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The non-contrast CT scan (Fig. 1 of Images in Neuroscience: Question) shows subarachnoid haemorrhage (SAH) within a right frontal lobe sulcus. While the arterial phase image (Fig. 2 of Images in Neuroscience: Question) of the digital subtraction angiography is normal, the early and late venous phase images (Fig. 3a and b of Images in Neuroscience: Question) demonstrate a persistent linear filling defect within the Vein of Trolard confirming thrombus. MRI to assess cerebral parenchyma shows a tubular structure showing high signal on T1-weighted imaging (Fig. 4a of Images in Neuroscience: Question) confined within the pre-central sulcus, indicating thrombus within a vessel or SAH. There is high signal on fluid attenuated inversion recovery MRI and blooming artefact on gradient echo imaging around the previously described tubular structure extending further along the sulcus (Fig. 4b and c of Images in Neuroscience: Question). Together with the CT scan findings and the venogram images, the MRI findings confirm Vein of Trolard thrombosis with adjacent SAH. Thunderclap headache is classically described as severe headache, which takes less than a minute or a few minutes from its onset to peak. The differential diagnosis for thunderclap headache includes SAH, cerebral venous sinus thrombosis, idiopathic benign recurrent headache disorder, arterial dissection, pituitary apoplexy, spontaneous intracranial hypotension, and hypertensive encephalopathy. Cerebral venous thrombosis confined to a cortical vein without involvement of the venous sinuses is rare. In our patient, this was associated with SAH. This phenomenon of SAH secondary to isolated cortical venous thrombosis has been described in a few

In their review of thunderclap headache Ducros and Bousser advocate visually inspecting cerebrospinal fluid (CSF) to look for the presence of blood …

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.

Background: The Call–Fleming syndrome (CFS) comprises acute severe recurrent (thunderclap) headaches, occasional transient or fluctuating neurological abnormalities and reversible segmental cerebral vasoconstriction. It is a benign condition with an excellent prognosis, yet because it is often clinically and radiologically similar to a number of commonly encountered conditions, diagnostic difficulties may arise, leading to inappropriate, and even potentially harmful, investigative and therapeutic approaches. Cases: Three personal cases are presented to highlight the occurrence of subarachnoid haemorrhage (SAH) as part of CFS. In two patients with a positive CT head, SAH involved the sulci in the upper cerebral convexity, an unusual location in aneurysmal SAH. Results: SAH is not an uncommon feature of CFS, occurring in approximately 25% of reported cases, and may pose a diagnostic challenge. CFS has a relatively characteristic spectrum of features, allowing a confident diagnosis in most cases, even when atypical features such as SAH are present. Conclusions: Recognising the spectrum of abnormalities seen in CFS, including particularly SAH, allows a sound approach to a safe diagnosis.

We present the first report of association between primary sexual headache (PSH) and hypnic headache (HH). A 36-year-old man without any relevant medical or headache history was referred to our Headache Clinic because of a 3-month history of headaches. They were of explosive onset, holocranial location, and exclusively triggered by sexual activity, and they lasted 5–30 min. At the time of consultation the patient had suffered more than 20 attacks. He also described another type of headache with episodes starting 1 month after the initial presentation of the orgasm-related headache. It was different in quality, dull, with bilateral location, moderate in intensity, awaking the patient, lasting for 3 h. He had had more than 20 episodes per month occurring only during sleep. There were no other symptoms and the examination was normal. MRI and MR angiography of the head were normal. Indomethacin 25 mg 30–60 min before sleep or sexual activity was started and decreased the headache intensity. A 50-mg dose was then prescribed, which resulted in resolution of headache. Although this atypical association could be explained in terms of coincidence, PSH and HH are infrequent types of headache and could be the expression of a common pathophysiological mechanism. An impaired metabolic cerebrovascular autoregulation [1] was described as a causative factor in PSH, and vasospasm was seen on angiography. On the other hand, HH has been related with an impairment of the suprachiasmatic nucleus and could cyclically activate a dysnociceptive mechanism leading to both sudden awakening and headache [2]. In any case, a common situational hypothalamic nucleus activation could induce disturbances of cerebrovascular autoregulation and a suprachiasmatic malfunction. Different studies on brain imaging show increased activation in the paraventricular nucleus of the hypothalamus during orgasm [3]. There are several cases providing evidence of the role of hypothalamus in the pathogenesis of vasospasm [4]. A hypothalamus malfunction could justify both types of headache, explaining the convergence of the two entities in our patient.

