Hemicrania continua is an uncommon primary headache disorder, characterized by continuous unilateral pain, where pain exacerbations are associated with cranial autonomic features. The hallmark of this condition is the absolute response to indometacin. We describe the phenotype of this condition in a large series of patients. Thirty-six (92%) patients had side-locked pain and 3 (8%) had side-alternating pain. The majority (82%) of the patients had the chronic (unremitting) form and the severity range of background pain was 1-10 out of 10 on verbal rating scale, with a mean of 6.5. Thirty-eight (97%) of the patients rated the painful exacerbations between 6.5 and 10 with a mean of 9 and 28 (71%) described their severe pain as excruciating. Of the cohort, 97% had at least one cranial autonomic feature during exacerbations: 73% had lacrimation, 51% nasal congestion, 46% conjunctival injection and 40% ptosis and facial flushing. Other cranial autonomic features included rhinorrhoea, forehead/facial sweating, itching eye, eyelid oedema, sense of aural fullness and periaural swelling, miosis, mydriasis and swelling of the cheek and face. Thirty-one (79%) had phonophobia, which was unilateral in 14 (48%); 29 (74%) had photophobia, which was unilateral in 14 (48%); and 27 (69%) had motion sensitivity. In addition, about two-thirds were agitated or restless, or both, and about one-quarter were aggressive, mainly verbally, with severe pain. All patients had a positive placebo-controlled indometacin test (100-200 mg intramuscularly) or a positive oral indometacin trial, or both. We suggest the International Headache Society criteria be revised to remove the absence of side-shift pain as a criterion. Furthermore, revised criteria should encompass a more extensive range of cranial autonomic features and consider pain as fluctuating with moderate, severe and very severe intensity. Currently the sine qua non for hemicrania continua is a response to indometacin. Since there is no reliable clinical marker of that response, we recommend an indometacin test, either orally or by injection, for any patient with unilateral pain, with or without cranial autonomic symptoms.

Little is known about the pathophysiology of cluster headache (CH), one of the most debilitating primary headaches. Interestingly, associations of lung affecting diseases or lifestyle habits such as smoking and sleep apnoea syndrome and CH have been described. Certain genotypes for alpha 1-antitrypsin (alpha(1)-AT) are considered risk factors for emphysema. Our aim was to investigate possible associations between common genotypes of the SERPINA1 gene and CH. Our study included 55 CH patients and 55 controls. alpha(1)-AT levels in serum and the genotype were analysed. Patients CH characteristics were documented. We could not detect any association between CH and a genotype that does not match the homozygous wild type for alpha(1)-AT. Interestingly, there is a significant difference of CH attack frequency in patients who are heterozygous or homozygous M allele carriers. We conclude that the presence of an S or Z allele is associated with higher attack frequency in CH.

Patients with severe primary headache may benefit from consultation with headache specialists, but doctor attendance rates in Japan are very low. More headache patients might consult headache specialists if these specialists were more widely recognized by the public. To determine what information prompted patients to seek consultation with a headache specialist, we questioned 256 primary headache patients about the source of the helpful information concerning consultation with headache specialists. From 191 patients, a total of 235 responses to the questionnaire were obtained. The most common response was 'websites'(33.2%), followed by 'professionals'(23.8%),'acquaintances'(20.9%),'print media'(6.8%) and 'TV/radio'(3.4%). Patients who indicated websites showed the most severe pain and highest impact of headache, and accounted for 52.4% of those with cluster headaches. Development of websites concerning headache specialists would seem likely to increase doctor attendance rates for patients with primary headache.

A slight predominance of cluster pain on the right side has been reported in several studies. The aim of this large retrospective Nordic multicenter study was to estimate the prevalence of right- and left-sided pain in cluster headache (CH) patients with side-locked pain, the prevalence of side shifts in episodic and chronic CH patients, and the occurrence of cranial autonomic symptoms related to pain side. Among 383 cluster patients, 55 (14%) had experienced pain side shift. Of the remaining 328 individuals without side shift, there was no significant difference between the occurrence of right-sided and left-sided pain (54 vs. 46%). The prevalence of side shift was similar for episodic and chronic CH and the occurrence of cranial autonomic symptoms was not influenced by the pain side. In conclusion, previous reports of a side difference in location of cluster pain could not be confirmed in this large Nordic sample.

