechanisms of genotype-phenotype interaction in Familiar Hemiplegic migraine type 2 (FHM2) are still far from clear. Different ATP1A2 mutations have been described, with a spectrum of phenotypes ranging from mild to severe. No genotype-phenotype correlations have been attempted. CASE PRESENTATION: We describe an Italian family with FHM and a missense ATP1A2 variant (L425H) not previously described. The clinical picture was mild in all the affected members. CONCLUSIONS: Co-segregation of the variant with the aura phenotype was complete in this family, suggesting a 100% penetrance. In silico protein prediction softwares indicate that this variant may change the 3D structure of ATPA1A2 at the cytoplasmic loop between the two central transmembrane helices. Milder FHM phenotypes are rarely reported in literature, likely because case reports are biased towards the most severe phenotypes, with milder forms possibly misdiagnosed as sporadic migraine with aura forms (MAs), even with complex auras. Further studies taking into account intra-familiar variability and functional consequences on the channel protein may help clarify genotype-phenotype correlations.

Frontal infrared thermography in healthy individuals and chronic migraine patients: Reliability of the method.

BACKGROUND: The use of frontal infrared thermography in the diagnosis of primary headaches provided scattering results due to measurement fluctuations and different types of headaches or research protocols. OBJECTIVE: This study aims to assess the reliability of frontal infrared thermography in healthy individuals and provide a preliminary evaluation in chronic migraine patients using a commercial infrared thermal camera. METHODS: Thermographic images were acquired in 20 controls and 15 patients at three consecutive time-points in two daily sessions. The Side Difference and Asymmetry Index parameters were defined. The reproducibility of the measurements, the correlation of Asymmetry Index and Side Difference with clinical evaluations and patient perceptions, and the ability of the parameters to discriminate between patients and controls were investigated. RESULTS: We reported a good reproducibility of the measurements (Inter-class Correlation Coefficient > 0.75 and Coefficient of Variation < 13.4%), independent from external factors. The Side Difference was significantly different between patients and controls ( p < 0.001). The Asymmetry Index showed good correlation with the side of unilateral pain ( p = 0.0056). CONCLUSIONS: Frontal infrared thermography can be used to quantify the difference between the right and the left side of frontal vascular changes in chronic migraine patients, provided that standardized conditions are satisfied.

Cervicogenic headache: too important to be left un-diagnosed.

A comparison has been made between the cervicogenic headache criteria in the new IHS classification of headaches (3rd edition-beta version) and The Cervicogenic Headache International Study Group's (GHISG) criteria from 1998. In a more recent version, the CHISG criteria consist of 7 different items. While "core cases" of cervicogenic headache (CEH) usually fulfill all 7 criteria, the IHS classification--3rd edition beta version--fulfills only 3 criteria. Although the new three beta version represents an improvement from the previous one, it does not quite seem to live up to the expectations for a diagnostic system for routine, clinical use.

Hemicrania continua: towards a new classification?

Hemicrania continua (HC) was described and coined in 1984 by Sjaastad and Spierings. Later cases, carrying this appellation, should conform to the original description. The proposed classification criteria (ICHD 3rd edition beta version) for HC focus e.g. on localized, autonomic and "vascular" features. Such features do, however, not belong to the core symptomatology of HC and should accordingly be removed. The genuine, original HC will then re-appear.The headache that the new criteria refer to, has in an unfair and unjustified manner been given the designation HC. A revision of the proposed criteria seems mandatory.

Hemicrania continua: major shortcomings in the new classification.

Hemicrania continua ( HC) was described and coined by Sjaastad and Spierings in 1984. Later cases, carrying this appellation should, grossly, conform to this original description. The proposed classification criteria (ICHD, 3rd edition beta version) for HC has major shortcomings, and ordinary HC cases do not fulfill the proposed criteria. Relatively rare symptoms and signs are e.g. made obligatory (point C 1). And the recommended dosage of indomethacin- both test and long-term dosages-is unallowably high. In this way, bogus HC cases are systematically created. This irrational diagnostic system is in urgent need of a major revision.

Diagnostic and therapeutic errors in cluster headache: a hospital-based study.