In considering thunderclap headache, the immediate need is to exclude the possibility of subarachnoid hemorrhage. Both our protagonists agree on this point, that the minimum required is to perform a plain brain CT and, if there is no evidence of hemorrhage, lumbar puncture. We agree with Moussouttas and Mayer that spectrophotometry to detect xanthochromia should be mandatory. It is at this point where many clinicians diverge in their …

A 59-year-old Asian woman suffered from a TCH followed by sudden, binocular blurred vision, mimicking pituitary apoplexy. The diagnostic workup (including fluorescein angiography, MRI of the brain, and CSF analysis) showed severe optic disc swelling and dye leakage of multiple faint hyperfluorescent spots at retinal pigment epithelium level, diffuse pachymeningeal hypertrophy, and monocytic pleocytosis, respectively. VKH disease should be considered in the differential diagnosis of patients presenting with a TCH followed by sudden, bilateral decreased visual acuity.

Purpose We describe a series of patients with hemicrania continua, a rare indomethacin-responsive primary headache syndrome, who presented for evaluation with neuro-ophthalmologic signs and symptoms. Design Observational case series. Methods Nine patients between the ages of 29 and 58 years were seen with various neuro-ophthalmologic findings and a unilateral continuous headache. A detailed history was taken from each patient, followed by a focused ophthalmologic and neurologic examination. The risks and benefits of treatment with indomethacin were discussed. Patients were instructed to call after several days of treatment to report any change in their headache and neuro-ophthalmologic symptoms, in addition to any adverse side effects. Results All patients responded favorably to indomethacin, with rapid near-complete or complete resolution of headache and autonomic symptoms, and treatment was initiated as early as possible. Conclusions Because of its absolute response to indomethacin, recognizing the neuro-ophthalmologic symptoms of hemicrania continua as a component of the headache syndrome is critical for prompt initiation of treatment.

The internal working model on attachment dimensions changes with significant emotional experiences. The purpose of this study was to evaluate if and how the internal working models correlate with primary headaches. Attachment dimensions of subjects suffering from primary headaches were studied. One hundred and fourteen subjects [68 with migraine, 23 with tension–type headache (according to ICHD–I criteria), 23 with chronic daily headache (according to Silberstein’s criteria)], were studied and compared with a control group of 57 subjects (matched in sex, age and social level) not suffering from any primary headache. Attachment dimensions were investigated using the Adult Attachment Questionnaire (AAQ) and the Attachment Style Questionnaire (ASQ). Headache sufferers seem to be characterised by attachment styles of the “insecure” type. In particular they seem to feel extremely ill at ease if there is an expectation of reduction of interpersonal distance.

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.

The aim of the study was to evaluate the association between sleep disturbance and headache type and frequency, in a random sample of participants in the third Nord-Trøndelag Health Survey. The headache diagnoses were set by neurologists using the ICHD-2 criteria performing a semi structured face-to-face interview. Sleep problems were measured by the two validated instruments Karolinska Sleep Questionnaire (KSQ) and Epworth Sleepiness Scale (ESS). Among 297 participants, 77 subjects were headache-free, whereas 135 were diagnosed with tension-type headache (TTH), 51 with migraine, and 34 with other headache diagnoses. In the multivariate analyses, using logistic regression, excessive daytime sleepiness, defined as ESSor= 10, was three times more likely among migraineurs compared with headache-free individuals (OR = 3.3, 95% CI 1.0-10.2). Severe sleep disturbances, defined as KSQ score in the upper quartile, was five times more likely among migraineurs (OR = 5.4, 95% CI 2.0-15.5), and three times more likely for subjects with TTH (OR = 3.3, 1.4-7.3) compared with headache-free individuals. Subjects with chronic headache were 17 times more likely to have severe sleep disturbances (OR = 17.4, 95% CI 5.1-59.8), and the association was somewhat stronger for chronic migraine (OR = 38.9, 95% CI 3.1-485.3) than for chronic TTH (OR = 18.3, 95% CI 3.6-93.0). In conclusion, there was a significant association between severe sleep disturbances and primary headache disorders, most pronounced for those with chronic headache. Even though one cannot address causality in the present study design, the results indicate an increased awareness of sleep problems among patients with headache.

