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Background: Headaches associated with sexual intercourse (coital cephalgia) have many different causes and are often divided in the literature into pre-orgasmic and orgasmic headaches., Objective: To present a case of orgasmic headache caused by a basilar artery dissection and to present a literature-based guide to the diagnosis and management of patients presenting with headaches related to sexual activity., Case Report: We report the case of a 34-year-old man without significant past medical history who presented to the Emergency Department with two episodes of orgasmic headache caused by basilar artery dissection., Conclusions: The cause of headaches related to sexual activity range from the benign to the life-threatening. Due to the dynamics of cerebral blood flow during sexual intercourse, basilar artery dissections and aneurysms should be considered in patients with sudden-onset headaches during orgasm. Appropriate brain imaging and, possibly, lumbar puncture may assist in identifying potentially life-threatening causes of coital headaches., (Copyright © 2012 Elsevier Inc. All rights reserved.)

In 1999, the nomenclature and case definitions for neuropsychiatric lupus syndromes were published by American College of Rheumatology (ACR), and the cognition of neuropsychiatric damage of systemic lupus erythematosus (SLE) was gradually unified and standardized. Lupus headache is an intractable problem in SLE, especially in SLE patients complicated with multiple organ injury. In general, vascular headache is common in most SLE patients, and a small number of SLE patients complicated with nervous headache are found in clinic. Moreover, its pathophysiological mechanism is far from being understood. Although early diagnosis is essential for good outcomes, the diagnosis method is rather confused in the world. There still exist some limitations in the proposal of clinical classification of headache from ACR and International Headache Society (IHS), and the proposal does not mention the classification of headache related to psychiatric damage. Current therapeutic regimens are almost exclusively based on empirical evidence. Treatment approaches include symptomatic treatment, immunosuppressive, anticoagulant and anti-aggregant therapies. It provides enormous and hopeful space in research of combined therapy strategy, especially in the field of traditional Chinese medicine. The authors discussed the relationship between lupus headache and headache due to internal injury in the view of integrated traditional Chinese and Western medicine, and suggested that the treatment strategy for lupus headache should be made in argument with the headache due to internal injury. Syndrome differentiation treatment according to deficiency in the root and excess in the branch and the therapy for activating blood to dredge collaterals maybe have great advantages in treatment of the headache in SLE.

Purpose of Review: In the past decade, primary and secondary central nervous system (CNS) vasculitides have been more commonly diagnosed and recognized than previously. With the increasing awareness of these disorders, it is crucial for the treating physician to differentiate between causes of CNS vasculitis and to recognize their marked clinical and pathophysiological heterogeneity. This review focuses on the major forms of primary CNS vasculitis, as well as secondary CNS vasculitis with emphasis on their clinical findings, diagnoses, and treatment., Recent Findings: The proposal of reversible cerebral vasoconstriction syndromes (RCVS) as a unifying concept for a group of disorders which are characterized by acute-onset severe recurrent headaches, with or without additional neurologic signs and symptoms, and prolonged but reversible vasoconstriction of the cerebral arteries, has been a major breakthrough in this field over the past decade. Recognition of this common mimic (i.e. RCVS) has allowed optimal management of a sizable group of patients previously confused with pathologically documented CNS vasculitis., Summary: Sound treatment decisions are based on accurate diagnosis. It is essential for the clinicians involved in the evaluation of patients with CNS vasculitis to be aware of its mimics especially RCVS. This article provides a comprehensive review of CNS vasculitis and its differential diagnosis. Furthermore, it touches upon workup and treatment of CNS vasculitis.

Tension-type headache and migraine are the most common types of primary headaches. Apart from these well known diseases, the group of primary headaches includes other relatively rare headache disorders. Some of these seldom syndromes have been described for the first time within the last twenty years and have been newly included in the revised IHS classification from 2004. Their typical symptomatic is less known, but offers an excellent opportunity to diagnose these syndromes. The importance of recognising these disorders is underlined by the fact, that rare primary headaches response often complete and rapid to a specific treatment. This review summarizes the current knowledge on the clinical presentation and treatment of cluster headache, paroxysmal hemicrania, SUNCT syndrome, hemicrania continua and hypnic headache.

