May was best forgotten as far as the weather was concerned – rain, cold and windy – once again one felt sorry for the tourists huddled on the open tour buses and the river cruise boats. However, as I write this, “June is bustin out” ……. “please disregard my previous letter…”
It’s funny how lectures and meetings seem to come in runs. Although I have cut down on travel, there were two overseas lectures, as well as the annual charity Patients’ Day at St Thomas’ hospital, here in London.
The first overseas meeting was in Rome, at the Italian ‘Ten Topics in Rheumatology’ organised by my good friend Guido Valesini – 250 attendees and a very high standard. The only downside was that my day in Rome coincided with a public transport strike. Gridlock.
The other overseas lecture was in Riyadh – the 1st Saudi Lupus Symposium. Numerous articles in the scientific journal LUPUS have suggested that lupus is common in Saudi – as it is in other Middle East countries such as Kuwait. The meeting mirrored that feeling – over 300 doctors in the audience, huge interest and enthusiasm.
Interestingly, a number of the doctors at the meeting had the feeling that Hughes syndrome was over-taking lupus – possibly secondary to the growing recognition of the syndrome by specialities such as cardiology, gastroenterology, surgery and …… neurology, the area of this month’s ‘patient of the month’.
Patient of the Month
Ms A.C., now aged 38, had a long history of sleep disturbance. She believed her problems started in her early teens when she went down with a prolonged bout of glandular fever. She never fully recovered and started to suffer headaches, sometimes migrainous. She also developed ‘absences’ and other ‘funny turns’, and was diagnosed as having ‘temporal lobe epilepsy’.
But in addition, she had another problem which was to become her over-riding complaint – sleep disturbance. As the years went by, she developed narcolepsy –falling suddenly asleep, for example, at the dinner table or at a desk.
Even more dramatic, she developed ‘cataplexy’ – a condition in which the patient can suddenly fall to the floor – sometimes triggered by emotion, such as laughing or anger.
She was investigated and various treatments tried – to no avail.
How did she end up in my clinic? Well, one of her physicians, knowing that her mother had lupus, wondered if Ms. A.C. had something similar. The lupus tests were negative, but interestingly, one of the three tests for antiphospholipid antibodies (aPL) was weakly positive. Could she have Hughes syndrome? In fact, there were clues – the previous migraines, the temporal lobe epilepsy (seen in a number of my patients), the dry eyes (Sjögrens), the blotchy appearance (‘livedo reticularis’) on the arms and thighs and, for what it’s worth, a history of two metatarsal fractures in the foot.
Worth treating? Absolutely. Aspirin was out – it had worsened her mild asthma in the past. So – on to daily clopidogrel (‘Plavix’) with plans for a trial of heparin if that failed. It didn’t! Back down to London two months later – “I’m cured” – the narcolepsy and cataplexy had gone. And no more headaches or ‘funny turns’.
Ms. A.C.’s history is one of the most dramatic I have witnessed. I had figured on at least a trial of heparin and possibly onto anticoagulants such as warfarin.
What is this patient teaching us?
Many lessons. Firstly, that Hughes Syndrome is very much a ‘neurological’ disease. We have only scratched at the surface of this aspect of the disease.
Secondly, never forget the family history.
Thirdly, even in the presence of weak or negative tests, the diagnosis can still be considered.
And finally, Hughes Syndrome is NOT just thrombosis and miscarriage
Professor Graham R V Hughes MD FRCP