Pheochromocytoma is a well known, catecholamine-producing tumor characterized by hypertension, headache, hyperglycemia, hypermetabolism, and hyperhydrosis. Approximately 65% of cases of pheochromocytoma were shown to be associated with hypertension. A case of pheochromocytoma that presented with thunderclap headache (TCH) and palpitations is reported. The patient never showed hypertension during the course of the disease. Paroxysmal headache and palpitations led to the identification of the underlying condition, and the final diagnosis was confirmed by histopathological examination of a surgical specimen. Pheochromocytoma should be identified as a less common although important cause of TCH. In addition, due to its lack of utility in identifying this disorder, negative cranial imaging may impede further investigation of extracranial lesions that may be the cause of a patient’s headache. According to the International Classification of Headache Disorders (ICHD)-II, headache attributed to pheochromocytoma usually develops concomitantly with an abrupt increase in blood pressure. In our case, however, hypertension was never observed, even when the patient was symptomatic. This is the first report of a case of pheochromocytoma with TCH without hypertension.

This study was planned to investigate the economic impact of headache on Turkish headache sufferers attending a tertiary care outpatient headache clinic.A total of 937 headache patients were included in this study and questioned using a questionnaire for the profile of patients and headache, quality of life of patients and economic impact of headache. The median total direct cost was found to be 88.0 USD and the median total cost was 160.7 USD. The drug treatment cost was the highest item followed by the specialist outpatient care cost. The average lost and inefficient work/school days was 1.5 (0-45) and 8.4 (0-100) days for one year. It was shown that loss of productivity was higher for migraine without aura group when compared with the episodic and chronic tension-type headache groups. The results of this nationwide university hospital based study methshowed that headache, especially migraine, has considerable economic impact on patients.

A case valsalva-induced cluster headache is presented. Cluster attacks induced by valsalva manoeuvres alone were only recently described, and such patients have features of cough headache and cluster headache. Attacks occurred a couple of times a week in the patient presented, solely triggered by valsalva manoeuvres including coughing, sneezing or straining and not by exercising.

A relationship between migraine and patent foramen ovale (PFO) has been observed in relatively small series of patients so far. Furthermore, the exact mechanism underlying such an association remains unknown. In the present study we determined the prevalence of PFO by contrast–enhanced transcranial Doppler (TCD) in a group of 260 patients with migraine with aura (MA+), 74 patients with migraine without aura (MA–), and 38 patients with cluster headache (CH). One–hundred–sixty–one MA+subjects (61.9%), 12 MA–subjects (16.2%), and 14 CH–subjects (36.8%) were PFO–carriers. The association was independent on the frequency of migraine attacks and complexity of aura. Finally, among the 15 patients who had a history of at least one migraine attack occurring during a Valsalva maneuver only one subject turned out to be PFO–carrier. Our findings confirm previous observations of a link between MA+, CH, and PFO. They also suggest that such an association is independent on migraine clinical phenotype and is probably unrelated to the pathogenic mechanism of paradoxical embolism.

Stabbing headache can be encountered in both primary and secondary forms, but has been infrequently reported among patients with stroke, and is not known to be associated with a small well-circumscribed brain lesion. A 95-year-old woman taking warfarin presented with the sudden onset of stabbing headache strictly in the right frontal and supraorbital regions, along with gait imbalance and dysarthria. Neuroimaging revealed a small left thalamic hematoma. This association of an acute thalamic lesion with stabbing headache in the contralateral trigeminal distribution is discussed, along with a brief review of stabbing headache occurring in cerebrovascular disease.