Patients complaining of pain in tooth bearing regions of the oral cavity may be suffering from a disorder unrelated to pulpal pathology. Unnecessary dental procedures may complicate the diagnosis and aggravate the underlying disorder. Referral of pain to the oral cavity and teeth may easily be explained by the extensive neurological connections of the trigeminal brain stem complex with other cranial nerves and input from the upper segment of the cervical spine. Accurate diagnosis of painful disorders can be a complex and time consuming process. The primary responsibility of the dentist in this scenario is to rule out the possibility of odontogenic pain.

Although the elderly generally suffer less often from headache, symptomatic headache and concomitant diseases are more frequent. For the diagnostic work-up of newly occurring headaches in old age, imaging examinations of the head (CT or MRI), together with laboratory investigations are necessary. The treatment of symptomatic headache is oriented to the underlying disease, and should be applied promptly, with the aim of preventing serious complications. When considering the management strategy, account must be taken of possible changes in pharmacokinetics, pharmacodynamics and the therapeutic response in the elderly. Numerous medications often cause headache or other side effects in patients of advanced age. Most antiheadache drugs have not been adequately been investigated in the group of over 65-year-olds. This means that drug treatment for headache is often applied in accordance with the principle: start low, go slow.

We present a review of 22 cases of headache mimicking chronic paroxysmal hemicrania (CPH) (17 female and five male; F : M ratio 3.4), nine cases mimicking hemicrania continua (HC) (seven female and two male) and seven cases mimicking SUNCT syndrome (five male and two female) found in association with other pathologies published from 1980 up to the present. All case reports were discussed with respect to diagnostic criteria proposed by International Headache Society (IHS) for CPH, by Goadsby and Lipton for HC and SUNCT, and evaluated to identify a possible causal relationship between the pathology and the onset of headache. The aim of the present review was to evaluate if the presence of associated lesions and their location could help elucidate the pathogenesis of trigeminal autonomic cephalalgias (TACs).

Paroxysmal hemicrania (PH) is one of the trigeminal autonomic cephalgias (TACs), a group of primary headache disorders characterized by unilateral trigeminal distribution pain that occurs in association with prominent ipsilateral cranial autonomic features. The TACs are relatively rare, which is likely to be why they are poorly recognized in primary care. TACs will thus be referred to neurologists eventually, offering an excellent opportunity to diagnose and treat these patients. PH responds in a dramatic and absolute fashion to indomethacin. The importance of recognizing these syndromes is underscored by their excellent but highly selective response to treatment. This is the case report of our patient with PH and the review of current knowledge about pathophysiology of TACs, as well as differential diagnosis of other entities from this headache group.

We describe a man with chronic paroxysmal hemicrania, who remained free of headaches on indomethacin, 25 mg once or twice daily. However, in this case, in contrast to typical cases of paroxysmal hemicrania, the pain of the headaches was nonlateralized and was located in the centre of the forehead. The headaches were not associated with local autonomic symptoms or signs involving the eyes or nose. Initially, the pain of the headaches lasted for seconds only and was brought on by coughing.

Trigemino-autonomic cephalalgias (TAC) constitute a rare group of primary headache conditions associated with unilateral fluctuating head pain and autonomic symptoms. These syndromes, including cluster headache, hemicrania continua, paroxysmal hemicrania and SUNCT, are much less prevalent than migraine and tension-type headache. The pathogenesis of TAC is unknown. Similar neuropeptide changes seen in all TAC syndromes suggest a shared underlying pathophysiology in these headaches. Some of them respond to the treatment with indomethacin and sumatriptan. Two patients suffering from one of the trigemino-autonomic cephalalgias are presented in the paper.

For the neurological differential diagnosis of facial pain, symptomatic pain must be differentiated from the so-called primary pain syndromes. Trigeminal neuralgia is usually readily diagnosed on the basis of the typical history. The treatment of choice is carbamazepine. If this fails, invasive options are available. Atypical facial pain should be diagnosed only when all known primary and secondary pain syndromes have been excluded. Treatment is difficult and comprises the administration of tricyclic antidepressants. Cluster headache and chronic paroxysmal hemicrania each has an unmistakable temporal course. Although the etiology remains unknown, specific therapeutic options are available. The Tolosa-Hunt syndrome is presumably caused by a granuloma in the cavernous sinus, and treatment is effected with corticosteroids. Painful craniomandibular dysfunction (CMD) is often misinterpreted as atypical facial pain.

