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Undertreatment in patients with primary headaches was evaluated in 600 patients attending 7 headache centres in Lombardy by assessing the rates of acute and prophylactic treatments used before the first visit and the rates of prescription of acute and prophylactic treatments after the visit at the headache centre. Our results clearly showed that most headache patients are likely to receive suboptimal treatments, confirming the utility of headache centres as well as the need for promoting education of GPs and the development of appropriate networks to reduce undertreatment rates, in order to highlight the negative impact caused by primary headache on individuals and on the society.

Stress induced by the events of daily life is considered a major factor in pathogenesis of primary tension-type headache. Little is known about the impact that could have a more stressful event, like a natural disaster, both in patients with chronic headache, both in people that do not had headache previously. The aim of the present study was to observe the prevalence of headache in the population following the devastating earthquake that affected the province of L'Aquila on April 6, 2009. The study population was conducted in four tent cities (Onna, Bazzano, Tempera-St. Biagio, Paganica). Sanitary access is recorded in the registers of medical triage, in the first 5 weeks, after the April 6, 2009. The prevalence of primary headache presentation was 5.53% (95% CI 4.2-7.1), secondary headache was 2.82% (95% CI 1.9-4.9). Pain intensity, assessed by Numerical Rating Scale score showed a mean value of 7±1.1 (range 4-10). The drugs most used were the NSAIDs (46%) and paracetamol (36%), for impossibility of finding causal drugs. This study shows how more stressful events not only have an important role in determining acute exacerbation of chronic headache, but probably also play a pathogenic role in the emergence of primary headache. Also underlines the lack of diagnostic guidelines or operating protocols to early identify and treat headache in the emergency settings., (© The Author(s) 2011. This article is published with open access at Springerlink.com)

Hemicrania continua (HC) is a rare primary headache syndrome, characterized by unilateral pain and an absolute response to indometacin. Since the term was first coined in 1984, more than 100 cases have been described worldwide. Most recently, detailed case series that provide more detailed information concerning the sometimes complex clinical presentation of HC have been reported. Functional imaging studies suggest a unique pattern of subcortical involvement in HC: contralateral to the pain posterior hypothalamic region, ipsilateral dorsal pons and ipsilateral ventral midbrain, which, along with the particular effect of indometacin, probably justifies its classification as a unique entity. Increasing the awareness of this primary headache form among clinicians will aid in its diagnosis while further work is being undertaken to characterize the syndrome.

Background: Hypnic headache (HH) is a rare primary headache disorder that is characterized by exclusively sleep-related headache attacks. Because of its low prevalence, clinical features and therapeutic options are widely unknown or under discussion., Methods: Twenty patients with HH were examined and interviewed using a standardized questionnaire in regard to their clinical characteristics and effective treatment regimens. Data were evaluated according to current International Headache Society (IHS) diagnostic criteria. Individual treatment history and effective treatment options were compared with expected efficacy based on current literature., Results: In conflict to current IHS criteria, 15% of patients reported trigemino-autonomic symptoms. All patients showed distinct motor behavior during their headache attacks. In acute pain attacks caffeine was most effective. Lithium, topiramate, melatonin, amitriptyline and indomethacin were sometimes useful prophylactic treatment options but were often associated with side effects., Conclusions: Our results underline the need for modification of the IHS diagnostic criteria of HH to better reflect the actual clinical characteristics of this headache. Caffeine should be considered as first-line acute therapy. Prophylactic medical treatment should be carefully evaluated in regard to side effects in this aged patient population, as this seems to be a major concern of patients apart from pure pain reduction.

Introduction: Nummular headache is a rare primary headache disorder described by a focal circumscribed area of pain (2-6 cm in diameter). Literature on this disorder is sparse., Patients and Methods: Here, we describe a case series of 16 patients (6 men, 10 women) seen at the Mayo Clinic., Results: Mean age of onset was 50 years (range, 19-79 years) and mean duration of headache was 7.9 years (range, 0.33-40 years). Location of headache varied and was found to be an average of 3.9 cm in diameter (range, 2-10 cm). Headache was episodic (< 15 days/month) in four patients and chronic (>15 days/month) in 12 patients. Attention was paid to therapeutic interventions. Resolution was seen in 38% of patients. Migraine was present in the history of 56% of patients and medication overuse headache was found in 25%., Conclusions: Our series results support previous findings. In our population, no specific therapy was identified to be effective in more than one patient.