This study evaluates osmophobia (defined as an unpleasant perception, during a headache attack, of odours that are non-aversive or even pleasurable outside the attacks) in connection with the diagnosis of primary headaches. We recruited 775 patients from our Headache Centre (566 females, 209 males; age 38+/-12 years), of whom 477 were migraineurs without aura (MO), 92 with aura (MA), 135 had episodic tension-type headache (ETTH), 44 episodic cluster headache (ECH), 2 chronic paroxysmal hemicrania (CPH) and 25 other primary headaches (OPHs: 12 primary stabbing headaches, 2 primary cough headaches, 3 primary exertional headaches, 2 primary headaches associated with sexual activity, 3 hypnic headaches, 2 primary thunderclap headaches and 1 hemicrania continua). Among them, 43% with MO (205/477), 39% with MA (36/92), and 7% with CH (3/44) reported osmophobia during the attacks; none of the 135 ETTH and 25 OPH patients suffered this symptom. We conclude that osmophobia is a very specific marker to discriminate adequately between migraine (MO and MA) and ETTH; moreover, from this limited series it seems to be a good discriminant also for OPHs, and for CH patients not sharing neurovegetative symptoms with migraine. Therefore, osmophobia should be considered a good candidate as a new criterion for the diagnosis of migraine.

Case reportAn 80-year-old woman abruptly developed an explosive,severe headache in the bilateral occipital and frontalregions associated with neck pain/stiffness while sitting onher couch watching television. There was no associatedloss of consciousness or awareness, nausea, vomiting,autonomic symptoms, photophobia or phonophobia. Shedenied any preceding neck pain or history of head or necktrauma.Prior to this event, she rarely experienced mild, infre-quent episodic headaches lasting a few hours only whichwere relieved by aspirin. Her medical history includeddiabetes, hypertension, coronary artery disease, prior cer-ebellar infarcts, glaucoma, and deep venous thrombosis forwhich she was receiving anticoagulation. Her medicationsincluded warfarin, aspirin, clopidogrel, simvastatin, val-sartan, and carvedilol. She was a heavy smoker, but deniedalcohol or drug use.In the emergency department she was afebrile and hadmild neck stiffness, particularly on neck flexion. Bloodpressure was 168/89 mm Hg with a heart rate of 85 beatsper minute. Neurological examination did not revealpapilledema or any focal, long tract, or lateralizing signs.Cranial computed tomography (CT) was negative forintracranial hemorrhage. Blood work revealed a markedlyelevated INR (6.0), but CBC, electrolytes, ESR (26 mm/h),urinalysis, and serum glucose (138 mg/dL) were normal.The patient was administered 4 U of fresh frozen plasma toreverse her coagulopathy to perform an emergent lumbarpuncture; during this time she underwent magnetic reso-nance imaging (MRI) and angiography (MRA) of the brainand neck (time-of-flight with fat-suppression), which wereremarkable only for evidence of prior bilateral cerebellarand small deep left middle cerebral artery territory infarcts.Although magnetic resonance venography (MRV) was notperformed, contrast-enhanced MRI revealed patency andnormal enhancement of the venous sinuses.Cerebrospinal fluid (CSF) examination demonstratedsubarachnoid hemorrhage (SAH) as well as a neutrophilicpleocytosis (540 red blood cells and 8 white blood cells perhigh power field intube 1,2,100red blood cells and15 whiteblood cells per high power field in tube 4, 96 % polymor-phonuclear leukocytes, glucose 100 mg/dL), with elevatedCSF protein levels (72 mg/dL). Gram stain and subsequentCSF cultures revealed no causative organism. Chest X-raydid not demonstrate any infiltrate. Given the possibility of acentral nervous system (CNS) infectious process, the patientwas empirically treated with intravenous antibiotics(vancomycin, ceftriaxone, ampicillin) and acyclovir.Because of the persistence of head and neck pain, herhistory of diabetes, and warfarin combined with dual anti-platelet agent use, a cervical epidural etiology (infectious orhemorrhagic) was suspected. Gadolinium-enhanced MRI ofthe cervical and thoracic spine demonstrated a heteroge-neous cervical epidural collection spanning from mid-C2through T1/T2 vertebral level, with a thin rim of contrastenhancement extending from the upper cervical cord tothe anterior pons (Fig. 1). Gradient-echo sequences (notshown) revealed few areas of hypointensity, suggestive of acervical epidural hematoma with acute elements.The headache and neck pain resolved several days afterwithholding anticoagulation and treating with empiricintravenous antibiotics (vancomycin and ceftriaxone).

We report the case of a woman with an apparent primary thunderclap headache which occurred frequently until she achieved a therapeutic dosage of gabapentin. Primary thunderclap headache is a rare type of headache that warrants significant testing to rule out more ominous possibilities. Whether gabapentin may help other primary thunderclap headache sufferers or not remains unclear. Further research is needed.