Two cases of paroxysmal hemicrania (PH) associated with trigeminal neuralgia are reviewed. The paroxysmal hemicrania component in one patient was episodic, while it was chronic in the other. Each headache type responded completely to separate treatment, highlighting the importance of recognizing this association. We review the six other cases of chronic paroxysmal hemicrania-tic (CPH-tic) reported, and suggest that the term paroxysmal hemicrania-tic syndrome (PH-tic) be used to describe this association.

The trigeminal autonomic cephalgias (TACs) are a group of primary headache disorders characterised by unilateral trigeminal distribution pain that occurs in association with ipsilateral cranial autonomic features. This group of headache disorders includes cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome). Although hemicrania continua has previously been classified amongst the TACs, its nosological status remains unclear. Despite their similarities, these disorders differ in their clinical manifestations and response to therapy, thus underpinning the importance of recognising them. We have outlined the clinical manifestations, differential diagnoses, diagnostic workup and the treatment options for each of these syndromes.

Calcitonin gene-related peptide (CGRP) has been detected in increased amounts in external jugular venous blood during migraine attacks. However, it is unknown whether this is secondary to migraine or whether CGRP may cause headache. In a double-blind crossover study, the effect of human alphaCGRP (2 microg/min) or placebo infused intravenously for 20 min was studied in 12 patients suffering from migraine without aura. Headache intensity was scored on a scale from 0 to 10. Two patients were excluded due to severe hypotension and one because she had an infection. In the first hour median peak headache score was 1.0 in the halphaCGRP group vs. 0 in the placebo group (P < 0.01). During the following 11 h all patients experienced headaches after halphaCGRP vs. one patient after placebo (P = 0.0004). The median maximal headache score was 4 after CGRP and 0 after placebo (P = 0.006). In three patients after halphaCGRP, but in no patients after placebo, the delayed headache fulfilled the IHS criteria for migraine without aura. As intravenous administration of halphaCGRP causes headache and migraine in migraineurs, our study suggests that the increase in CGRP observed during spontaneous migraine attacks may play a causative role.

Headaches are a universal experience and one of the most common causes for physician consultation. The physician must determine whether a neuroimaging study is warranted to aid in the diagnosis of primary or secondary headaches. Guidelines on neuroimaging of headache patients have been developed based on review of the literature; however, their applicability must be adapted to specific clinical situations. In general, neuroimaging is most likely to be useful if the history is not typical of a primary headache type (e.g., tension type, migraine, cluster headaches) or the neurological examination is abnormal. Neuroimaging has been crucial in the investigation of the pathogenesis of migraine and cluster headaches. Secondary headaches, which may be diagnosed by neuroimaging studies, include subarachnoid hemorrhage, cerebral venous thrombosis, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, aqueductal stenosis, and arterial dissection.

Background: Hemicrania continua is an indomethacin-responsive headache disorder characterized by a continuous, moderate to severe, unilateral headache. More than 90 cases of hemicrania continua have been reported, but there is still uncertainty about its clinical features., Methods: The authors compared 34 new cases (24 women, 10 men) with previously reported cases. All the patients met Goadsby and Lipton's proposed criteria. The authors compared baseline (continuous background headache) and exacerbation (attacks of severe periods of headaches)., Results: The baseline headache was typically mild to moderate in intensity and usually not associated with severe disability. In contrast, the headache exacerbations were severe and associated with photophobia, phonophobia, nausea, and disability. At least one autonomic symptom was present in 25 patients (74%). Jabs and jolts were present in 14 patients (41%). The mean indomethacin dose was 136.7 +/- 60 mg (range 25 to 225 mg). Twenty-four patients (70.6%) met International Headache Society criteria for migraine in their exacerbation period. Occipital tenderness was observed in 23 patients (67.6%). The temporal pattern was remitting in four patients (11.8%), continuous from onset in 18 (52.9%), and continuous evolving from remitting in 12 (35.3%)., Conclusion: Hemicrania continua is not a rare disorder. All cases of chronic unilateral daily headaches should receive an indomethacin trial early if not first in treatment.