Primary cough headache, primary exertional headache and primary headache associated with sexual activity are distinct entities, even though they share several features: acute onset, the absence of structural brain disease and exertional factors as precipitating events. In this short review, we illustrate the possible treatment strategies on the basis of information collected from a systematic analysis of the international literature.

Objectives: In subjects with primary headaches, rhinologic pathologic condition may be associated with treatment refractoriness. In some cases, surgical correction of intranasal pathologic condition may decrease medication use. We aimed to evaluate the benefits of a rhinologic perspective in primary headache subjects by using neurologic management., Methods: Subjects with primary headache were examined by a neurologist and otolaryngologist. Initially, neurologic assessment was made, and medication was started. Migraine symptoms and pain severity were recorded using the Migraine Disability Assessment Scale and a 0 to 10 visual analog scale (VAS). Subject's pain severity of tension type headache was evaluated by VAS. Direct otorhinolaryngologic history of all primary headache subjects was taken, and they all underwent physical examination, rigid nasal endoscopy, sinus computed tomography, and mucosal contact point test. All examination and radiologic findings were noted. Subjects were separated into groups after 1-month medical neurologic management., Results: One hundred nine subjects were enrolled. Ninety-nine subjects completed follow-up. Seventy-eight percent of the subjects were women. The mean age was 33.6 years (range, 18-63 y). Twenty-six subjects had no significant intranasal pathologic condition (group 1). Twenty subjects had an intranasal pathologic condition but responded to the neurologic treatment (group 2). Fifty-three subjects had an intranasal pathologic condition, and the neurologic treatment failed for these 53 subjects. Surgical intervention was planned for these 53 subjects (group 3). Thirty-eight subjects accepted the operation (group 3a), and 15 subjects refused the surgical intervention (group 3b). All subject's Migraine Disability Assessment Scale and VAS scores were compared. A total of 73 subjects had rhinologic abnormalities. Groups 1 and 2 benefited from the neurologic treatment, but headache severity of group 3a reduced after rhinologic surgery. Group 3b who rejected surgical intervention did not respond to the neurologic treatment., Conclusions: This study describes a series of subjects presenting with various primary headaches who also have underlying rhinologic abnormalities. Surgical treatment of the underlying rhinologic pathologic abnormalities had a beneficial effect on headache.

Hypnic headache is a rare primary headache disorder that is characterized by exclusively sleep-related headache attacks in patients usually beyond 50 years of age. Only 174 cases have been reported in the literature so far. Owing to its low prevalence, clinical features and therapeutic options, as well as underlying pathophysiologic mechanisms, are widely unknown or being controversially discussed. The association of hypnic headache with rapid-eye movement sleep was strongly contradicted in recent articles, while an association with hypothalamic dysfunction is thought to be one important pathophysiological mechanism. Common acute, as well as prophylactic, treatment is caffeine intake, either in the form of a cup of coffee or a caffeine tablet. Lithium, indomethacin and melatonin were promoted as potent medical treatments, while almost every other drug commonly used to treat headache or other pain syndromes was reported to be effective in single case reports on hypnic headache.