Cluster headache was first described over 300 years ago, but in the last century our knowledge of the disorder has exploded through both clinical observation and epidemiological data. Although some of the data are conflicting and more need to be obtained, much is known about the disorder. This article reviews the data to date on the prevalence and incidence of the disorder, population differences including gender and race, genetics, comorbid conditions, risk factors for development of the disorder, prognosis, and socioeconomic burden.

OBJECTIVE: To describe the self-treatment of cluster headache with kudzu. BACKGROUND: Many cluster headache patients take over-the-counter (OTC) kudzu extract in the belief that it helps their cluster attacks. Kudzu's actual efficacy has not been studied. METHODS: A database of cluster headache patients was questioned about their use of various alternative remedies to treat their cluster headache. Of 235 patients identified, 16 had used kudzu, consented to interviews, and provided medical records. RESULTS: In total, 11 (69%) experienced decreased intensity of attacks, 9 (56%) decreased frequency, and 5 (31%) decreased duration, with minimal side effects. CONCLUSION: Anecdotal evidence suggests that a component in OTC products labeled as kudzu may prove useful in managing cluster headache. This hypothesis should be tested with a randomized clinical trial.

BACKGROUND: The authors review the epidemiology, clinical features, pathophysiology, diagnosis, treatment, orofacial presentations and dental implications of trigeminal autonomic cephalalgias (TACs): cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). TYPES OF STUDIES REVIEWED: The authors conducted PUBMED searches for the period from 1968 through 2007 using the terms "trigeminal autonomic cephalalgias,""cluster headache,""paroxysmal hemicrania,""short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing,""epidemiology,""pathophysiology,""treatment,""oral,""facial" and "dentistry." They gave preference to articles reporting randomized, controlled trials and those published in English-language peer-reviewed journals. RESULTS: TACs refers to a group of headaches characterized by unilateral head pain, facial pain or both with accompanying autonomic features. Although their pathophysiologies are unclear, CH, PH and SUNCT may be differentiated according to their clinical characteristics. Current treatments for each of the TACs are useful in alleviating the pain, with few refractory cases requiring surgical intervention. Patients with TACs often visit dental offices seeking relief for their pain. CLINICAL IMPLICATIONS: Although the prevalence of TACs is small, it is important for dentists to recognize the disorder and refer patients to a neurologist. This will avoid the pitfall of administering unnecessary and inappropriate traditional dental treatments in an attempt to alleviate the neurovascular pain.

A fixed location unilateral headache suggests involvement of a precise nervous structure, and neuroimaging investigations are essential to seek to identify it. Nevertheless, side-locked primary headaches also occur, although they are rare. Side-locked primary headaches are more frequently found in the group of the short-lasting (</= 4 hours) headaches but long-lasting headache forms may also present with the pain always on the same side, including migraine, tension-type headache, new daily persistent headache and cervicogenic headache. Future studies should address the issue whether patients with side-locked headache form differ from those with non-side-locked form both in terms of natural history and biological markers. Among 63 consecutive chronic cluster headache patients seen by us from 1999 to 2007, 32 (51%) had side shift. We also found that the duration of the chronic condition was significantly longer in those with side shift than those without. The high frequency of side shift in chronic cluster headache should be considered when proposing surgical treatment for severe intractable forms of the disease.

The short- and long-term effects of administration of lithium carbonate in cluster headache (CH) have been investigated. Of the 90 patients treated (78 males and 12 females), 68 had episodic CH and 22 had the chronic form of the disease. The doses used were almost always 900 mg/day. Eleven of the 22 patients with chronic CH showed a definite, constant improvement both short and long term. In 7 of the 22 patients, lithium treatment provided excellent results initially but was later followed by some transient worsening; in the remaining 4 only partial benefits were observed initially and treatment proved still less effective after a few months. The effects of cessation of lithium administration after at least five months of continuous treatment were studied in 9 cases. In 6 of them the attacks re-appeared immediately, whereas in 3 the attacks occurred again only after free intervals of four to six months. Of the 68 patients with episodic CH, 26 proved highly responsive to treatment, 26 only partially responsive, and 16 refractory. In 3 cases, after one to three years of continuous treatment, euthyroid goitre developed, which disappeared after the drug was discontinued.