Background: and purposes. The purpose of this study was to identify the specific characteristics of headaches associated with cerebral arteriovenous malformations in order to differentiate them from other known entities of headaches such as migraine, cluster headache, and trigeminal neuralgia. This differentiation allows an early diagnosis of cAVM and a treatment to be administrated before any cerebral hemorrhage., Patients and Methods: The study included 700 patients with cAVM and treated by radiosurgery. Out of this series, only 109 (48 males, 61 females, mean age of 33) presented with headaches. Headaches were studied as a possible revelation mode of a cAVM, either as an isolated sign, preceding an epileptic seizure, a cerebral hemorrhage, or associated with a neurological deficit. Analysis concerned 13 clinical parameters and 30 anatomic parameters based on angiography., Results: Headaches were found in 15.6%; they were isolated in 6%. They preceded a cerebral hemorrhage in 12.6%, constituting an early alarm signal when increasing in intensity, frequency and duration. They were associated with seizures or a neurological deficit in 9.6%. We found a predominant female sex-ratio (0.78) and occurrence at a young age (72.3% between 10 and 40 years). Headaches were non-pulsating in 95.3%; nausea, vomiting, light or sound phobia were only found in 4.7%. Headaches were unilateral and homolateral to the malformation in 80%, corresponding to the malformation topography in 97.4% in posterior location and 80% in anterior location. Associated neurological symptoms existed in 20.2%; related to the malformation and lasting 5 to 30 minutes. Duration of pain episodes was less than 3 hours in 77% with a frequency of 1 to 2 per month in 82.5%. Pain was mild and responded to simple analgesics. A family migraine was found in only 3 patients. The angiographic characteristics of the malformations were meningeal afferences, superficial venous drainage and posterior location., Conclusions: Headaches associated with cerebral arterio-venous malformations form a distinct category that can be determined from specific characteristics; this should help an early diagnosis of cerebral arterio-venous malformations in order to start a treatment before the occurrence of cerebral hemorrhage.

Classifying headaches as primary (migraine, tension-type or cluster) or secondary can facilitate evaluation and management A detailed headache history helps to distinguish among the primary headache disorders. "Red flags" for secondary disorders include sudden onset of headache, onset of headache after 50 years of age, increased frequency or severity of headache, new onset of headache with an underlying medical condition, headache with concomitant systemic illness, focal neurologic signs or symptoms, papilledema and headache subsequent to head trauma. A thorough neurologic examination should be performed, with abnormal findings warranting neuroimaging to rule out intracranial pathology. The preferred imaging modality to rule out hemorrhage is noncontrast computed tomographic (CT) scanning followed by lumbar puncture if the CT scan is normal. Magnetic resonance imaging (MRI) is more expensive than CT scanning and less widely available; however, MRI reveals more detail and is necessary for imaging the posterior fossa. Cerebrospinal fluid (CSF) analysis can help to confirm or rule out hemorrhage, infection, tumor and disorders related to CSF hypertension or hypotension. Referral is appropriate for patients with headaches that are difficult to diagnose, or that worsen or fail to respond to management

The association of chronic paroxysmal hemicrania and ticlike pain-chronic paroxysmal hemicrania-tic (CPH-tic)-is a recently described syndrome. The authors found only two previously reported cases. They report three new cases of this rare syndrome with both chronic paroxysmal hemicrania and ticlike pain concurrently and ipsilaterally. The trigeminal-autonomic cephalalgias (TAC) are considered as differential diagnoses. CPH-tic syndrome could be a different clinical entity.

Objective: To offer reference of the screening procedure for aircrew and astronaut selection., Method: The competitive frequency-fluctuation characteristics of the dominant frequency in the band of 7-12 Hz in EEG and power density spectra of alpha band power-fluctuation were analyzed with a newer system of electroencephalofluctuographic technology in 20 patients (male 13, female 7, aged 17-37 years) with paroxysmal headache., Result: During episodes of headache in the affected sites the cumulated dominant fluctuation probability of each frequency components was in the order of 7, 8, 9, 10, 11, 12 Hz from high to low; The relative entropy value was 92.25% +/- 3.41%; Two peaks having at least 25% higher value than those of its neighbors were observed at 1.86 mHz and 4.65 mHz in power density spectra of power-fluctuation. During the intervals of episodes the above-mentioned probability character disappeared; The relative entropy value was decreased to 69.08% +/- 4.16% ; A new peak was found at 0.93 mHz in spectra power-fluctuation, with a reduction in peak value power at 4.65 mHz., Conclusion: It not only offered a parameter for pathophysiological and functional evaluation of paroxysmal headache, but also provided reference of the screening procedure for aircrew and astronaut selection.