Hypnic headache (HH) is a primary headache disorder, which occurs exclusively during sleep and usually begins after 50 years of age. There are no controlled trials for the treatment of HH. We reviewed all the available papers, including 119 cases published in literature up to date, reporting the efficacy of the medications used to treat HH. Acute treatment is not recommended, since no drug proved to be clearly effective and also because the intensity and the duration of the attacks do not require the intake of a medication in most cases. As for prevention, a wide variety of medications were reported to be of benefit in HH. The drugs that were found to be effective in at least five cases are: lithium, indomethacin, caffeine and flunarizine. Lithium was the most extensively studied compound and demonstrated to be an efficacious treatment in 32 cases. Unfortunately, despite its efficacy, significant adverse effects and poor tolerability are not rare, mainly in elderly patients. Many patients reported a good response to indomethacin, but some could not tolerate it. Caffeine and melatonin treatments did not yield robust evidence to recommend their use as single preventive agents. Nevertheless, their association with lithium or indomethacin seems to produce an additional therapeutic efficacy. A course of lithium should be tried first, followed 3-4 months later by tapering. If headache recurs during tapering, a longer duration of therapy may be needed. If lithium treatment does not provide a significant response, indomethacin can be commenced as second-line approach. If these treatments prove to be ineffective or poorly tolerated, other agents, such as caffeine and melatonin, can be administered.

We describe a 23-year-old male patient who presented with spontaneous intermittent and increasing attacks of severe, left-sided thunderclap headache combined with rapidly progressive muscle weakness and dysphasia, including gradual loss of consciousness. Subsequent CT, MRI and DSA showed progressive brain ischaemia and oedema within the left cerebral hemisphere with strict ipsilateral segmental arterial vasoconstriction. Despite extensive medical care, including steroids, the patient deteriorated rapidly. However, the clinical course changed dramatically within 15 h after the start of an intravenous infusion of prostacyclin at a dose of 0.9 ng/kg/min, with an almost complete recovery of consciousness and speech. In addition the pathophysiological alterations seen on magnetic resonance (imaging and digital) subtraction angiography including diffusion-weighted imaging and apparent diffusion coefficient maps shortly before prostacyclin treatment were clearly reduced when the patient was examined 3-4 days later and he continued to recover thereafter. Although not fully compatible, our case had several clinical characteristics and radiological findings reminiscent of those of the 'segmental reversible vasoconstriction syndrome', sometimes called the Call-Fleming syndrome.

The coexistence of hemicrania continua with another primary headache disorder is a very rare event. We report a male patient with both hemicrania continua of 16- year duration and pre-orgasmic headache of three-year duration. Both headache disorders responded to indomethacin. The patient had also in addition persistent elevation of fasting serum insulin.

Headache is the third leading cause of referral to a pediatric emergency room. It is imperative for providers to be able to rule out rare but possible life-threatening disorders, such as meningitis, intracranial hemorrhage, brain tumor, or hydrocephalus. Most of the presenting headaches are secondary to viral illnesses followed by primary headache and migraine. A detailed initial evaluation is essential to guide toward necessary testing as well as diagnosis., (Copyright 2010 Elsevier Inc. All rights reserved.)

Primary stabbing headache is an ultra-short headache, associated with primary headaches, more prevalent in women and with a poor response to therapy. The effect of botulinum neurotoxin type-A (BoNTA) on primary stabbing headache was investigated in 24 patients. Three patients showed complete remission. Nineteen patients showed a decrease in their primary stabbing headaches that started in the second week, and that was sustained during approximately 63 days. In two patients BoNTA showed no therapeutic effect. The BoNTA seems to be an excellent therapeutic option for primary stabbing headache.

Primary stabbing headache (PSH) is a short-lasting but troublesome headache disorder, which has been known for several decades. The head pain occurs as a single stab or as a series of stabs generally involving the area supplied by the first division of trigeminal nerve. Stabs last for approximately a few seconds, occurring and recurring from once to multiple times per day in an irregular pattern. For the diagnosis of PSH, it is mandatory that any other underlying disorder is ruled out. Indomethacin represents the principal option in the treatment of PSH, despite therapeutic failure in up to 35% of the cases. Recent reports showed that cyclooxygenase-2 (COX-2) inhibitors, gabapentin, nifedipine, paracetamol and melatonin may also be effective. In this report, we focus on the therapy of PSH summarizing the information collected from a systematic analysis of the international literature over the period 1980-2009.

