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 Hughes Syndrome Foundation National 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
“Sleep problems”.
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.
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MaryF
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Apologies for posting on such an old post! How does anyone find a consultant to look into this kind of thing in more depth? I'm struggling with some kind of "funny turns" and just generally feeling weird (sometimes it's like my eyes are crossed, feeling lightheaded, weak, hard to describe really). Some descriptions of epilepsy and narcolepsy sound similar. I have repeatedly tested positive for IgM anticardiolipin antibodies, one positive ANA test, symptoms of Lupus, Sjögrens and/or APS, also previous migraines and livedo reticularis. I've had some brain scans that found a narrowing caused by arachnoid granulations, which have since been classed as normal/developmental.
I see a rheumatologist, but only having blood tests every 6-12 months for Lupus, Sjögrens & APS (I think). I take Hydroxychloroquine, but it hasn't helped with the symptoms. I get shut down by rheumatology and GPs everytime I try to seek help for the funny turns, as they think it's anxiety. I wish I could see Dr Graham Hughes! Are there any good private consultants with a similar outlook, who anyone can recommend? I sort of need someone to look into the whole autoimmune and APS thing as a whole, I think. I'm in the north west UK, but would consider anywhere in the UK if it meant finding the right person.
You could refer yourself privately to The London Lupus Centre, London Bridge Hospital. Also just to rule it out, you could order your own panel of tests from Medichecks (their advanced thyroid test), which includes many other things including B12. You then get an email from the doctor who reads the results, showing your ranges and outcomes. The reason I say this, is getting tested on the NHS is not very reliable for Thryoid and at times also B12 deficiency.
At least you may have a clue to yes or no with that. I did this myself to prove that I had not only a thyroid problem that had not shown up on the basic TSH test done by GP's and the same for my B12. MaryF
I had heard something about the NHS failing to test for a certain part of thyroid screening, so that might be worth looking into. I didn't know about the B12 test, though! Mine was ok when checked about 6 months ago, but vitamin D was a bit low. I don't know why, but my zinc levels are always low, even when they've given me high strength prescription zinc. I notice the GP doesn't even test it anymore, probably because they were fed up with it never changing. I know something is going on that isn't anxiety.I have thought about the London Lupus centre, but was a bit confused about which consultant when I looked at the website. I probably should just email them as they must get many people like me and have more of an idea about who I need to see. I do have the name of a neuropsychiatrist within the NHS in my area and going to ask my psychiatrist if they can refer me. I suspect they'll say no, but it's worth a try.
if you are taking any supplement it can influence the result for up to four months, so I am led to believe. If any of your blood tests have shown high Homocystein that can be a clue, for problems with B12, but not always just for B12, and sometimes a test to see what your intrinsic factor are doing can also prove a need. Be aware the levels are set rather low for dosing B12, The Pernicious Anaemia Society has good information, but the private tests is a starting point. MaryF
Sorry to pester you on the same topic, but I've only ever vaguely heard of Medichecks before and was quite intrigued when I looked on their website. Do the NHS recognise their blood test results or would they try to doubt the results? I worry I'd pay a lot of money for testing, then they'd just dismiss it.
I have always had mine accepted and they were found to be useful, however they are read by a doctor who adds their own notes on. We should not have to do this, but in many cases it turns out to be necessary. MaryF
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Professor Graham Hughes' October blog