We report the case of a 42-year-old man with repeated attacks of headache associated with retrograde amnesia. Neuropsychological tests before and after the major episode of amnesia showed mild neuropsychological deficits but with spared anterograde memory and learning functions. The amnesia was dense for a period of 15-20 years and included people and events (public and private). There was also a suggestion of amnesia for learned skills. Neurologically he had mild clinical signs and focal EEG-abnormalities in the left fronto-temporal region, but CT, MRI, and SPECT showed no abnormality. Five years after the onset of amnesia there was no recovery of the retrograde memory deficit, but a PET (glucose) scan was normal and neuropsychological testing showed no deficits. An association with migraine has been reported for some non-classical amnesias, but this is the first case of selective retrograde amnesia in a patient with headache as a primary neurological diagnosis.

Orofacial pain, especially if the problem is chronic, presents a diagnostic and management challenge to all health practitioners. This paper suggests how clinicians might simplify the diagnosis of orofacial pain. First, the pain is classified into one of the three basic pain categories: somatic, neuropathic, or psychogenic pain. Somatic pain results from noxious stimulation of normal neural structures. Neuropathic pain is caused by a structural abnormality in the nervous system. Psychogenic pain arises from psychic causes; there is no apparent physiologic or organic basis for the pain. The next step is to determine the tissue system from which the pain arises: intracranial, extracranial, musculoskeletal, neurovascular, neurogenous, or psychological. Finally, some of the more common orofacial pain syndromes within each category are discussed.

We present a case of facial pain associated with sweet stimulus. An immediate, electric-like, short, unilateral pain was evoked by strong sweet gustatory stimulation. This was followed 6 to 8 hours later by a bilateral severe headache associated with bilateral tearing, rhinorrhea, periorbital swelling, flushing, and photophobia that lasted up to 2 days. The immediate pain that was experimentally induced with 2.5 grams of sucrose placed on the tongue could be abolished with carbamazepine. However, carbamazepine did not prevent the headache complex that appeared 6 to 8 hours later. Conversely, a trial with indomethacin abolished the late-onset headache, but not the immediate neuralgic-type pain. The independent nature of these pains suggests different pathophysiological mechanisms which are discussed.

Basilar migraine is a complicated headache which the International Headache Society describes as 'migraine with aura symptoms clearly originating from the brainstem or from both occipital lobes'. For years this headache was thought to originate from a transient disturbance in the vertebrobasilar circulation, but more recent studies suggest that a central neuronal disorder may be the source of migraine. Basilar migraines may have certain symptoms which are similar to other neurologic, vascular, psychiatric and metabolic diseases, yet there are specific criteria which can help differentiate it from other diagnoses. It is characterized by a throbbing occipital headache which may be preceded by an aura. The unusual symptoms of basilar migraine, which may precede and continue throughout the duration of the headache and even after it, include bilateral visual symptoms, altered mental status, vertigo, gait ataxia, bilateral paresthesia, bilateral paralysis and dysarthria. We describe a 29-year-old black female whose husband brought her to the emergency department complaining of confusion, headache, and left-sided weakness for 2 h prior to arrival.

A comprehensive study of cerebral hemodynamics and microcirculation was made in 261 patients complaining for headache. Clinical-diagnostic criteria for headache of vascular origin, a scheme of vascular headache pathogenesis are proposed. It was found that the first to appear were negative alterations in the venous microcirculation. Neurophysiological methods with functional loading tests for objectivation of vascular cerebral defects at early stages and in the follow-up proved diagnostically effective.