Distinguishing primary headache from secondary headache is the first objective of every new clinical encounter with a patient complaining of headache. The history is king in headache medicine-90% of patients presenting with headache have a primary headache disorder and the examination is normal. The history must be elicited because patients will not always volunteer seminal information. A standard series of questions must be asked of each patient to guide an appropriate diagnostic evaluation and ensure that secondary causes are not overlooked. The second objective, of course, is making the correct diagnosis of the primary headache disorder. Although at first glance this appears obvious and almost patronizing, making the correct diagnosis is often not a priority, nor is it a process that is emphasized in undergraduate and postgraduate training programs. Knowing some simple rules and standard questions will make the process almost fail proof., ((c) Thieme Medical Publishers.)

Background: A 33-year-old woman experienced a thunderclap headache immediately postpartum. The headache recurred over the next 10 days, and the patient also developed generalized tonic-clonic seizures. A subarachnoid hemorrhage was demonstrated on a head CT scan., Investigations: Physical examination, laboratory tests, brain CT scan, brain MRI scan, brain magnetic resonance angiogram, brain magnetic resonance venogram, cerebral angiography, transcranial Doppler ultrasound, EEG., Diagnosis: Postpartum cerebral angiopathy., Management: NSAIds and opioid analgesics administered on postpartum day 3 provided partial headache relief. The next day, a blood patch was performed and intravenous caffeine, fluid and opioid analgesia were given to treat a suspected dural puncture headache. Following diagnosis of postpartum cerebral angiopathy on postpartum day 10, nimodipine was initiated to treat the vasospasm, and the headache was treated with opioid analgesics and toradol, followed by naproxen. The patient also received a 3-day course of intravenous magnesium sulfate.

Aim: To determine the prevalence and the clinical-epidemiological and therapeutic characteristics of primary headaches in a rural population in Cuba., Subjects and Methods: A cross-sectional study was conducted in a rural population of the Policlinica Mabay in the province of Granma, Cuba. The second International Headache Society classification was used. Sample size, which was calculated by means of the program EpiInfo 2002, was 360 adult workers over 18 years of age, who were selected by simple random sampling., Results: The prevalence of primary headaches was 44.72%. There was a predominance of females (71.4%). 31.1% of cases were between 50 and 59 years old. 36.6% had a family history of headaches. Onset of headaches occurred before the age of 15 years in 44.1% of patients. The most frequent location was occipital (45.3% of cases). The most frequent precipitating factors were stress (27.9%) and menstruation (27.3%). The predominant accompanying symptom was dizziness (26.1%). Tension-type headache was the most prevalent, with 92 patients (25.56%), followed by migraine, with 61 diagnoses (16.94%). 78.3% of the patients with tension-type headache and 70.5% of those with migraine tried self-medication with dipyrone and paracetamol. Patients with migraine made more frequent use of prophylactic treatment than those who suffered from tension-type headache (32.8% versus 16.3%; p = 0.01)., Conclusions: The prevalence of primary headaches is high in the rural population under study; the high rate of prescription drug abuse (mainly painkillers and non-steroidal anti-inflammatory drugs) has led to a significant number of headaches becoming chronic. This health problem is also seen to have an important effect on occupational affairs.

Nummular headache (NH) has been defined as a focal head pain that is exclusively felt in a small area of the head surface. Here we describe three patients who presented with focal head pain in two separate areas. This finding seems to be consistent with bifocal NH and further enlarges the clinical diversity of this headache disorder. The pathogenic mechanisms of NH may be active in multiple cranial areas in some particular patients.