BACKGROUND: Cluster headache (CH) is a severe, disabling form of headache. Even though CH has a typical clinical picture it seems that its diagnosis is often missed or delayed in clinical practice. CH patients may thus face: misdiagnosis, unnecessary investigations and delays in accessing adequate treatment. This study was conducted to investigate the occurrence of diagnostic and therapeutic errors with a view to improving the clinical and instrumental work-up in affected patients. METHODS: Our study comprised 144 episodic CH patients: 116 from Italy and 28 from Eastern European countries (Moldova, Ukraine, Bulgaria). One hundred six patients (73.6%) were examined personally and 38 (26.4%) were evaluated through telephone interviews conducted by headache specialists using an ad hoc questionnaire developed by the authors. RESULTS: The sample was predominantly male (M:F ratio 2.79:1) and had a mean age of 42.4 ± 9.8 years; approximately 76% of the patients had already consulted a physician about their CH at the onset of the disease. The mean interval between onset of the disease and first consultation at a headache center was 4.1 ± 5.6 years. The patients had consulted different specialists prior to receiving their CH diagnosis: neurologists (49%), primary care physicians (35%), ENT specialists (10%), dentists (3%), etc. Misdiagnoses at first consultation were recorded in 77% of the cases: trigeminal neuralgia (22%), migraine without aura (19%), sinusitis (15%), etc. The average "diagnostic delay" was 5.3 ± 6.4 years and the condition was diagnosed approximately ("doctor delay": one year). Instrumental and laboratory investigations were carried out in 93% of the patients prior to diagnosis of CH. Some of the patients had never received abortive or preventive medications, either before or after diagnosis. Medical prescription compliance: 88% of the cases. CONCLUSIONS: Our results emphasize the need to improve specialist education in this field in order to improve recognition of the clinical picture of CH and increase knowledge of the proper medical treatments for de novo CH. Continuous medical education on CH should target general neurologists, primary care physicians, ENT specialists and dentists. A study on a larger population of CH patients may further improve error-avoidance strategies.

The evolution of headache from childhood to adulthood: a review of the literature.

Headache is one of the most common disorders in childhood, with an estimated 75% of children reporting significant headache by the age of 15 years. Pediatric migraine is the most frequent recurrent headache disorder, occurring in up to 28% of older teenagers. Headaches rank third among the illness-related causes of school absenteeism and result in substantial psychosocial impairment among pediatric patients. The aim of this study was to clarify the evolution of the clinical features of primary headache in the transition from childhood to adulthood through a review of relevant data available in the PubMed and Google Scholar databases for the period 1988 to July 2013.The search strategy identified 15 published articles which were considered eligible for inclusion in the analysis (i.e., relevant to the investigation of pediatric headache outcome). All were carried out after the publication of the first version of the International Classification of Headache Disorders (ICHD-I). The availability of data on the evolution of primary headaches over a period of time is important from both a clinical and a public health perspective. The identification of prognostic factors of the evolution of headache (remission or evolution into another headache form) over time should be an objective of future headache research for the development of prevention strategies. Given that headache is a major factor contributing to school absenteeism and poorer quality of life not only in childhood but also in adolescence, understanding the natural history and the management of the different headache forms is vital for our future.

The Headache Under-Response to Treatment (HURT) Questionnaire: assessment of utility in headache specialist care.

The HURT Questionnaire consists of eight questions which the patient answers as a measure of effectiveness of intervention against headache. This first assessment of clinical utility was conducted in headache specialist centres in three countries in order to demonstrate that HURT was responsive to change induced by effective management. We administered HURT on three occasions to 159 consecutive patients seeking non-urgent care from centres in Denmark and the United Kingdom: the first before the initial visit to the centres; the second at the initial visit; and the third when the specialist judged that the best possible outcome had been achieved in each patient. Questionnaires were also answered by 42 patients at initial and final visits to a centre in Italy. Internal consistency reliability was very good (α = 0.85) while test-retest reliability was fair to low (κ = 0.38-0.62 and r (s) = 0.49-0.76), possibly because headache was unstable prior to start of management. There were significant changes in responses post-intervention compared with baseline (p < 0.01), indicating a favourable outcome overall in up to 77% of patients, and responsiveness to change, but there was no improvement in patients' concerns about side effects of medication (p = 0.18). We conclude that the questionnaire has utility across headache disorders. It can help patients describe headache frequency and headache-attributed disability, medication use/efficacy/tolerability, self-efficacy and knowledge about headache. It may guide physicians in assessment of disability of individual patients, how to proceed with management towards the best possible outcome, and in evaluating the quality of management.

