Primary Headache Associated with Sexual Activity: A Review of the Literature.

PURPOSE OF REVIEW: This review summarizes the evolution in diagnosis, evaluation, and treatment of primary headache associated with sexual activity (PHASA). RECENT FINDINGS: Despite increased access to patient information and advances in imaging, the pathophysiology of PHASA remains not fully understood. There are many secondary headaches that may present with headache during sexual activity, and a thorough workup is indicated to rule out potentially life-threatening etiologies. Many recent case series discuss the efficacy of known treatments of PHASA, as well as suggest other potential therapies for this condition including the newer CGRP-targeted therapies. Headaches during sexual activity can be worrisome events which necessitate urgent evaluation, particularly when presenting with sudden-onset and severe "thunderclap" headaches. A thorough workup including imaging should be conducted to rule out etiologies such as subarachnoid hemorrhage, reversible cerebral vasoconstrictive syndrome (RCVS), vasospasm, and dissection. PHASA is commonly comorbid with migraine, tension-type headache, exertional headache, and hypertension. PHASA can present as a dull headache that progresses with sexual excitement, or an explosive headache at or around orgasm. Pain is primarily occipital, diffuse, and bilateral. The headaches are discrete, recurrent events with bouts that typically self-resolve, but may also relapse and remit or continue chronically in some patients. PHASA can be treated preemptively with indomethacin and triptans administered prior to sexual activity, or treated prophylactically with beta-blockers, topiramate, and calcium channel blockers. CGRP-targeted therapies may provide relief in PHASA based on a few case reports, but there are no randomized controlled trials looking at specific efficacy for these therapies.

Update on primary headache associated with sexual activity and primary thunderclap headache.

BACKGROUND: This narrative review aims to provide an update on primary headache associated with sexual activity and primary thunderclap headache. METHODS: We conducted a literature search on PubMed with the keywords "headache associated with sexual activity", "sexual headache", "orgasmic cephalalgia", and "coital cephalalgia" in addition to "thunderclap headache" to assess the appropriateness of all published articles in this review. RESULTS: Primary headache associated with sexual activity is a "primary" headache precipitated by sexual activity, which occurs as sexual excitement increases (progressive at onset), or manifests as an abrupt and intense headache upon orgasm (thunderclap at onset) or combines these above two features. Primary headache associated with sexual activity is diagnosed after a thorough investigation, including appropriate neuroimaging studies, to exclude life-threatening secondary causes such as subarachnoid hemorrhage. According to the criteria of the third edition of the International Classification of Headache Disorders, primary thunderclap headache is also a diagnosis by exclusion. The pathophysiology of primary headache associated with sexual activity and primary thunderclap headache remains incompletely understood. Treatment may not be necessary for all patients since some patients with primary headache associated with sexual activity and primary thunderclap headache have a self-limiting course. CONCLUSION: A comprehensive neuroimaging study is needed for distinguishing primary headache associated with sexual activity or primary thunderclap headache from secondary causes. Primary headache associated with sexual activity and primary thunderclap headache are self-limited diseases and the prognoses are good, but some patients with primary headache associated with sexual activity may have a prolonged course.

Successful treatment of primary headache associated with sexual activity using erenumab: Case report.

BACKGROUND: Primary headache associated with sexual activity is a rare benign headache disorder that is provoked by sexual excitement. It is a diagnosis of exclusion after the underlying secondary causes have been ruled out. The cause of this headache disorder is still unknown. Cerebral vasospasm, pericranial muscular contraction, hemodynamic and respiratory changes during sexual activity are most commonly listed as possible pathophysiological mechanisms. Several acute and prophylactic options with good clinical response have been described in the case series. CASE REPORT: We present a case of a 30-year-old woman with primary headache associated with sexual activity and comorbid with frequent episodic migraine without aura. Both headache disorders were successfully managed with monoclonal antibody against calcitonine gene-related peptide (CGRP) receptor erenumab 70 mg. CONCLUSION: The efficacy of erenumab in our case presumes that CGRP might also be an important mediator in facilitating primary headache associated with sexual activity.

Diagnosis and classification of headache associated with sexual activity using a composite algorithm: A cohort study.