Fibromyalgia syndrome (FMS) is a chronic pain condition of unknown aetiology characterized by diffuse pain and tenderness at tender points. The aim of the study was to assess the prevalence and clinical features of FMS in the different forms of primary headaches, in a tertiary headache centre. Primary headache patients (n = 217) were selected and submitted to the Total Tenderness Score, anxiety and depression scales, Migraine Disability Assessment, allodynia questionnaire, Short Form 36 Health Survey and the Medical Outcomes Study-Sleep Scale. In patients with FMS, the Multidimensional Assessment of Fatigue, the Pain Visual Analog Scale, the Manual Tender Point Survey and the Fibromyalgia Impact Questionnaire were employed. FMS was present in 36.4% of patients and prevailed significantly in tension-type headache and in patients with higher headache frequency. Headache frequency, pericranial muscle tenderness, anxiety and sleep inadequacy were especially associated with FMS comorbidity. In the FMS patients, fatigue and pain at tender points were significantly correlated with headache frequency. FMS seems increasingly prevalent with increased headache frequency, for the facilitation of central sensitization phenomena favoured by anxiety and sleep disturbances.

Background: Diversity of treatments used for headache, and varied quality of research conduct and reporting make it difficult to accurately assess the literature and to determine the best treatment(s) for patients., Objectives: To compare the quality of available research evidence describing the effects and outcomes of conventional, and complementary and alternative medicine (CAM) approaches to treating primary (migraine, tension, and/or cluster-type) headache., Study Design: A systematic review of quality of research studies of conventional and alternative treatment(s) of primary headache., Methods: Randomized, controlled clinical trials (RCTs) of treatment(s) of chronic primary headache (in English between 1979 to June 2004) were searched through MEDLINE, PsycInfo, EMBASE, Cochrane Library, and the NIH databases. Studies were evaluated using standard approaches for assessing and analyzing quality indicators., Results: 125 studies of conventional, and 121 CAM treatments met inclusion criteria. 80% of studies of conventional treatment(s) reported positive effects (p<0.05), versus 73% of studies of CAM approaches (chi(2) = 3.798, 1 df, p=0.051). Overall, the literature addressing the treatment of primary headache received a mean Jadad score of 2.72 out of 5 (SD 1.1). The mean Jadad score for studies of conventional therapeutics was significantly better than for those studies of CAM approaches: 3.21 +/- 0.9 vs 2.23 +/- 1.1 (t=7.72, 246 df, mean difference 0.98, p < 0.0005)., Conclusions: Studies of conventional treatments scored higher on reporting quality than studies of CAM approaches. It is possible that these differences may reflect distinctions in 1) methodologic integrity, 2) therapeutic paradigm(s), and/or 3) bias(es) in the approach(es) used to evaluate certain types of therapies. Each of these possibilities -- and the implications -- is addressed and considered.

Hemicrania continua (HC) is a primary headache disorder characterized by a continuous, unilateral headache that varies in intensity, waxing and waning without disappearing completely. Ipsilateral cranial autonomic features and response to indomethacin are essential features for the diagnosis of HC. We hereby, describe three patients with the clinical phenotypes of HC in whom response to indomethacin was either incomplete or not sustained. We also review the literature especially for the presence of indomethacin response and ipsilateral cranial autonomic features.

A 67-year-old male and a 43-year-old female were referred to hospital with headaches after intracranial pressure increasing activities, such as coughing. Both patients were diagnosed with primary cough headache. In the case of the first patient, this diagnosis was considered shortly after presentation. His headache disappeared with the standard treatment for primary cough headache, consisting of indomethacin and a proton pump inhibitor. The second patient received medicinal and surgical treatment for rhinosinusitis. These treatments did not improve the headache symptoms. Eventually she was also diagnosed with primary cough headache, and became pain-free with indomethacin and a proton pump inhibitor. Primary cough headache should be differentiated from secondary cough headache, in which the symptoms are caused by structural abnormalities of the brain. Additional investigation is required to differentiate between the two. The diagnosis of primary cough headache is supported by a positive reaction to trial treatment with indomethacin.

Reversible cerebral vasoconstriction syndrome (RCVS) usually presents with recurrent thunderclap headaches and is characterized by multifocal and reversible vasoconstriction of cerebral arteries that can sometimes evolve to severe cerebral ischemia and stroke. We describe the case of a patient who presented with a clinically typical RCVS and developed focal neurological symptoms and signs despite oral treatment with calcium channel blockers. Within hours of neurological deterioration, she was treated with intra-arterial milrinone, a phosphodiesterase inhibitor, which resulted in a rapid and sustained neurological improvement.