Diagnostic and therapeutic errors in trigeminal autonomic cephalalgias and hemicrania continua: a systematic review.

Trigeminal autonomic cephalalgias (TACs) and hemicrania continua (HC) are relatively rare but clinically rather well-defined primary headaches. Despite the existence of clear-cut diagnostic criteria (The International Classification of Headache Disorders, 2nd edition - ICHD-II) and several therapeutic guidelines, errors in workup and treatment of these conditions are frequent in clinical practice. We set out to review all available published data on mismanagement of TACs and HC patients in order to understand and avoid its causes. The search strategy identified 22 published studies. The most frequent errors described in the management of patients with TACs and HC are: referral to wrong type of specialist, diagnostic delay, misdiagnosis, and the use of treatments without overt indication. Migraine with and without aura, trigeminal neuralgia, sinus infection, dental pain and temporomandibular dysfunction are the disorders most frequently overdiagnosed. Even when the clinical picture is clear-cut, TACs and HC are frequently not recognized and/or mistaken for other disorders, not only by general physicians, dentists and ENT surgeons, but also by neurologists and headache specialists. This seems to be due to limited knowledge of the specific characteristics and variants of these disorders, and it results in the unnecessary prescription of ineffective and sometimes invasive treatments which may have negative consequences for patients. Greater knowledge of and education about these disorders, among both primary care physicians and headache specialists, might contribute to improving the quality of life of TACs and HC patients.

Sexual function and distress in women treated for primary headaches in a tertiary university center.

INTRODUCTION: Primary headaches are common in women and impact on their quality of life and psychosocial functioning. Few data are available on sexuality in female headache sufferers. AIM: An observational pilot study was conducted to assess sexual function and distress in women treated for primary headaches in a tertiary university center. METHODS: From a total of 194 women consecutively observed over a 3-month period, 100 patients were recruited. Migraine with and without aura, and tension-type headache, both episodic and chronic (CTTH), were diagnosed according to the International Classification of Headache Disorders. A detailed pharmacological history was collected, and anxiety and depression were assessed using validated scales. The Female Sexual Function Index (FSFI) and Female Sexual Distress Scale-Revised were administered. MAIN OUTCOME MEASURES: The main outcome measures are sexual symptoms and distress in women treated for primary headaches. RESULTS: More than 90% of the women had a median FSFI full-scale score under the validated cutoff, while 29% reported sexual distress. Hypoactive sexual desire disorder (HSDD) was diagnosed in 20% of the women and the pain domain score (median 2, score range 0-6) was highly affected by the head pain condition. However, the FSFI domain and full-scale scores did not significantly differ by headache diagnosis. The women with CTTH displayed a high rate of sexual distress (45.5%) and a strong negative correlation between desire, arousal, and full-scale FSFI score and number analgesics/month (r: -0.77, P=0.006; r: -0.76, P=0.006; and r: -0.68, P=0.02, respectively). Depression was positively correlated with sexual distress (r: 0.63, P=0.001) only in the women with CTTH. CONCLUSION: Women treated for primary headaches were found to display a high rate of sexual symptoms and distress. Both migraine and tension-type headache were associated with sexual pain and HSDD, but women with CTTH seem to be more prone to develop sexual distress.

An electronic diary on a palm device for headache monitoring: a preliminary experience.

