A 41-year-old man was admitted to the general emergency room (ER) because of an extremely severe headache of instantaneous onset (1 min at most), probably the worst ever in his life. He had described of having two previous similar episodes within a few days all during sexual intercourse, including the final one that brought him to the ER.

His neurological and general examinations were totally normal, and the patient had no more pain for 12 h. No other triggers such as exposure to vasoactive drugs or any other circumstances related to these severe headache episodes other than sexual activity could be demonstrated. However, the absence of any associated symptoms and strictly normal physical and neurological examinations do not exclude a serious underlying life-threatening cause and an urgent diagnostic workup is needed in such cases.

The patient was referred to the emergency headache centre (EHC) for appropriate investigations. His cranial computerized tomography (CT) scan was normal. The initial neuroimaging study then was followed by a spinal tap, which showed a normal opening pressure, no cells and normal biochemistry. Later in the afternoon he was further investigated with MRI, MRA and MRV, with none of them revealing any abnormality. Despite that all these studies were normal, it was decided to carry out a conventional angiography to rule out definitely a reversible cerebral vasoconstriction syndrome (RCVS). This study also disclosed no abnormality, and therefore, as all exams were normal, it was concluded that his diagnosis was “primary headache associated with sexual activity”.

It is a severe high-intensity headache of abrupt onset; we must investigate with a detailed questionnaire:

The patient must be urgently referred to an emergency department and if possible to a hospital with neurological department.

The prevalence of sexual headache is unknown. In the only population-based epidemiological study, the lifetime prevalence was 1 % with a broad confidence interval and similar to that of primary cough and exertional headaches. The prevalence of this headache may be underestimated, since patients often feel embarrassed about reporting it. In terms of consultation in headache clinics, it accounts for 0.2–1.3 % of all headache patients.

The age of onset of patients consulting due to this headache is 35–39 years (range 20–50 years). Similar to exertional headache, the male-to-female ratio is 3–4/1. The dull type occurs in less than one-quarter of patients. Two-thirds of patients have their headache in a bout: at least two attacks occurring in over 50 % of sexual activities and then none for more than 2 weeks.

The number of attacks per bout ranges from 2 to 50, and the mean duration of the symptomatic period is 3 months, though a minority of patients suffer from sexual headache for several years without apparent remission. Most of these patients experience infrequent (<20 % of sexual activities) attacks.

Pain characteristics are also similar to those described for primary exertional headache. The duration of pain is heterogeneous, ranging from 1 min to 24 h. Most patients have severe pain for between 1 and 3 h followed by mild pain for about 4 h. Pain is bilateral in two-thirds of patients, usually occipital or diffuse, and of a dull (47 %), throbbing (47 %) or stabbing (45 %) quality.

Patients with sexual headache are usually healthy people, with no vascular disease. Two-thirds, however, suffer from other headache disorders such as episodic tension-type headache (35 %), migraine (25 %) and chronic tension-type headache (10 %). Comorbid migraine and exertional headache are more frequent in orgasmic headache.

Subarachnoid haemorrhage occurs during sexual activity in 4–12 % of cases.

Decreased levels of consciousness, vomiting, meningeal signs, focal symptoms and severe pain lasting more than 24 h should be interpreted as “red flags” requiring immediate diagnostic workup. A minority of patients experiencing cough headache due to Chiari type I malformation or some other posterior fossa abnormality also notice head pain during orgasm. This is logical if we consider that sexual intercourse is a mixture of prolonged physical exercise and Valsalva manoeuvres.

To advise that the course of the condition is limited in time, explain that the headaches recur during several sexual encounters over a period of time and never return again, but also inform that the course may be unpredictable; some patients experience them from time to time throughout their lifetime.

It has been reported that when patients resume sexual activity within days after an attack, the headache may recur, so advising the patients to refrain from sex for a week after an attack might be prudent.