Hypnic headache (HH) is a rare sleep-associated primary headache disorder that usually begins after the age of 60 years. Here we report a 19-year-old male with 4-year history of predominantly left sided HH. He is the youngest person reported who fulfills the IHS diagnostic criteria for HH. The patient had history of relapsing-remitting course. The headache occurred every night at a constant time in each relapse. It was non-throbbing, moderate to severe, for 30 min to 5 h, and usually after 3 h of sleep. The patient showed complete response to indomethacin (75 mg at bedtime). Frequent tapering of indomethacin was required to look for the remission phase.

We report a case of Call-Fleming syndrome complicating orgasmic thunderclap headache. Recognizing this benign condition that mimics symptoms of subarachnoid hemorrhage prevents unwarranted invasive investigations and interventions that may cause harm.

Some patients suffering from headache require neurologic examination. The objective of the guidelines for the treatment of headaches is to help physicians in their daily care for headache patients. In most patients, the diagnosis of migraine has not been made by a physician and they have not received appropriate care to treat migraine attacks. New therapeutic methods (for acute and preventive treatment) have been introduced in the past fifteen years. Triptans should be offered to patients that fail to respond to usual analgesics, those with moderate to severe migraine in particular. Depending on comorbidity, preventive therapy should be recommended to individuals with frequent or prolonged migraine attacks. In patients with tension headaches, organic causes underlying the headache should be ruled out, while the treatment includes pharmacological and non-pharmacological measures. Although rare, patients with cluster headaches suffer severe pain; oxygen inhalation or triptans are recommended for acute attack, and preventive therapy may be indicated in some cases. The guidelines provide classification, diagnostic criteria and therapeutic principles for primary headaches. All recommendations listed in the guidelines are based on meta-analyses and recommendations from the world literature, with special reference to therapeutic options available in Croatia.

Hypnic headache is rarely reported in Asians, and the proposed International Classification of Headache Disorders (ICHD)-2 criteria have never been field-tested. We studied 17 consecutive Taiwanese patients (M/F: 9/8, mean age at onset 69.6 years) with hypnic headache from a headache clinic. Fifteen patients (88%) reported >15 headache attack days per month. Polysomnography studies done on 11 patients recorded 12 attacks in seven patients: two during rapid eye movement (REM) sleep, three during non-REM sleep and two having both. Five of the seven patients reported their headache profile during polysomnography studies. The clinical course was mostly episodic without recurrence (n = 9, 53%), followed by relapsing/remitting (n = 5) and chronic (n = 3). The ICHD-2 criteria were not fulfilled in 35% (6/17) patients based on patient recall or 60% (3/5) patients based on direct questioning during polysomnography studies. The major reason was the presence of pulsatile rather than dull headache in our patients. Unlike previous studies, our study showed hypnic headache occurred equally in both REM and non-REM sleep, and most patients ran an episodic course.

Chronic headache is still a frequent problem in old age, affecting about 10% of all women and 5% of all men older than 70 years. The incidence of primary headache decreases with advancing age, while that of secondary headache increases. The clinical characteristics of migraine can also change with age; for example, vegetative symptoms are less prominent, and less intense migrainous pain localized predominantly in the neck is frequently reported. Migraine aura can also be experienced more frequently in isolation, without a headache. Hypnic headache is a rare primary headache syndrome that occurs almost exclusively in the elderly. Most of the secondary headache syndromes that occur more frequently in old age present clinically as tension-type headache. Examples of rather common reasons for secondary headache syndromes in the elderly are intracranial space-occupying lesions, ophthalmological problems and autoimmune diseases such as giant cell arteritis. Elderly patients are especially likely to have a number of illnesses at any one time for which they take various medications each day, so that headaches can also quite often be caused by their medication or by withdrawal of these. As a result of such multimorbidity the homeostasis is disturbed in such patients, leading to various conditions that can entail concomitant headaches (sleep apnoea syndrome, dialysis headache, headache attributed to arterial hypertension or hypothyroidism). Familiar facial neuralgias, such as trigeminal neuralgia or postherpetic neuralgia following manifest herpes zoster affecting the face, become markedly more frequent with age. In general, in the treatment of headaches in the elderly it is essential to pay careful attention to potential interactions with the multiple drugs needed because of other diseases; in addition, the comorbidities themselves have to be taken into account, especially depression, anxiety and cognitive impairment, necessitating multimodal, interdisciplinary therapy plans.