Patients suffering from headache are usually asked to use charts to allow monitoring of their disease. These diaries, providing they are regularly filled in, become crucial in the diagnosis and management of headache disorders because they provide further information on attack frequency and temporal pattern, drug intake, trigger factors, and short-/long-term responses to treatment. Electronic tools could facilitate diary monitoring and thus the management of headaches. Medication overuse headache (MOH) is a chronic and disabling condition that can be treated by withdrawing the overused drug(s) and adopting specific approaches that focus on the development of a close doctor-patient relationship in the post-withdrawal phase. Although the headache diary is, in this context, an essential tool for the constant, reliable monitoring of these patients to prevent relapses, very little is known about the applicability of electronic diaries in MOH patients. The purpose of this study was to evaluate the acceptability of and patient compliance with an electronic headache diary (palm device) as compared with a traditional diary chart in a group of headache inpatients with MOH. A palm diary device, developed in accordance with the ICHD-II criteria, was given to 85 MOH inpatients during the detoxification phase. On the first day of hospitalization, the patients were instructed in the use of the diary and were then required to fill it in daily for the following 7 days. Data on the patients' opinions on the electronic diary and the instructions given, its screen and layout, as well as its convenience and ease of use, in comparison with the traditional paper version, were collected using a numerical rating scale. A total of 504 days with headache were recorded in both the electronic and the traditional headache diaries simultaneously. The level of patient compliance was good. The patients appreciated the electronic headache diary, deeming it easy to understand and to use (fill in); most of the patients rated the palm device handier than the traditional paper version.

Italian guidelines for primary headaches: 2012 revised version.

The first edition of the Italian diagnostic and therapeutic guidelines for primary headaches in adults was published in J Headache Pain 2(Suppl. 1):105-190 (2001). Ten years later, the guideline committee of the Italian Society for the Study of Headaches (SISC) decided it was time to update therapeutic guidelines. A literature search was carried out on Medline database, and all articles on primary headache treatments in English, German, French and Italian published from February 2001 to December 2011 were taken into account. Only randomized controlled trials (RCT) and meta-analyses were analysed for each drug. If RCT were lacking, open studies and case series were also examined. According to the previous edition, four levels of recommendation were defined on the basis of levels of evidence, scientific strength of evidence and clinical effectiveness. Recommendations for symptomatic and prophylactic treatment of migraine and cluster headache were therefore revised with respect to previous 2001 guidelines and a section was dedicated to non-pharmacological treatment. This article reports a summary of the revised version published in extenso in an Italian version.

Cervicogenic headache: a real headache.

Although theories regarding headache originating in the neck have existed for more than 150 years, the term "cervicogenic headache" originated in 1983. Early descriptions pinpoint the characteristic symptoms as dizziness, visual disturbances, tinnitus, and "posterior" headache, conceivably as a consequence of arthrosis, infliction upon the vertebral artery, or with a "migrainous" background and occurring in "advanced age." Cervicogenic headache (mean age of onset, 33 years) displays a somewhat different picture: unilateral headache, starting posteriorly, but advancing to the frontal area, most frequently the main site of pain; usually accompanied by ipsilateral arm discomfort, reduced range of motion in the neck, and mechanical precipitation of exacerbations (eg, through external pressure upon hypersensitive, occipital tendon insertions). Treatment options in treatment-resistant cases include cervical stabilization operations and extracranial electrical stimulation. In a personal, population-based study of 1,838 individuals (88.6% of the population), a prevalence of 2.2% "core" cases was found.

Overview of diagnosis and management of paediatric headache. Part I: diagnosis.

Headache is the most common somatic complaint in children and adolescents. The evaluation should include detailed history of children and adolescents completed by detailed general and neurological examinations. Moreover, the possible role of psychological factors, life events and excessively stressful lifestyle in influencing recurrent headache need to be checked. The choice of laboratory tests rests on the differential diagnosis suggested by the history, the character and temporal pattern of the headache, and the physical and neurological examinations. Subjects who have any signs or symptoms of focal/progressive neurological disturbances should be investigated by neuroimaging techniques. The electroencephalogram and other neurophysiological examinations are of limited value in the routine evaluation of headaches. In a primary headache disorder, headache itself is the illness and headache is not attributed to any other disorder (e.g. migraine, tension-type headache, cluster headache and other trigeminal autonomic cephalgias). In secondary headache disorders, headache is the symptom of identifiable structural, metabolic or other abnormality. Red flags include the first or worst headache ever in the life, recent headache onset, increasing severity or frequency, occipital location, awakening from sleep because of headache, headache occurring exclusively in the morning associated with severe vomiting and headache associated with straining. Thus, the differential diagnosis between primary and secondary headaches rests mainly on clinical criteria. A thorough evaluation of headache in children and adolescents is necessary to make the correct diagnosis and initiate treatment, bearing in mind that children with headache are more likely to experience psychosocial adversity and to grow up with an excess of both headache and other physical and psychiatric symptoms and this creates an important healthcare problem for their future life.