The short and long-term effects of Lithium carbonate in 90 patients with episodic (68 cases) and chronic (22 cases) cluster headache are reported. The results are discussed in particular in relation to the response of the episodic forms, the development of tolerance, the ability of the substance to modify the natural history of the disease, and the side-effects from long-term treatment.

In this multicentric random double-blind study versus placebo, the therapeutic activity of timed-release dihydroergotamine preparation 10 mg/per os/day has been tested in 90 patients affected by common migraine. The drug presented a prophylactic effect on recurrent headache, mainly on night and awakening migraine. A low incidence of side-effects with no weight changes or other effects on physiologic events was registered.

The effects of flunarizine administration (10 mg/day, at bed time) were studied in 120 common migraine patients who were followed for 24 months with quarterly controls. Besides headache index (HI) and analgesic use, other variables were monitored, such as arousal (Tolouse Pieron test), mood (Hamilton rating scale for depression), sleep/wake (hrs) and body weight. The study was open-type and after the 6th month control some responder (R) cases (HI reduction greater than or equal to 60%) presenting HI scores less than or equal to 4 could continue the survey off-treatment. The percentage of R cases was 54.5% at the 3rd month, a figure that further increased up to 72% by the 9th month; relapses on treatment were not observed and rebound-headache occurred in 1/4 of R cases let off-treatment. Lower (p less than 0.05) baseline HI values characterized non-responders. Side-effects not requiring withdrawal were drowsiness (42% within the 1st month) and weight gain (mean 7.9 +/- 6.9 kg) in 54% of the cases, while a retarded type depression was the most frequent cause of drop-out from trial (7.5%). The results, while confirming the high prophylactic activity of flunarizine in common migraine, stress the importance of clinical long-term survey of side-effects using antimigraine drugs and suggest the need for further investigations about flunarizine effects on CNS.

A multicentre study with an open experimental design was carried out on 118 patients suffering from mild to moderate cognitive decline due to cerebrovascular and degenerative disorders (chronic cerebrovascular disorders, CCVD; multi-infarct dementia, MID; aging brain, AB; dementia of Alzheimer's type, DAT). All patients, after a wash-out period of 3 weeks, were treated with idebenone (45 mg twice daily by oral route) for a period of 6 months. Behavioral and cognitive measures (Sandoz Clinical Assessment of Geriatrics, SCAG; Serial Learning Test) were applied to evaluate the long term therapeutical aspects. The results, analyzed by multivariate analysis of variance and chi2 test, showed a significant improvement of the cognitive profile in all patients, more evident in CCVD and AB groups. No remarkable side-effects were found in all groups of patients, thus confirming good tolerability of idebenone.

One hundred and sixty-seven subjects (91 females and 76 males) aged 18 to 55 and suffering from recurrent headache, daily headache, and cluster headache, were studied. In order to collect detailed information about their clinical as well as social and environmental conditions, a multi-page card was used which had been specially prepared for this purpose. The patients' personality patterns were assessed by the following methods: MMPI (the Hs, Hy, D, and Ma scales, in particular); EPQ (Eysenck Personality Questionnaire); and AS IPAT (Cattel's Anxiety Scale). The statistical analysis of the data obtained made it possible to define the personality patterns of both male and female subjects and then to differentiate between them in relation to the various types of headache. Furthermore, possible relationships between personality traits and headache suffering were investigated. If the personality patterns of females seem to have existed prior to the disease, those of males raise some questions. In cluster patients, in particular, the age of onset seems to be related to certain personality traits; in patients with daily headache, by contrast the association between the duration and severity of the disorder appears to play a major trigger role. Some of these correlations do not have a linear character and suggest new working hypotheses that go beyond the limits of standard correlations.