This is a clinical report of a 63-year-old woman, with a 3-year history of severe episodes of hypnic headache responding to low-doses of topiramate (25 mg at bedtime). Topiramate has been used at the dosage of 100 mg/day for hypnic headache prevention in one recent case report with benefit. This report confirms the efficacy of topiramate in hypnic headache even using low-dose regimen therapy.

In order to analyze the clinical features, approach and treatment of patients with acute primary headaches seen at the Clinics Hospital of the Federal University of Uberlândia (HC-UFU) throughout 2005, the medical charts of 109 patients were evaluated through a standardized questionnaire as to age, gender, main diagnosis, characteristics of the headache attacks, diagnostic tests and treatment. Probable migraine was the most common type of primary headache (47.7%), followed by probable tension-type headache (37.6%), unspecified headache (11.9%), and headache not elsewhere classified (2.8%). As to characteristics of the crisis, the location of the pain was described in 86.2% of the patients. The most commonly used drugs for treatment of acute headache attacks were dipyrone (74.5%), tenoxicam (31.8%), diazepam (20.9%), dimenhydrate (10.9%), and metochlopramide (9.9%). The data collected are in agreement with those reported in literature. In most cases, treatment was not what is recommended by consensus or clinical studies with appropriate methodology. Therefore, we suggest the introduction of a specific acute headache management protocol which could facilitate the diagnosis, treatment and management of these patients.

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the association of severe headaches with or without additional neurological symptoms and a 'string and beads' appearance on cerebral arteries, which resolves spontaneously in 1-3 months. We present the clinical, neuroimaging and outcome data of 67 consecutive patients prospectively diagnosed over 3 years in our institution with an angiographically confirmed RCVS. There were 43 females and 24 males with a mean age of 42 years (19-70). RCVS was spontaneous in 37% of patients and secondary in the 63% others, to postpartum in 5 and to exposure to various vasoactive substances in 37, mainly cannabis, selective serotonin-recapture inhibitors and nasal decongestants. The main pattern of presentation (94% of patients) was multiple thunderclap headaches recurring over a mean period of 1 week. In 51 patients (76%), headaches resumed the clinical presentation. Various complications were observed, with different time courses. Cortical subarachnoid haemorrhage (cSAH) (22%), intracerebral haemorrhage (6%), seizures (3%) and reversible posterior leukoencephalopathy (9%) were early complications, occurring mainly within the first week. Ischaemic events, including TIAs (16%) and cerebral infarction (4%), occurred significantly later than haemorrhagic events, mainly during the second week. Significant sex differences were observed: women were older, had more frequent single-drug exposure and a higher rate of stroke and cSAH. Sixty-one patients were treated by nimodipine: 36% had recurrent headaches, 7% TIAs and one multiple infarcts. The different time courses of thunderclap headaches, vasoconstriction and strokes suggest that the responsible vasospastic disorder starts distally and progresses towards medium sized and large arteries. No relapse was observed during the 16 +/- 12.4 months of follow-up. Our data suggest that RCVS is more frequent than previously thought, is more often secondary particularly to vasoactive substances, and should be considered in patients with recurrent thunderclap headaches, cSAH or cryptogenic strokes with severe headaches.