Background: Headache disorders are common worldwide, causing pain and disability. India appears to have a very high prevalence of migraine, and of other headache disorders in line with global averages. Our objective was to estimate the burdens attributable to these disorders in order to inform health policy. Methods: In a door-to-door survey, biologically unrelated adults (18–65 years) were randomly sampled from urban and rural areas of Bangalore and interviewed by trained researchers. The validated structured questionnaire enquired into several aspects of burden. Results: Of 2,329 participants (non-participation rate 7.4 %), 1,488 (63.9 %; 621 male, 867 female) reported headache in the preceding year. Symptom burden was high. Migraine (1-year prevalence 25.2 %) occurred on average on 28 days/ year but, in 38.0 % of cases (ie, 9.6 % of adults), on ≥3 days/month (≥10 % of days). All causes of headache on ≥15 days/ month (prevalence 3.0 %) occurred on a mean of 245 days/year. Both these and migraine were rated severe in intensity. Participants with headache lost 4.3 % of productive time; those with migraine lost 5.8 % (equating to 1.5 % fromthe adult population). Lost paid worktime accounted for 40 % of this, probably detracting directly from GDP. We estimated population-level disability attributable to migraine using the disability weight from GBD2010 for the ictal state (0.433). Mean disability per person with migraine was 1.8 %, reducing the functional capacity of the entire adult population by 0.46 %. Fewer than one quarter of participants with headache had engaged with health-care services for headache in the last year. Actual expenditure on headache care was greatest among those with headache on ≥15 days/month (especially probable medication-overuse headache), but otherwise not high. Expressed willingness to pay for effective treatment for headache was higher, signalling dissatisfaction with current treatments. Conclusions: In Karnataka State, southern India, prevalent headache disorders, especially migraine, give rise to commensurately heavy burdens. Limited access to health care fails to alleviate these. Structured headache services, with their basis in primary care, are the most efficient, effective, affordable and equitable solution. They could be implemented within the health-care infrastructure of India and are likely to be cost-saving. This solution requires political will, itself dependent on awareness. Keywords: Headache disorders, Migraine, Tension-type headache, Medication-overuse headache, Burden, Health policy, Willingness to pay, India, South-East Asia, Global campaign against headache © 2015 Rao et al. Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

Background The current definition of cough headache includes provocation of the symptom by Valsalva manoeuvre, and it is generally believed that all cough headache results from a sudden increase in intracranial pressure. We sought to question that presumption and to determine whether the Valsalva test might distinguish primary from secondary cough headache. Methods We examined 16 consecutive cough headache patients using a modified Valsalva test (exhalation into the connecting tube of a standard anaeroid sphygmomanometer to 60 mm Hg for 10 seconds). A positive response was recorded if the manoeuvre provoked headache. All patients subsequently underwent brain MRI. Results None of the patients had neurological signs. Eleven had positive modified Valsalva tests. Ten were found subsequently to have posterior fossa pathologies (secondary cough headache: 8 Chiari Type 1 malformations, 2 posterior fossa meningiomas). The cough headache was relieved following surgery in all cases. One patient with a positive Valsalva test had an apparently normal brain MRI but measurements of hindbrain and posterior fossa dimensions were consistent with ‘posterior fossa crowdedness’. The remaining 5 patients had negative (4 patients) or equivocal (1 patient) Valsalva tests and normal MRI scans (primary cough headache). Conclusions These findings suggest that secondary cough headache results from a transient increase in intracranial CSF pressure during exertion in the presence of obstruction to normal cerebrospinal fluid dynamics. The modified Valsalva test can also determine whether tonsillar herniation found on brain MRI is symptomatic. Primary cough headache appears to be caused by a different mechanism, possibly through congestion of the orbital venous plexus in the presence of jugular venous incompetence and a reduced threshold for trigeminal sensory activation.

Primary headaches among children and adolescents have a substantial impact on quality of life, daily activities, social interaction, and school performance in combination with psychopathological symptoms. The main purpose of the present paper is to summarize clinical and epidemiological evidence for psychiatric comorbidity among children and adolescents with headaches, to describe how evidence in headache research suggest different pathways involved in the development and maintenance of these comorbid conditions, and finally suggest some elements professionals may find helpful to assess the scope of complaints, related functional impairment, and potential precipitating factors in planning of more targeted treatments.