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 headaches associated with physical exertion.

BACKGROUND: Headaches associated with physical exertion include headache precipitated by coughing or other Valsalva maneuvers, headache brought on by prolonged physical exercise, sexual headaches and cardiac cephalalgia. OBJECTIVE: To review and update the clinical characteristics, etiologies, pathophysiology and management of these headaches related to exertion. METHODS: In depth review of the publications, both in PubMed and in the main textbooks, of the different headaches induced by physical exercise. RESULTS: Cough, exercise and sexual headaches can be primary or secondary; therefore, complementary studies are mandatory to rule out structural lesions. However, clinical characteristics, such as an old age and response to indomethacin for cough headache or being a young male and response to beta-blockers for exercise and sexual headaches, plus a normal examination are suggestive of a primary etiology. Etiology for secondary varieties, as posterior fossa lesions for cough headache or vascular malformations for exercise and sexual headaches, are also different. Finally, headache as a distant manifestation of myocardial ischemia, also known as "cardiac cephalalgia", appears at exertion in around two-thirds of cases and typically lasts less than 30 minutes and is relieved by nitroglycerine. CONCLUSIONS: Primary and secondary cough headache can usually be suspected based on clinical characteristics and separated from exercise and sexual headaches, which share many aspects. Cardiac cephalalgia is not necessarily an exertional headache and should be considered in adult patients with short lasting headaches and patent vascular risk factors.

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.

Uncommon (Group 4.0) Primary Headaches: Less Familiarity and More Missed Diagnosis.

BACKGROUND: Other primary headaches make up group 4.0 of the International Classification of Headache Disorders third edition (ICHD-3). This group includes a clinically varied group of headache entities whose pathophysiology is not well understood and whose treatments are based on open-label reports. OBJECTIVE: To review and update the uncommon primary headaches included under group 4.0 in ICHD-3. METHODS: We reviewed the headache disorders listed under group 4.0 of ICHD-3 for the clinical features, diagnostic criteria, and management, and described recent updates of these relatively rare disorders. RESULTS: The entities included in this group have a characteristic presentation in practice. Some of them are activity-related and can be provoked by Valsalva maneuver (primary cough headache), some by prolonged exercise (primary exercise headache), and some by sexual excitation (primary headache associated with sexual activity); primary thunderclap headache has also been included here because all the above-listed entities can sometimes present in thunderclap fashion too. Two of the entities in this group 4.0 are linked to direct physical stimuli viz. cold stimulus headache and external pressure headache. Also included in this group are primary stabbing headaches and nummular headaches that are presumed to be due to the involvement of the terminal branches of the sensory nerves supplying the scalp and are, therefore, termed "epicranial headaches." Hypnic headache syndrome and new daily persistent headache are included here as "miscellaneous headaches" because we still do not know enough about their causation. CONCLUSION: The headache disorders included under group 4.0 in ICHD-3 are uncommon, heterogeneous entities, which may pose diagnostic and management challenges to the physicians. The diagnosis may be missed owing to a lack of familiarity.

Clues in the differential diagnosis of primary vs secondary cough, exercise, and sexual headaches.

Activity-related headaches can be provoked by Valsalva maneuvers ("cough headache"), prolonged exercise ("exertional headache") and sexual excitation ("sexual headache"). These entities are a challenging diagnostic problem as can be primary or secondary and the etiologies for secondary cases differ depending on the headache type. In this paper we review the clinical clues which help us in the differential diagnosis of patients consulting due to activity-related headaches. Cough headache is the most common in terms of consultation. Primary cough headache should be suspected in patients older than 50 years, if pain does not predominate in the occipital area, if pain lasts seconds, when there are no other symptoms/signs and if indomethacin relieves the headache attacks. Almost half of cough headaches are secondary, usually to a Chiari type I malformation. Secondary cough headache should be suspected in young people, when pain is occipital and lasts longer than one minute, and especially if there are other symptoms/signs and if there is no response to indomethacin. Every patient with cough headache needs cranio-cervical MRI. Primary exercise/sexual headaches are more common than secondary, which should be suspected in women especially with one episode, when there are other symptoms/signs, in people older than 40 and if the headache lasts longer than 24 hours. These patients must have quickly a CT and then brain MRI with MRA or an angioCT to exclude space-occupying lesions or subarachnoid hemorrhage.

Ischaemic stroke provoked by sexual intercourse.

The association between long term risk factors and stroke has been well established, but very little is known about factors that may precipitate acute stroke. We describe two young women presenting with ischaemic stroke triggered by sexual intercourse. Patient 1 presented with a cardioembolic stroke probably secondary to the interaction between a patent foramen ovale and thrombophilic abnormalities; Patient 2, presenting with orgasmic headache, had a cryptogenic striatocapsular infarct. Sexual intercourse should be considered as an unusual, but possible, trigger of cerebral ischaemia, especially in young patients presenting with cryptogenic stroke.

Otras cefaleas primarias / Other primary headaches

El capítulo de otras cefaleas primarias, abarca un grupo de cefaleas descritas como desórdenes clínicos heterogéneos. Aunque estas entidades no son frecuentemente encontradas en el escenario clínico es relevante conocer los aspectos epidemiológicos, fisiopatológicos, clínicos y de tratamiento concernientes. Una de las características de mayor relevancia en este grupo está dado por el pobre entendimiento de los mecanismos fisiopatológicos y por las recomendaciones terapéuticas basadas en reportes anecdóticos por lo tanto la Asociación internacional de dolor de cabeza estimula la investigación de estas entidades en pro de adquirir un mayor grado de comprensión de este tipo de cefaleas. Presentamos una revisión de los principales artículos relacionados con hemicrania continua, cefalea hípnica, cefalea primaria por tos, cefalea primaria relacionada con actividad sexual, cefalea primaria por ejercicio, cefalea primaria tipo punzada, cefalea primaria tipo trueno y nueva cefalea diaria persistente.

This chapter, other primary headaches, encompasses a group of headaches that are described as heterogeneous clinical disorders. Although these entities are not frequently seen in the setting of general consultation, it is relevant to know the most important aspects related to epidemiology, clinical manifestations, pathogenesis and treatment. One of the most relevant features of this group is that pathogenesis and suggested treatments are based in hypothetical descriptions and anecdotal reports therefore the International headache association aims to stimulate further investigations of these entities in order to get broader comprehension of this group. We review recent important papers pertaining to hemicrania continua, hypnic headache, primary cough headache, primary headache related to sexual activity, primary exertional headache, primary stabbing headache, primary thunderclap headache and new daily persistent headache.