The therapeutic response to L-5HTP, a serotonin precursor, was studied in an attempt to identify clinical subgroups of primary headache patients. The results at the 4th month control in 100 patients under L-5HTP treatment at the dose of 300 mg/day confirm previous observations on the activity profile of the drug, the effects of which are equally distributed between recurrent and daily forms as well as among clinical subtypes. The emerging profile of the responsive patients is characterized by such peculiar traits as prevalence of previous major mood disturbances and minor frequency of anxiety, longer duration of the illness and higher occurrence of some associated symptoms, lower incidence of exogenous and hormonal trigger factors, and previously positive response to pizotifen treatment.

Common migraine (CM) is an evolutive disease characterized by a progressive increase in the number of attacks and a consequent reduction in the free periods, eventually reaching a state of continuous migraine with interparoxysmal headache (MIH). To evaluate the role of central pro-opiocortin-related peptides in the pathogenesis of the disease, cerebrospinal fluid (CSF) levels of beta-lipotropin (beta-LPH), beta-endorphin (beta-EP) and ACTH were measured in two groups of migraine sufferers with increasing severity of the disease (CM and MIH), and in healthy controls. ACTH values were similar in the 3 groups, while beta-LPH levels were significantly lower (P less than 0.005) in patients affected by MIH (10.4 +/- 8.6 fmol/ml) than in patients with CM (35.7 +/- 8.3) and in controls (32.9 +/- 15.33). beta-EP levels were closely correlated with the severity of the disease: they decreased significantly from those found in healthy controls (86.1 +/- 37 fmol/ml) to those of CM sufferers (38.5 +/- 3.5; P less than 0.005) and showed a further significant fall (P less than 0.01) to the lowest levels which were found in MIH patients (14.8 +/- 9.8). These data showing that the progressive evolution of migraine is concomitant with a progressive impairment in the CSF levels of beta-EP, sustain the concept that non-organic central pain is related to a reduced activity of the neurons responsible for the CSF content of beta-EP.

OBJECTIVE To present results from the United States (US) Cluster Headache Survey including data on cluster headache demographics, clinical characteristics, suicidality, diagnostic delay, triggers, and personal burden. BACKGROUND There are few large-scale studies looking at cluster headache patients and none from the USA. This manuscript will present data from The US Cluster Headache Survey, the largest survey ever completed of cluster headache patients living in the USA. METHODS The total survey was composed of 187 multiple-choice questions that dealt with issues related to cluster headache including demographics, clinical characteristics, comorbid medical conditions, family history, triggers, smoking history, and personal burden. The survey was placed on a Web site from October through December 2008. RESULTS A total of 1134 individuals completed the survey (816 male, 318 female). Some key highlights from the survey include the following:(1) diagnostic delay: there remains a significant diagnostic delay for cluster headache patients on average 5+ years with only 21% receiving a correct diagnosis at time of initial presentation.(2) Suicidality: suicidal ideations are substantial, occurring in 55%.(3) Eye color: the predominant eye color in cluster headache patients is brown and blue, not hazel as suggested in previous descriptions.(4) Laterality: cluster headache has a right-sided predominance.(5) Attack profile: in US cluster headache sufferers, most attacks occur between early evening and early morning hours with peak time of headache onset between midnight and 3 am; the circadian periodicity for cluster headache is present but is not as predominant in the population as previously thought.(6) Triggers: beer is the most common type of alcohol trigger in US cluster headache patients; noted migraine triggers such as weather changes and smells are also very common cluster headache triggers.(7) Medical comorbidities: peptic ulcer disease does not have a high prevalence in US cluster headache patients as suggested by previous literature; cluster headache is associated with a low prevalence of cardiac disease as well as cerebrovascular disease even though the majority of patients are chronic heavy smokers. In US cluster headache sufferers, there appears to be comorbidity with restless leg syndrome, and this has not been demonstrated in non-US cluster headache populations.(8) Personal burden: cluster headache is disabling to the individual as almost 20% of cluster headache patients have lost a job secondary to cluster headache, while another 8% are out of work or on disability secondary to their headaches. CONCLUSION Some findings from the US Cluster Headache Survey expound on what is currently known about cluster headache, while some of the results contradict what has been previously written, while other information is completely new about this fascinating headache disorder.