BACKGROUND: To differentiate primary headache associated with sexual activity from other devastating secondary causes. METHODS: In this prospective cohort, we recruited consecutive patients with at least 2 attacks of headache associated with sexual activity from the headache clinics or emergency department of a national medical center from 2005 to 2020. Detailed interview, neurological examination, and serial thorough neuroimaging including brain magnetic resonance imaging and magnetic resonance angiography scans were performed on registration and during follow-ups. Patients were categorized into four groups, i.e. primary headache associated with sexual activity, reversible cerebral vasoconstriction syndrome, probable reversible cerebral vasoconstriction syndrome, and other secondary headache associated with sexual activity through a composite clinic-radiological diagnostic algorithm. We compared the clinical profiles among these groups, including sex, age of onset, duration, quality, and clinical course ("chronic" indicates disease course ≥ 1 year). In addition, we also calculated the score of the reversible cerebral vasoconstriction syndrome2, a scale developed to differentiate reversible cerebral vasoconstriction syndrome from other intracranial vascular disorders. RESULTS: Overall, 245 patients with headache associated with sexual activity were enrolled. Our clinic-radiologic composite algorithm diagnosed and classified all patients into four groups, including 38 (15.5%) with primary headache associated with sexual activity, 174 (71.0%) with reversible cerebral vasoconstriction syndrome, 26 (10.6%) with probable reversible cerebral vasoconstriction syndrome, and 7 (2.9%) with other secondary causes (aneurysmal subarachnoid hemorrhage (n = 4), right internal carotid artery dissection (n = 1), Moyamoya disease (n = 1), and meningioma with hemorrhage (n = 1)). These four groups shared similar clinical profiles, except 26% of the patients with primary headache associated with sexual activity had a 3 times greater chance of running a chronic course (≥ 1 year) than patients with reversible cerebral vasoconstriction syndrome. Of note, the reversible cerebral vasoconstriction syndrome2 score could not differentiate reversible cerebral vasoconstriction syndrome from other groups. CONCLUSION: Our composite clinic-radiological diagnostic algorithm successfully classified repeated headaches associated with sexual activity, which were predominantly secondary and related to vascular disorders, and predicted the prognosis. Primary headache associated with sexual activity and reversible cerebral vasoconstriction syndrome presented with repeated attacks of headache associated with sexual activity may be of the same disease spectrum.

Headache Associated with Sexual Activity-A Narrative Review of Literature.

Headache associated with sexual activity (HAWSA) has accompanied humanity since ancient times. However, it is only since the 1970s that it has become the subject of more extensive and detailed scientific interest. The purpose of this review is to provide an overview of the development of the concept of HAWSA, its clinical presentation, etiopathogenesis, diagnosis and treatment especially from the research perspective of the last 20 years. Primary HAWSA is a benign condition, whose etiology is unknown; however, at the first occurrence of headache associated with sexual activity, it is necessary to exclude conditions that are usually immediately life-threatening. Migraine, hypnic headache or hemicrania continua have been reported to co-occur with HAWSA, but their common pathophysiologic basis is still unknown. Recent advances in the treatment of HAWSA include the introduction of topiramate, progesterone, and treatments such as greater occipital nerve injection, arterial embolization, and manual therapy. Whether these new therapeutic options will stand the test of time remains to be seen.

Orgasmic migraine aura: Report of two cases.

BACKGROUND: Paroxysmal neurological symptoms occurring with sex cause considerable anxiety and sometimes have a serious cause. Thunderclap headache is the most well-known and requires urgent investigation at first presentation for subarachnoid haemorrhage and other significant pathologies. After exclusion of underlying causes, many prove to be primary headache associated with sexual activity. Orgasmic migraine aura without headache is not currently recognised as a clinical entity. CASE REPORTS: We report two patients with acephalgic orgasmic neurological symptoms fulfilling the criteria for migraine aura. CONCLUSIONS: The incidence of acephalgic orgasmic migraine aura is unknown. It should be considered as part of the differential of paroxysmal sex-related neurological symptoms, and clinically differentiated from fixed deficits, reversible cerebral vasoconstriction syndrome and post-orgasmic illness syndrome.