The clinical features of idiopathic stabbing headache ("jabs and jolts syndrome") were studied in 38 patients who were diagnosed throughout a 1-year period. Mean age at the onset of symptoms was 47.1 years +/- 14.5 (SD), and a clear female preponderance was demonstrated (female/male ratio = 6.6). Painful attacks were ultrashort, i.e. virtually all attacks in more than two thirds of cases lasted only one second. The frequency of attacks varied immensely, ranging from 1 attack per year to 50 attacks daily. The pain paroxysms usually occurred with an irregular or sporadic temporal pattern. The localization of painful attacks was reported frequently as unifocal, usually in the orbital area, but also multifocal patterns were observed, the attacks frequently changing location from one area to the next. The majority of attacks occurred spontaneously, and accompanying phenomena were reported only rarely. Indomethacin treatment (75 mg daily) seemed to have a complete or partial effect in most patients treated as such (n = 17).

Objective: To determine the value of performing computed tomography (CT) on magnetic resonance imaging (MRI) studies in children with chronic headaches., Background: Headache is a common complaint in children. With the proliferation of brain imaging centers and the increasing patient demand for CT or MRI studies, brain imaging has become widely used to evaluate headaches., Methods: A retrospective chart review was conducted of all children referred to the pediatric neurology clinic for evaluation of headaches over a 2-year period. Charts were reviewed for headache characteristics, clinical indications for performing CT and MRI studies, and imaging results. Particular attention was paid to evidence of brain tumors, vascular anomalies, or hydrocephalus., Results: A total of 133 records were studied. Subjects ranged in age from 3 to 18 years. Most patients were diagnosed as having either vascular migrainous headaches (52%) or chronic tension headaches (21%). Other headache diagnoses were mixed tension-migraine, psychogenic, and post-traumatic. Headaches were unclassified in 25 patients (19%). Seventy-eight patients (59%) had brain imaging: 45 had MRI, 27 had CT, and 6 patients had both. In most cases, brain imaging studies were performed in patients with atypical headache pattern, presence of neurologic abnormalities during the headache, general symptoms (ie, weight loss or fatigue), or because of parents' or doctors' concerns about brain tumors. Cerebral abnormalities were found on brain imaging in four patients, but none indicated the presence of a treatable disease and all were deemed unrelated to the presenting complaint. Our findings of no relevant abnormalities in a series of 78 brain imaging studies indicate that the maximal rate at which such abnormalities might appear in this population is 3.8%., Conclusions: These results indicate that brain imaging studies have very limited value in evaluating headaches in pediatric patients without clinical evidence of an underlying structural lesion.

Study Objective: To compare the effectiveness of i.v. and PR prochlorperazine for treatment of acute benign vascular or tension headache., Design: Prospective, randomized, double-blind trial., Setting: University emergency department with 50,000 annual census., Participants: Forty-five adult patients enrolled on 46 visits., Interventions: Patients received 10 mg prochlorperazine i.v. and placebo suppository or 25 mg prochlorperazine PR and placebo injection. Pain assessment was made using a 10-cm visual-analog scale; scores were analyzed using Wilcoxon/Kruskal-Wallis rank-sum tests (alpha of .01)., Results: Mean 60-minute pain scores for i.v. and PR groups were 0.6 and 3.5, respectively (P = .0002). Two patients (8.7%) in the i.v. group and six patients (26.1%) in the PR group required rescue analgesia (P = .12)., Conclusion: i.v. prochlorperazine appears to provide more effective relief than PR prochlorperazine for benign vascular or tension headaches.

Background: Postpartum cerebral angiopathy is associated with the use of of ergot alkaloids. The exact mechanism is unclear but may be related to their sympathomimetic properties, as evidenced in patients already on other ergot derivatives who deteriorated only after taking additional sympathomimetic drugs. We postulate that sympathomimetic agents, independent of ergot alkaloids, may produce the same complication., Case Description: A postpartum patient, initially presenting with headaches, subsequently manifested rapid neurological deterioration after ingesting isometheptene, a sympathomimetic drug. She was not on any ergot derivative but presented similar clinical and radiological manifestations. She experienced increased headache severity, visual disturbance, and seizures associated with multiple segmental cerebral vasoconstriction on angiography and increased T2-weighted signal in the occipital areas on magnetic resonance imaging., Conclusions: This case is additional evidence that sympathomimetic actions of some drugs, such as ergot derivatives and isometheptene, may lead to postpartum cerebral angiopathy. Documentation of medication used by postpartum women suffering similar complications is needed to verify these findings.