Electrocardiographic changes mimicking an acute coronary event with T-wave inversion have been reported in the resting electrocardiogram in patients without ischemic heart disease but with acute ischemic stroke, or subarachnoid hemorrhage, or intracerebral hemorrhage, or a severe head injury. We present a case of T-wave inversion in a 73-year-old Italian woman admitted to the Emergency Department following a severe head injury. Pericarditis, pericardial effusion, and acute coronary event were excluded. Ischemic stroke, subarachnoid hemorrhage, and intracerebral hemorrhage were also excluded. Also this case focuses attention on T-wave inversion after a severe head injury without ischemic heart disease.

The purpose of our study was to identify general factors and distinctive clinical features differentiating patients with chronic cluster headache (CH) evolved from episodic CH and patients with episodic CH. Our study sample included 28 patients suffering from chronic CH evolved from episodic CH and 258 patients with episodic CH; all were referred to the Headache Center of Parma between December 1975 and June 1998. Patients with episodic CH were selected from all episodic CH referrals (n = 485) and selection was based on the duration of the disorder, which was to exceed the average period needed for an episodic form to turn into a chronic form (4.5 years for females and 7.0 years for males). At CH onset, the mean age for patients with chronic CH evolved from episodic CH was older than for those with episodic CH. Among patients with chronic CH, more were smokers or heavy drinkers, and had suffered a head injury. Clinically, episodic CH evolving into chronic CH was characterized by a high frequency of cluster periods, a larger proportion of patients with attacks not occurring strictly within cluster periods, and remission periods lasting less than 6 months. Possible predictive factors in the development of chronic CH appear to be CH onset from the third decade of life onward, the occurrence of more than one cluster period a year, and the short-lived duration of remission periods. The role played by head injury and cigarette smoking in the evolution of the disorder still cannot be established with certainty.

AIM: The study of clinical running of gastric or duodenal ulcer in associated coronary heart disease (CHD). MATERIALS AND METHODS: 209 CHD patients with gastric ulcer (GU) or duodenal ulcer (DU) were examined clinically plus histological examination of gastric or duodenal mucosa biopsies was made. RESULTS: In CHD patients GU occurred more frequently (56%) than DU. The lesions involved more frequently lesser curvature of the stomach and pyloric part of the stomach. Males developed ulcers 3.5 times more frequently than females. Ulcers tended to a painless course without season exacerbations. The disease manifested first with gastric bleeding in 52% of the patients. GU and DU ran with frequent recurrences and long-term exacerbations (76% of patients) which coincided in time with CHD exacerbations. 68% of patients developed exacerbations within 10 days after myocardial infarction or aortocoronary bypass operation. Helicobacter pylori was present as a resolving factor in arising ulcer in 26% of patients. Microcirculatory disorders, reduced blood flow speed in gastric or duodenal mucosa, hypocoagulation syndrome, dyslipidemia provoked exacerbations in 62% of patients. Examinations of biopsies from gastric and duodenal mucosa showed marked dystrophic changes in the mucosa, its connective tissue basis in the vessels in the presence of mild inflammation at ulcer site. CONCLUSION: The onset of ulcers and erosions in the mucosa of the gastrointestinal tract in CHD may be due to circulatory disorders in gastric mucosa. The main factors of aggression are hypoxia, hypoxia-induced trophic defects in gastric and duodenal mucosa, circulatory disorders.