Recurrent, Severe Coital Headaches Associated With Bilateral Carotid Artery Aneurysms and the Effect of Endovascular Treatment.

Headaches are one of the most common chief complaints in the outpatient setting. Distinguishing between benign and life-threatening headaches can be difficult, particularly in the setting of a pre-existing history of headaches. Here, we present a 41-year-old female with a past medical history of migraines and uterine leiomyoma status post hysterectomy about nine months ago who presented to the clinic for severe coital headaches and worsening migraines starting eight months ago. Computer tomography angiogram (CTA) head and neck demonstrated bilateral para-ophthalmic internal carotid artery (ICA) aneurysms (right, 7.5, left 6 mm). A diagnostic cerebral angiogram (DSA) was subsequently done and confirmed the CTA findings. The patient underwent left and right flow-diverting stent placement two and four months later, respectively. One week after the right ICA stent placement, her headaches had improved to one to two times per week. At six months after the stent placement, she resumed her normal sex life and her migraines returned to baseline. Our case suggests that recurrent severe coital headaches are associated with bilateral carotid artery aneurysms. Thus, while assessing a patient with recurrent coital headaches, it is important to have a wide arsenal of differentials to rule out possibly catastrophic causes.

Gender and hormonal influences in reversible cerebral vasoconstriction syndrome.

INTRODUCTION: The reversible cerebral vasoconstriction syndromes, including postpartum angiopathy, have been characterized over the last decade. Women are predominantly affected. Some studies suggest that postpartum angiopathy carries a worse prognosis. PATIENTS AND METHODS: We compared the clinical, neuroimaging, and angiographic features of 36 men, 110 non-pregnant women and 16 postpartum women included in our single-center cohort of patients with reversible cerebral vasoconstriction syndromes encountered from 1998 to 2016. RESULTS: As compared to men, non-pregnant women were older (48 ± 11 vs. 34 ± 13 years, p < 0.001), had more underlying migraine (49% vs. 19%, p = 0.002), depression (53% vs. 14%, p < 0.001) and serotonergic antidepressant use (45% vs. 11%, p < 0.001), developed more clinical worsening (18% vs. 3%, p = 0.022), more infarcts (39% vs. 20%, p = 0.031) and worse angiographic severity scores (23 ± 14 vs. 10.9 ± 10.3, p < 0.001), but had similar discharge outcomes (modified Rankin scale scores 0-3, 90% vs. 91%, p = 0.768). Sexual activity was an important trigger in men (22% vs. 4%, p = 0.002). As compared to non-pregnant women, postpartum angiopathy patients were younger (33 ± 6 years, p < 0.001) and had less vasoconstrictive drug exposure (25% vs. 67%, p = 0.002) but showed similar clinical, radiological and angiographic findings and similar discharge outcomes (modified Rankin scale scores 0-3 in 94%, p = 0.633). There were no significant differences between pre- and post-menopausal women, or those with and without hysterectomy. DISCUSSION/CONCLUSION: The observed gender differences in reversible cerebral vasoconstriction syndromes may result from hormonal or non-hormonal factors. Hormonal imbalances may trigger reversible cerebral vasoconstriction syndromes. Given the absence of significant differences in the female subgroups, hormonal factors do not appear to significantly affect the course or outcome of reversible cerebral vasoconstriction syndromes.

Αn uncommon coexistence of primary sexual, cough and exercise headaches: the first three cases from Greece.

BACKGROUND: The "other primary headaches" encompasses a group of uncommon but distinct headache disorders. The coexistence of their subforms such as primary sexual and exercise headache is not a new phenomenon, but in association with cough headache is rather uncommon. Report of cases: We report three cases with a rare coexistence of primary cough, exercise and associated with sexual activity headache. Indomethacin was effective in all patients. The leading pathophysiological explanation involves a rapid rise in intra-abdominal pressure exertional factors or an inappropriate reaction in the cerebral vasculature. CONCLUSIONS: Further studies are needed to confirm a common pathogenic mechanism in these patients. Τhe coexistence of these headaches needs to be taken into account in the final classification that is expected to be available in 2016. Hippokratia 2015; 19 (4): 369-371.