Primary stabbing headache (PSH) is a short-lasting but troublesome headache disorder which has been known for several decades. We surveyed and registered consecutive patients with PSH in a headache clinic in Taiwan. A total of 80 patients (24 M/56 F, 53.2 +/- 16.2 years) were enrolled in our study. Migraine was reported in 20 (25%) patients and was less common in those with PSH onset at >50 years than those with onset at <50 years (14% vs. 38%, P = 0.02). The headache was unilateral in 59% of the patients and always in a fixed area in 36%. The head pain frequently involved extratrigeminal regions (70%) and in 30 patients (38%) was accompanied by jolts, i.e. head or body movements. Indomethacin was effective (74%) in patients who received it. Our study showed primary stabbing headache was a common and easily treated headache disorder in headache clinic. However, 70% of our patients could not fulfil criterion A 'exclusively or predominantly in the distribution of the first division of the trigeminal nerve' and 15% could not fulfil criterion C 'no accompanying symptoms' of the International Classification of Headache Disorders-II criteria proposed for PSH.

Hypnic headache is a rare form of primary headache with attacks occurring exclusively with sleep. As it is typical of the elderly, a wide range of alternative diagnosis must be considered. We present a case report of a 54-year-old woman with unsuspected secondary hypnic headache that was relieved by anti-hypertensive therapy. We reviewed the literature to evaluate the usual diagnostic workup performed in hypnic headache patients. We suggest that 24 h ambulatory blood pressure monitoring may be included in the evaluation of nocturnal headache complaints especially in the elderly, in whom essential hypertension is a very frequent comorbidity.

Background: Dipyrone is used to treat headaches in many countries, but is not available in others (particularly the USA and UK) because of its association with potentially life-threatening blood dyscrasias such as agranulocytosis., Objectives: To determine the effectiveness and safety of dipyrone for acute primary headaches in adults and children., Search Strategy: We searched the Cochrane Pain, Palliative & Supportive Care Group's Trials Register; the Cochrane Central Register of Controlled Trials; MEDLINE; EMBASE; LILACS, and the reference lists of included studies., Selection Criteria: Double-blind randomised controlled trials of dipyrone for the symptomatic relief of acute primary headaches in adults and children., Data Collection and Analysis: Three authors independently screened articles, extracted data, assessed trial quality and analysed results. Relative risks (RRs), risk differences (RDs), weighted mean differences (WMDs), and numbers-needed-to-treat (NNTs) were calculated as appropriate., Main Results: Four trials involving a total of 636 adult subjects were included. Methodological quality was generally high. One study each evaluated oral and intravenous dipyrone for episodic tension-type headache (ETTH); two trials evaluated intravenous dipyrone for migraine, but only one of these described pain outcomes. No pediatric trials were identified. The largest trial (n = 356) evaluated two doses (0.5 g, 1 g) of oral dipyrone for ETTH, which were significantly better than placebo for pain relief. The 1 g dose was also significantly better than acetylsalicylic acid (ASA) 1 g . A smaller trial (n = 60) evaluated intravenous dipyrone 1 g versus placebo for ETTH. RRs were statistically significant favouring dipyrone for pain-free and headache improvement outcomes. Finally, one trial (n = 134) evaluated intravenous dipyrone 1 g versus placebo for pain outcomes in patients with migraine. RRs were again statistically significant favouring dipyrone for pain-free and headache improvement outcomes. Two of the four trials assessed adverse events. No serious adverse events were reported, and no significant differences in adverse events were detected between dipyrone and comparators (placebo and ASA)., Authors' Conclusions: Evidence from a small number of trials suggests that dipyrone is effective for ETTH and migraine. No serious adverse events were observed in the included trials, but agranulocytosis is rare and would probably not be observed in such a relatively small sample. A study now ongoing in Latin America may clarify the true risk of agranulocytosis associated with dipyrone use.

The Convergence Hypothesis postulates a single pathophysiological mechanism to explain the clinical spectrum of primary headaches seen in patients with migraine. The history and the scientific underpinnings of the Convergence Hypothesis are presented. Extrapolations from the Convergence Hypothesis are used to explore the evolution of episodic to chronic migraine and the development of common migraine co-morbidities as a consequence of frequent migraine. A patient staging system is presented to illustrate this transformation process in migraine patients.