A 43-year-old man presented with a 27 year history of mental debility, fatigue and headache among other symptoms () associated with ejaculation and orgasm during any form of sexual activity. Involuntary nocturnal emission also produced it. The syndrome occurred 20 min after sexual activity and lasted for exactly 72 h. During this 72 h the pounding headache was constant and with other symptoms impacted on his ability to function. It would switch off after 72 h as quickly as it started. If the patient underwent sexual activity every 3 days, which was not usual, he would never be free of symptoms.
The patient’s past health was good. He had two psychiatric assessments in 1981 and 1996, and three neurological assessments in 1996 and 1997, with a tertiary referral being made to the national Hospital for Neurological Diseases. He has been fully investigated with 24 h electroencephalogram (EEG), magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) scans of the brain and numerous blood tests. No abnormality or reason for his symptoms could be elucidated.
When he attended this medical centre he was keen to seek further help. Propranolol 10 mg twice daily was prescribed prophylactically at this time but the patient did not persevere with treatment.
He presented again 3 years later, in July 2008. He said he had both good news and bad news. Firstly, 8 weeks into his wife’s and the patient’s own first pregnancy, sexual activity no longer brought on his syndrome. After the baby was born, however, his condition fully returned.
At 8 weeks serum progesterone values rise greatly in a pregnant women and similarly fall after delivery. The patient had done his own research and wanted me to prescribe progesterone. His condition was now also a stress for his wife. I was reluctant to prescribe oral progesterone to a relatively young male. Instead I prescribed progesterone cream 8% which I suggested he apply around the nostril and upper lip area daily. This proved to be ineffective as did a subsequent prescription of a trial of the dopamine agonist, bromocriptine 2.5 mg daily.
In October 2008 I agreed to prescribe oral progesterone. The patient signed a letter of consent after a discussion of possible side effects. I prescribed norethisterone 5 mg (10 tablets). He was to take a tablet around 30 min before sexual activity.
When I saw him again 4 months later, the treatment had been very successful with complete prevention of his attacks. He had obtained a further supply of norethisterone from a colleague at the medical centre in the interim as I was not available, and was “pleased beyond words” (sic.).
He said if he took a norethisterone tablet before the attack it alleviated his symptoms 95%, with only a background headache remaining for about 24 h.
He then usually took a further 5 mg norethisterone daily for the following 2 days to alleviate the residual symptoms and negate the possibility of his symptoms regenerating, although it is not clear in this regard if the extra medication was necessary.
On several occasions he took 10 mg norethisterone within a few minutes after sexual activity, but less than the latent period, and his syndrome did not manifest at all.
Over a 9–10 month period norethisterone was consistently effective. The patient was relieved of more than 30 episodes of his syndrome by the progestogen without exception.
Correspondence from Merck, the manufacturer of norethisterone, at this time confirmed there were no known harmful side effects in males.
During the compilation of this report the patient’s partner became pregnant again. Once more the patient found his condition spontaneously relieved 8–10 weeks into the pregnancy.
It was arranged to take blood from the patient to measure his serum progesterone during his wife’s pregnancy.