Migraine and psychiatric comorbidity: a review of clinical findings.

Migraine is an extremely common disorder. The underlying mechanisms of this chronic illness interspersed with acute symptoms appear to be increasingly complex. An important aspect of migraine heterogeneity is comorbidity with other neurological diseases, cardiovascular disorders, and psychiatric illnesses. Depressive disorders are among the leading causes of disability worldwide according to WHO estimation. In this review, we have mainly considered the findings from general population studies and studies on clinical samples, in adults and children, focusing on the association between migraine and psychiatric disorders (axis I of the DSM), carried over after the first classification of IHS (1988). Though not easily comparable due to differences in methodology to reach diagnosis, general population studies generally indicate an increased risk of affective and anxiety disorders in patients with migraine, compared to non-migrainous subjects. There would also be a trend towards an association of migraine with bipolar disorder, but not with substance abuse/dependence. With respect to migraine subtypes, comorbidity mainly involves migraine with aura. Patients suffering from migraine, however, show a decreased risk of developing affective and anxiety disorders compared to patients with daily chronic headache. It would also appear that psychiatric disorders prevail in patients with chronic headache and substance use than in patients with simple migraine. The mechanisms underlying migraine psychiatric comorbidity are presently poorly understood, but this topic remains a priority for future research. Psychiatric comorbidity indeed affects migraine evolution, may lead to chronic substance use, and may change treatment strategies, eventually modifying the outcome of this important disorder.

Overview of diagnosis and management of paediatric headache. Part II: therapeutic management.

A thorough evaluation of headache in children and adolescents is necessary to make the correct diagnosis and initiate treatment. In part 1 of this article (Özge et al. in J Headache Pain, 2010), we reviewed the diagnosis of headache in children and adolescents. In the present part, we will discuss therapeutic management of primary headaches. An appropriate management requires an individually tailored strategy giving due consideration to both non-pharmacological and pharmacological measures. Non-pharmacological treatments include relaxation training, biofeedback training, cognitive-behavioural therapy, different psychotherapeutic approaches or combinations of these treatments. The data supporting the effectiveness of these therapies are less clear-cut in children than in adults, but that is also true for the data supporting medical treatment. Management of migraine and TTH should include strategies relating to daily living activities, family relationships, school, friends and leisure time activities. In the pharmacological treatment age and gender of children, headache diagnosis, comorbidities and side effects of medication must be considered. The goal of symptomatic treatment should be a quick response with return to normal activity and without relapse. The drug should be taken as early as possible and in the appropriate dosage. Supplementary measures such as rest in a quiet, darkened room is recommended. Pharmaco-prophylaxis is only indicated if lifestyle modification and non-pharmacological prophylaxis alone are not effective. Although many prophylactic medications have been tried in paediatric migraine, there are only a few medications that have been studied in controlled trials. Multidisciplinary treatment is an effective strategy for children and adolescents with improvement of multiple outcome variants including frequency and severity of headache and school days missed because of headache. As a growing problem both children and families should be informed about medication overuse and the children's drug-taking should be checked.

Guidelines for the organization of headache education in Europe: the headache school II.

In order to promote education on headache disorders, European Headache Federation (EHF) in conjunction with National Headache Societies organizes educational courses meeting uniform standards according to previous published guidelines. Based on six headache summer schools' experience, an EHF subcommittee has reviewed these guidelines, and here the revised version is presented. The goals remain the same: quality courses that will attract physicians and neurologists seeking to increase their knowledge, skills, and understanding in the area of primary and secondary headache. Detailed guidelines, a day-to-day program, and a multiple-choice test battery have now been outlined. It is recommended to include practical sessions with patient interviews and hands-on demonstrations of non-pharmacological treatment strategies. For countries that want a 'low cost' education program, a Video School program of a similar scientific standard has been developed. To be certified for CME credits, patronage, and financial support from EHF, it is highly recommended to adhere to the suggested teaching strategies. We hereby aim to promote and professionalize the education in headache disorders and endorse the educational courses meeting uniform standards of excellence.