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1.  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.
doi:10.1186/1129-2377-15-8
PMCID: PMC3936800  PMID: 24524413
Hemicrania continua; Headache classification
2.  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.
doi:10.1186/1129-2377-15-15
PMCID: PMC3995299  PMID: 24641507
Migraine; Tension-type headache; Cluster headache; Childhood; Adolescence; Evolution
3.  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.
doi:10.1186/1129-2377-15-56
PMCID: PMC4166399  PMID: 25178541
Cluster headache; Diagnostic mistakes; Therapeutic mistakes; Headache guidelines
4.  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.
doi:10.1186/1129-2377-15-61
PMCID: PMC4182754  PMID: 25216994
Hemicrania continua; Headache classification indomethacin
5.  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.
doi:10.1186/1129-2377-14-14
PMCID: PMC3620440  PMID: 23565739
Cluster headache; Paroxysmal hemicrania; SUNCT; Trigeminal autonomic cephalalgias; Hemicrania continua; Error; Pitfall; Misdiagnosis; Mismanagement
6.  An electronic diary on a palm device for headache monitoring: a preliminary experience 
The Journal of Headache and Pain  2012;13(7):537-541.
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.
doi:10.1007/s10194-012-0473-2
PMCID: PMC3444534  PMID: 22842873
Medication overuse headache; Electronic diary; Headache; Feasibility; Compliance
7.  Italian guidelines for primary headaches: 2012 revised version 
The Journal of Headache and Pain  2012;13(Suppl 2):31-70.
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.
doi:10.1007/s10194-012-0437-6
PMCID: PMC3350623  PMID: 22581120
Guidelines; Primary headaches; Symptomatic and prophylactic treatment; Pharmacological and non pharmacological
8.  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.
doi:10.1007/s10194-011-0297-5
PMCID: PMC3056001  PMID: 21359874
Headache; Childhood; Paediatric headaches; Diagnosis; Epidemiology; Defining features
9.  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.
doi:10.1007/s10194-011-0297-5
PMCID: PMC3056001  PMID: 21359874
Headache; Childhood; Paediatric headaches; Diagnosis; Epidemiology; Defining features
10.  Migraine and psychiatric comorbidity: a review of clinical findings 
The Journal of Headache and Pain  2011;12(2):115-125.
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.
doi:10.1007/s10194-010-0282-4
PMCID: PMC3072482  PMID: 21210177
Migraine; Comorbidity; Psychiatric disorders; Depression; Meta-analysis
11.  Migraine and psychiatric comorbidity: a review of clinical findings 
The Journal of Headache and Pain  2011;12(2):115-125.
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.
doi:10.1007/s10194-010-0282-4
PMCID: PMC3072482  PMID: 21210177
Migraine; Comorbidity; Psychiatric disorders; Depression; Meta-analysis
12.  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.
doi:10.1007/s10194-010-0256-6
PMCID: PMC3072476  PMID: 21170567
Migraine; Tension-type headache; Symptomatic treatment; Pharmacological prophylaxis; Non-pharmacological treatment
13.  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.
doi:10.1007/s10194-010-0256-6
PMCID: PMC3072476  PMID: 21170567
Migraine; Tension-type headache; Symptomatic treatment; Pharmacological prophylaxis; Non-pharmacological treatment
14.  Guidelines for telematic second opinion consultation on headaches in Europe: on behalf of the European Headache Federation (EHF) 
The Journal of Headache and Pain  2010;11(4):345-348.
The seeking of a second opinion is the long-established process whereby a physician or expert from the same or a similar specialty is invited to assess a clinical case in order to confirm or reject a diagnosis or treatment plan. Seeking a second opinion has become more common in recent years, and the trend is associated with significant changes in the patient-doctor relationship. Telemedicine is attractive because it is not only fast but also affordable and thus makes it possible to reach highly qualified centres and experts that would otherwise be inaccessible, being impossible, or too expensive, to reach by any surface transport. In Europe, the European Headache Federation (EHF), being able to draw on a group of headache experts covering all the European languages, is the organisation best placed to provide qualified second-opinion consultation on difficult headache cases and to develop a Headache Medical Opinion Service Centre. The provision of good quality clinical information is crucial to the formulation of a valid, expert second opinion. This preliminary step can be properly accomplished only by the primary health care provider through the furnishing of an appropriate clinical report, together with the results of all available tests, including original films of all imaging studies already performed. On receiving the EHF’s proposed standardised data collection form, properly filled in, we may be sure that we have all the relevant data necessary to formulate a valid expert second opinion. This form can be accessed electronically and downloaded from the EHF website. Once finalised, the EHF second opinion project should be treated as a pilot strategy that requires careful monitoring (for the first year at least), so that appropriate changes, as suggested by the retrospective analysis and its quality control, can be implemented.
Electronic supplementary material
The online version of this article (doi:10.1007/s10194-010-0211-6) contains supplementary material, which is available to authorized users.
doi:10.1007/s10194-010-0211-6
PMCID: PMC3476343  PMID: 20373124
Headaches; Guidelines; Telemedicine; Telematic; Second opinion; Consultation
15.  Guidelines for the organization of headache education in Europe: the headache school II 
The Journal of Headache and Pain  2010;11(2):161-165.
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.
doi:10.1007/s10194-010-0195-2
PMCID: PMC3452286  PMID: 20179986
Education; Europe; Headache
16.  A review of current European treatment guidelines for migraine 
Migraine is one of the ten most disabling disorders worldwide, and despite recent developments in the management of migraine, it remains underdiagnosed and undertreated. Guidelines for the management of migraine aim to improve the quality of patient care and to assist professionals in decision making in relation to the overall healthcare process. Most European countries have published national clinical practice guidelines for migraine treatment. These guidelines need to be kept up-to-date with the most recent best clinical evidence and therapeutic strategies to ensure their optimal use to improve health outcomes. The aim of this review is to compare the English language guidelines available across Europe, analyzing differences and similarities, in order to provide a general overview to assist in assessing whether a European consensus on migraine treatment can be achieved.
doi:10.1007/s10194-009-0179-2
PMCID: PMC3452183  PMID: 20020170
National guidelines; Migraine management; Stratified care; Stepped care
17.  Meeting patient expectations in migraine treatment: what are the key endpoints? 
The Journal of Headache and Pain  2008;9(4):207-213.
Clinical outcomes of migraine treatment are generally based on two major endpoints: acute pain resolution and effects on quality of life (QOL). Resolution of acute pain can be evaluated in a number of ways, each increasingly challenging to achieve; pain relief, pain freedom at 2 h, sustained pain-freedom, and SPF plus no adverse events (SNAE, the most challenging). QOL questionnaires help assess the burden of migraine and identify optimal treatments. Pain resolution and improved QOL form the basis of the ultimate target—meeting patient expectations, to achieve patient satisfaction. To achieve this, it is crucial to choose appropriate endpoints that reflect realistic treatment goals for individual patients. Moreover, SNAE can help discriminate between triptans, with almotriptan having the highest SNAE score. Kaplan–Meier plots are also relevant when evaluating migraine treatments. The use of symptomatic medication may lead to the paradoxical development of medication-overuse headache. In general practice, patients should use simple tools for pain measurement (e.g. headache diary) and a QOL questionnaire. A composite endpoint of pain resolution and QOL restoration would constitute a step forward in migraine management.
doi:10.1007/s10194-008-0052-8
PMCID: PMC3451945  PMID: 18607535
Migraine treatment; Outcome; Quality of life

Results 1-17 (17)