The cause of R-wave amplitude changes in the ECG under load remain unknown. One of the possible aetiological factors is myocardial ischaemia. The aim of the present study was to investigate possible relationships between amplitude changes and transient myocardial ischaemia. In this retrospective study, 89 patients (74 men, 15 women) were investigated. The results of exercise ECG, a dipyridamole test and coronary angiography were available for all patients participating. The patients were divided into four groups (group I: patients with relevant coronary stenosis [n = 46], group II: patients with no relevant coronary stenoses [n = 43], group III: patients with pathological results in all three procedures [n = 29], and group IV: patients with unremarkable findings in all three investigations [n = 14] and the R-wave amplitude changes in various phases of ergometry and dipyridamole provocation were measured. All four groups showed a significance decrease in R-wave amplitude at maximum provocation as compared with baseline values. A qualitative analysis of the amplitude changes indicated a tendency for them to be dependent on the severity of coronary stenosis. A quantitative analysis, however, revealed no significant correlations between the R-wave amplitude and ST segment changes or angiographic findings. This means that R-wave changes during an exercise ECG or a dipyridamole test do not permit us to distinguish between patients with or those without coronary artery stenosis. Changes in the R-wave amplitude observed under loading must be due to factors other than myocardial ischaemia.

This study examined the thesis that patients with coronary heart disease (CHD) comprise discrete personality subtypes and that identification of these subtypes may benefit biobehavioral research on CHD. Measures of Negative Affectivity, Social Inhibition, and Self-Deception were used to generate a personality taxonomy through cluster analysis in a sample of 405 men with CHD. This empirical taxonomy served as a basis for the development of a conceptual model which delineates hardy, distressed, inhibited, and repressive subtypes. Coronary proneness may differ across these subtypes to the extent that potential behavioral correlates of CHD were associated with subtype membership. Distressed individuals (characterized by elevated levels of Type A behavior, anger, hostility, and life stress) and inhibited individuals (characterized by the nonexpression of anger) may be particularly coronary prone. It is concluded that research should focus on the interaction of global traits that may define subtypes of patients with CHD.

We present the case of a 38-year-old woman with no previous history of coronary heart diseases, who suffered from cluster headaches and had undergone a subcutaneous sumatriptan treatment for two years. With no previous history of underlying ischaemic heart diseases or Prinzmetal's angina or any other significant coronary diseases supported by an angiographic exam, the patient suffered from an episode of coronary vasospasm following a subcutaneous administration of sumatriptan. Therefore particular care should be placed when subcutaneous sumatriptan is administered to patients who experienced chest pain or other related symptoms after the use of the drug.

Among the diseases or symptoms most frequently associated with stress are peptic ulcers, coronary heart disease (CHD), high blood pressure, backaches, and breathing disabilities. Many who have not experienced these severe symptoms have had milder ones, such as tension, cold hands, colds, headaches, tight muscles, and irritability, or feelings of disorganization, apathy, boredom, dissatisfaction, and even moderate levels of hostility. Although unpleasant, many people accept these as the cost of doing business or simply living. This need not be the case. If the mild symptoms are managed, more severe ones like CHD and ulcers may be avoided altogether. However severe or mild the symptoms, the important question is:"How can the symptoms be managed and even eliminated?" The answer is to become aware of stress and to engage in stress management strategies. These strategies are addressed here in some detail.

We report a His-bundle tachycardia in a 6 year-old patient without evidence of heart disease. Diagnosis and follow-up were assessed by exercise stress testing and repeated long-term electrocardiographic recording. As sarcoidosis was present in the mother and the aunt, this condition was discussed in this child also. However, since there is no absolute proof for this disease, the hypothesis of an antenatal injury of the bundle of His was discussed: this could be secondary to the transplacental transfer of abnormal maternal antibodies, as frequently observed in women with clinical or biologic evidence of connective tissue disease.

A total of 586 men who survived an initial attack of unstable angina or myocardial infarction have been followed for up to 15 years. A policy of early mobilization and sustained risk factor advice was employed. A conservative approach to treatment was adopted during the acute and follow-up stages. Drugs were employed only for symptomatic reasons, and only two patients proceeded to coronary artery bypass surgery. Survival at 5, 10, and 15 years was 80%, 61%, and 43%. Older patients and those with more severe initial attacks had a higher mortality, but these factors did not relate to combined fatal and nonfatal recurrence of myocardial infarction. Of 22 studies reviewed, 18 report a higher mortality than does our study. Four studies, none strictly comparable, report a similar 5-year mortality. A conservative approach to management does not appear to be harmful and may be beneficial.